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ATLAS OF 

CONGENITAL CARDIAC DISEASE 




Jean-Baptiste de Senac 
(1693-1770) 


Giovanni Baptista Morgagni 
(1682-1771 



Thomas B. Peacock (1812-1882) 


Sir Arthur Keith (1866- 


Great Scientists Who Have Made Fundamental Contributions to Our 
Knowledge of Congenital Heart Disease 


) 

(Frontispiece.) 










ATLAS 

OF 

CONGENITAL CARDIAC 

DISEASE 


BY 

MAUDE E. ABBOTT, B.A., M.D., F.R.C.P. (Canada) 

Curator of the Historical Medical Museum and 
Assistant Professor of Medical Research 
McGill University, 

Montreal, Canada 


Published by 

THE AMERICAN HEART ASSOCIATION 

50 West 50th Street, New York, N. Y. 


Copyright, 1936, by 
Maude E. Abbott 



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FOREWORD 


Senac, Peacock, Rokitansky, and Keith, one after the other, richly ad¬ 
vanced our knowledge of congenital heart disease, but it was left to Maude Abbott, 
fired by a spark from Osier, to make the subject one of such general and widespread 
interest that we no longer regard it with either disdain or awe as a mystery for the 
autopsy table alone to discover and to solve. She has been the most important 
of the pioneers in establishing congenital heart disease as a living part of clinical 
medicine. 

For a long time the medical world has awaited a textbook on congenital heart 
disease by Dr. Abbott, whose contributions in this field have up to this time been 
accessible only in her system-monographs or in the periodical literature. In 
assembling her material in preparation for a future work of such importance she 
presented a notable exhibit before the Centennial Meeting of the British Medical 
Association in London in 1934 and again before the Joint Meeting of the American 
and Canadian Medical Associations in Atlantic City in 1935 and before the Ontario 
Medical Association in 1936. It is this exhibit with some modifications and addi¬ 
tions that has been put into permanent and convenient form as an Atlas. 

Paul D. White. 

Boston, Mass. 

July , 1936. 





INTRODUCTION 


This volume presents, in a somewhat unusual form, a 
pictorial retrospect of the author’s personal experience in 
what may now be considered a specialized field of clinico- 
pathological research. A first-hand knowledge of the 
exact morphology of a large range of cardiac anomalies, 
obtained in the first place through an intensive early 
study of the rich material accumulated under the author’s 
care as Curator of the Medical Museum of McGill Uni¬ 
versity, has been continuously applied and amplified 
through more than three decades of activity, by observa¬ 
tion of congenital heart disease in the wards and autopsy 
rooms of many great hospitals both at home and abroad. 
Of recent years, also, an ever-increasing consultant corre¬ 
spondence has grown up between the writer and clinicians 
in many parts of this continent, who have sent in their 
material, often unique of its kind, to the McGill Museum 
for description and diagnosis. This circumstance has 
brought a wider range of these rare conditions under 
personal notice than would have been otherwise possible 
and has thus supplied a large opportunity for the 
evaluation of the various aspects of this subject and the 
segregation in it of the different clinical types into which 
these conditions naturally fall. The flood of recent 
literature and the more exact data now available through 
modern laboratory methods have been likewise conducive 
to the clearer understanding of this subject, which is only 
now coming into its own as a field of vital human interest. 
It is, however, a well-recognized fact that, in clinical 
medicine, the intimate personal knowledge of a relatively 
small number of individual cases is likely to yield a 
richer harvest in the understanding of diseased condi¬ 
tions than wider generalizations covering a more vast 
material. The concentration upon the clinical picture 
presented by his own carefully studied patients was the 
source of Sydenham’s contribution to the nosology of 
disease; and Osier himself looked back upon the 750 
autopsies performed by himself at the Montreal General 
Hospital as the very foundation stones of his career. In 
the intriguing subject before us, the study of each 
individual case is indeed the sole key to the compre¬ 
hension of these obscure conditions, in the investigation 
of which “the music of the spheres” is dimly perceptible 
behind the inevitable fulfillment of ontogenetic and 
teleologic laws. It is, therefore, this service which the 
present Atlas seeks to render to the student, in placing 
before him, in objective form, the actual morphological 
substratum of the experience of a single individual 
worker. Not all the cases presented were the author’s 
“own,” in the narrower sense of the term, but all, even 
those culled from the writings of the great observers of an 
earlier generation, may be considered, through the 
writer’s intimate familiarity with their detail and con¬ 


tent, a personal observation, in the deeper meaning of the 
term. The conditions illustrated here represent a fairly 
complete range of those cardiac anomalies “of clinical 
significance” observed to date, the only serious omissions 
(made for lack of space) being those of congenital pul¬ 
monary dilatation (on which see B. S. Oppenheimer, 
TV. Assn. Amer. Phys., 1933, 48 : 290) and pericardial 
defect (reviewed by the writer in Osier’s Modern Medi¬ 
cine, 1927, 4: 657). 

A short autobiographical statement, relating how the 
writer’s attention was first drawn to this extraordinarily 
interesting subject, the part which Sir William Osier 
took in rendering effective her early work in this field, 
and the evolution of the research itself will be, we 
believe, in place here. Appointed to the curatorship 
of the McGill Museum in the year 1899, just after her 
return from two years’ preliminary training in Internal 
Medicine and Pathology under Ortner and Kolisko in 
Vienna, the author became at once interested in an 
unlabeled specimen in the collection. This was a three- 
chambered adult heart presenting an anomalous septum 
cutting off a small supplementary cavity, from which 
arose the (untransposed) pulmonary artery (PL XXI, 
Fig. 6a). At the suggestion of the late Professor Wyatt 
Johnston (himself a museum enthusiast), an inquiry was 
addressed to Dr. Osier, then in Baltimore, asking whether 
he could identify the case. He replied promptly that he 
remembered the specimen perfectly, having often demon¬ 
strated it to students during his professoriate at McGill, 
and that it had been reported in an early Edinburgh 
journal by “old Dr. Andrew Holmes” (first Dean of 
the McGill Medical Faculty). The writer accordingly 
located the article in the Transactions of the Edinburgh 
Medico-Chirurgical Society in 1824, where it appeared 
with a fine copper plate engraving of the heart, and 
learned from it that the autopsy on this case (which is 
still unique in the literature of cardiac anomalies) was 
performed by Dr. Holmes at the Montreal General 
Hospital in 1823, the year of the foundation of the 
McGill Medical School, in the presence of the other three 
founders of that institution. This remarkable combina¬ 
tion of interesting features and circumstances led to her 
republication, in 1901, of Dr. Holmes’s report along 
with his engraving of the heart and a semidiagrammatic 
sketch of the circulation by Dr. R. Tait Mackenzie 
(PI. XXI, Fig. 66), together with a biographical sketch 
of this early clinico-pathologist, who is one of the most 
important and interesting figures in the medical history 
of that period on this continent. It was this semi- 
historical, semipathological article, together with the 
writer’s activities in the cataloguing of the Pathological 
Collection made by Dr. Osier during his term as Patholo- 


Vll 


INTRODUCTION 


gist to the Montreal General Hospital and housed 
at that time under her care in the Museum, that led 
Sir William, in 1905, to extend to her the invitation to 
write a section on Congenital Cardiac Disease in his new 
System of Modern Medicine. He himself suggested that 
the subject might be treated “statistically” and the first 
edition of this monograph accordingly contained a chart 
of 412 cases of congenital heart disease with autopsies 
analyzed from the literature. Fully one-third of these 
were drawn from the Transactions of the Pathological 
Society of London, that goldmine of early clinico-patho- 
logical observation, and Vierordt’s great monograph, 
which had appeared shortly before this and was actually 
the first which had handled the subject from the statis¬ 
tical standpoint, was used as a guide and sourcebook 
throughout. 

Dr. Osier received the proof of the first edition of this 
monograph at his home in Oxford in January, 1908. 
With his characteristic generosity toward younger 
workers, and with the insight that impelled him to the 
instant recognition of what he believed to be a contri¬ 
bution of scientific merit, he immediately sent off a letter 
to the writer expressing his warm approval of its 
contents. 

In the second edition of Osier and McCrae’s System, 
which appeared in 1915, the number of cases analyzed 
by the writer was raised to 631, and in its third edition 
published in 1927, there were 850 cases analyzed. In 
1924 another monograph by the writer on the Treatment 
of Congenital Heart Disease appeared, and in 1928, one 
on the diagnosis of this condition (Part 1 with Dr. E. 
Weiss), and finally in 1932, in Nelson’s Loose-leaf Medicine, 
a fourth, containing a review of the recent literature on 
this subject from the publication of the third edition of 
Osier and McCrae’s System in 1927 to that date, with a 


chart of 1,000 cases analyzed (republished here by kind 
permission of Thomas Nelson & Sons). Finally, the 
Atlas here published is merely preliminary to a larger 
volume on congenital heart disease which the author has 
under preparation. In its objective presentation of the 
individual cases studied, it has, we believe, a supple¬ 
mentary function of real value, which only a publication 
of this type can adequately fulfill. 

The writer’s sincerest thanks are expressed to the 
American Heart Association for their valuable support 
in their publication of this Atlas and especially to Dr. H. 
M. Marvin, Executive Chairman, and Miss Gertrude 
Wood, Office Secretary, for their courtesy and coopera¬ 
tion ; to Dr. Paul White and his staff and the many other 
contributors who have placed their unpublished cases so 
freely at the writer’s disposal for publication here; to 
Mr. Lucius N. Littauer of the Littauer Foundation for 
financial support kindly given for the preparation for 
publication of the writer’s researches in this field; and 
to Dr. Maurice Kugel for his generous action in the same 
regard; to Messrs. Lea and Febiger and the other 
publishers and the medical artists mentioned in the 
text, whose original drawings and fine reproductions 
embellish these pages; to Dr. David Seecof of the 
Jewish Hospital of Montreal for kind assistance in the 
photographic and other preliminary work incidental 
to the inception of this Atlas and to Dr. Otto Kruger for 
his valuable help in the construction of the plates; and, 
last but not least, to the writer’s efficient secretary, Miss 
Edna F. Graham, with whose help and faithful coopera¬ 
tion this Atlas and the exhibits upon which it is based 
have attained their present adequate form. 

Maude E. Abbott. 

Montreal, Canada. 

July 15, 1936. 


Vlll 


TABLE OF CONTENTS 


Frontispiece. Great scientists who have made fundamental contributions to our knowledge of congenital heart disease. 

Jean-Baptiste de Senac (1693-1770), known as the father of cardiac pathology, who is said to have first ascribed la 
maladie bleue to absence of the interventricular septum. 

Giovanni Baptista Morgagni (1682-1771), founder of modern pathological anatomy, who published the first case of 
congenital cyanosis in pulmonary stenosis. 

Thomas B. Peacock (1812-1882), London clinician, author of the first standard textbook on Malformations of the 
Human Heart (1855). 

Carl Freiherr Rokitansky (1804-1878), Professor of Pathological Anatomy in the University of Vienna, author of 
epoch-making investigations into the pathogenesis of cardiac septal defects in the light of the new science of embryology. 

Sir Arthur Keith (1866- ), anatomist and archaeologist, Curator of the Museum of the Royal College of Surgeons 

of England from 1908 to 1933 and now Master of its Surgical Research Farm at Down, Kent, who applied the facts of 
comparative embryology and phylogenesis to the elucidation of cardiac anomalies with brilliant results. 


Page 

Foreword .. v 

Introduction . vii 


PART I 

DEVELOPMENT AND COMPARATIVE ANATOMY OF THE HEART 

Plate I. Development of the Reptilian and Mammalian Heart . 2-3 

Transverse section through the heart of a 10-mm. pig embryo—early stages in the development of heart of Lacerta agilis (Greil) 

—reconstruction of interior of bulbar region and origin of great trunks in two turtle embryos—the same at different stages up 
to end of eighth week in the human heart (Bremer). 


Plate II. Comparative Anatomy. Fish and Amphibian Heart. 4-5 

Hearts of teleost fish (angler) and of Ganoidei ( Lepidosteus osseus or bony pike)—of Elasmobranchii (skate and shark fishes)— 
of Necturus or mud-puppy (amphibian)—diagrams of the bulbus cordis of the fish—of the human heart at end of sixth week. 

Plate III. Comparative Anatomy ( Continued ). The Reptilian Heart. 6-7 

Heart of turtle ( Chelonia mydax )—diagram of reptilian heart—heart of water-snake ( Matrix sitedon )—of python—of croco¬ 
dile—transformation of aortic arches. 

Plate IV. Incomplete Torsion in the Causation of Cardiac Defects (Spitzer’s Theory). 8-9 


Spitzer’s diagrams showing relationship of semilunar cusps and septa in the human and the reptilian heart and in the tetralogy 
of Fallot—the same showing relative position of the arterial ostia in the normal heart and in the different types of transposi¬ 
tion—Mautner and Lowys case showing persistence of the right reptilian aorta in a case of “true” transposition— normal 
human heart showing niche of the obliterated right reptilian aorta. 

PART II 

CLINICAL CLASSIFICATION OF CONGENITAL CARDIAC DISEASE 


Plate V. Clinical Classification of Cardiac Defects. Illustrative Diagrams .12-14 

Diagrams of the circulation with illustrative cases in Group I (no abnormal communication)—in Group II, arterial-venous 
shunt with terminal reversal of flow—in Group III, congenital cyanosis with permanent venous-arterial shunt. 

General References . 14 

Group I 

No Abnormal Communication (Acyanotic Group) 

Plate VI. Anomalies of the Aortic Arch and Its Branches .16-17 

Right aortic arch with persistent left root—double aortic arch—double aortic arch with obliterated isthmus—right sub¬ 
clavian artery from descending arch—right pulmonary from innominate artery. 

Plate VII. Coarctation of the Aorta of the Adult Type. Historic Cases .18-19 

Collateral circulation in Reynaud’s case (1828)—in Jordan’s case (1830)—in Meckel’s case, showing excavation of the ribs 


(1827)—heart and aorta with rupture of latter in Otto’s case (1824)—the same in Roemer’s case of sudden death (1839)— 


aortic arch in Cruveilhier’s case (1835) —in Bonnet’s case (1903). 

Plate VIII. Coarctation of the Aorta (Continued). Personal Cases and Clinical Features.20-21 

Complete atresia with cerebral death—Double stricture with bicuspid aortic valve—mycotic aneurysm at seat of coarctation 
—roentgenographs showing characteristic contour and erosion of ribs—vascular signs—ruptured cerebral aneurysm. 

Plate IX. A. Bicuspid Aortic Valve. B. Supernumerary Aortic Cusps.22-23 


A. Diagrams by Lewis and Grant showing architecture of normal and congenitally bicuspid valve—Peacock’s case with 
microphotograph of same—four of Osier’s sixteen cases—microphotographs of normal and bicuspid valve—ease of con¬ 
genitally bicuspid valve with aneurysm of sinus of Valsalva—with dissecting aneurysm of ascending aorta rupturing into 
pericardium—with subacute bacterial endocarditis. B. Fourth aortic cusp incompletely united with third. 

Plate X. Other Anomalies of the Endocardium .24-25 

Subaortic stenosis—aberrant chordae in left ventricle—network of Chiari in right auricle—double mitral orifice—congenital 
tricuspid insufficiency. 


IX 















Page 

Plate XI. A. Hypertrophy of the Heart in Infants. B. Congenital Rhabdomyoma .26-27 

A. Heart and x-ray in a case of glycogenic cardiomegaly—the same with electrocardiogram in anomalous origin of left 
coronary from pulmonary artery—heart with x-ray, electrocardiogram and microphotograph in a case of chronic inflam¬ 
matory changes in myocardium (Ivugel). B. Rhabdomyoma (nodular form). 

Plate XII. Anomalies of Coronary Sinus .28-29 

Receives both pulmonary veins as a single trunk—receives right coronary artery by arterio-venous loop—receives left superior 
cava in absence of right. 

Group II 

Cases of Arterial-venous Shunt with Terminal Reversal of Flow (Cyanose tardive) 


Plate XIII. Patent Ductus Arteriosus .32-33 

Diagrams showing the normal and foetal circulation and that in patent ductus—case of patent ductus with infective pul¬ 
monary endarteritis—skiagraph showing x-ray cap—graphic record of heart signs—electrocardiogram. 

Plate XIV. Defects of Interauricular Septum and Patent Foramen Ovale .34-35 


Diagrams of circulation in the above—case of persistent ostium secundum with pulmonary dilatation and insufficiency—case 
of mitral stenosis with interauricular insufficiency—orthodiagraph of same—persistent ostium primum, two cases, one in 
mongolian idiocy—paradoxical embolism in patent foramen ovale—Osier’s case of premature closure of foramen ovale. 

Plate XV. A. Defects of Interventricular Septum. B. Defects of Aortic Septum .36-37 

A. Diagram of the circulation—hearts from three cases of defect at base of interventricular septum, one with infective endo¬ 
carditis—graphic record of heart signs (Segall)—orthodiagraph—electrocardiogram showing partial heart block. B. Dia¬ 
gram of circulation—case of ruptured aneurysm of the sinus of Valsalva with infective endocarditis—views of both ventricles 
and explanatory model of embryonic bulbus cordis. 

Group III 

Cases of Permanent Venous Arterial Shunt and Retardation of Flow (Cyanotic Group) 

Plate XVI. Symptomatology of Congenital Cyanosis .40-41 

Clubbing of fingers and toes—cyanotic facies—cyanosis retinae—capillary changes—diagrams illustrating pathogenesis 
(Lundsgaard and van Slyke)—characteristic electrocardiogram in tetralogy of Fallot. 

Plate XVII. Pulmonary Stenosis at Lower Bulbar Orifice. All Cardiac Septa Closed .42-43 

Diagram showing course of the circulation—heart and x-rays in conus stenosis with infective endocarditis—heart, x-rays, 
electrocardiogram and myocardium in Eakin and Abbott’s case with multiple somatic defects. 

Plate XVIII. A. Pulmonary and Tricuspid Stenosis with Closed Ventricular Septum. B. Eisenmenger Complex . . 44-45 
A. Diagram of the circulation—heart in valvular pulmonary stenosis—in pulmonary and tricuspid stenosis, two cases, one by 
William Osier. B. Diagram of the circulation in biventricular aorta—heart with x-ray and electrocardiogram showing 
bundle-branch block. 

Plate XIX. Pulmonary Stenosis and Atresia with Defect of Ventricular Septum (Tetralogy of Fallot) .46-47 

Diagram of circulation in pulmonary stenosis with dextroposition of aorta and ventricular septal defect—hearts from two 
cases, one with electrocardiogram—orthodiagram showing coeur-en-sabot —Osier’s case of pulmonary atresia with septal 
defect—diagram of the circulation in this case—electrocardiogram showing partial heart block. 


Plate XX. Pulmonary Atresia with Closed Septum. Aortic, Mitral and Tricuspid Atresias.48-49 

Diagrams of the circulation in the above—two cases of pulmonary atresia, one with x-ray—three cases of aortic atresia and 
mitral aplasia—x-ray of heart in a case of tricuspid atresia. 

Plate XXI. Cor Triloculare Biatriatum and Biventriculare and Persistent Ostium Commune.50-51 

Diagram of the circulation in persistent ostium atrio-ventriculare commune—three cases with mongolian idiocy—one with 
associated absence of transverse arch and mitral atresia—diagram of the circulation in cor triloculare biatriatum—two cases 
with small supplementary chamber cut off by anomalous septum and giving off in one the pulmonary artery (Andrew Holmes’ 
case, 1823) and in the other the transposed aorta—diagram of circulation in cor biventriculare—illustrative case. 

Plate XXII. A. Cor Biloculare. B. Persistent Truncus Arteriosus.52-53 


Diagram of the circulation in cor biloculare with transposition of great trunks with illustrative case—diagrams of the circula¬ 
tion in persistent truncus arteriosus—Feller’s diagrammatic presentation of his three cases—hearts from two personal cases 
of persistent truncus—x-ray of heart and microphotograph of pulmonary cusp in Allan Roos’s case. 

Plate XXIII. Complete Transposition of Great Trunks with Closed Ventricular Septum. 54-55 

Diagrams of the circulation—drawing of a case by Louis Gross—heart, x-ray and electrocardiogram from another case— 
heart and myocardium in complete transposition with aortic atresia. 

Plate XXIV. Complete Transposition of Great Trunks with Defect of Ventricular Septum. 56-57 

Diagram of the circulation—heart and torso in a case with thoracopagus parasiticus—heart, x-ray and electrocardiogram 
from a case with rudimentary interventricular septum, conus stenosis of right ventricle, double mitral orifice, double right 
superior cava and complete congenital heart block (C. F. Moffatt and M. E. Abbott). 

Plate XXV. A. Dextrocardia. B. Corrected Transposition and Complete Congenital Heart Block. 58-59 

Mirror-picture dextrocardia in partial situs inversus, two cases, one with mitral atresia, the other in cor biloculare with pul¬ 
monary atresia—the same in complete situs inversus with cor biatriatum triloculare, malposed pulmonary veins and 
persistent “left” superior cava into coronary sinus—case of corrected transposition of great trunks with inversion of 
ventricles, great dilatation of pulmonary artery and complete congenital heart block (Allan Roos). 


Chart I. Statistics of Congenital Cardiac Disease. 60-61 

Index .... 62 


x 


















PART I 

DEVELOPMENT AND COMPARATIVE ANATOMY OF THE HEART 


PLATE I 


DEVELOPMENT OF THE REPTILIAN AND MAMMALIAN HEART 


An understanding of the elementary facts of human and comparative embryology is essential to an intelligent grasp of the ontogenetic 
problems of congenital cardiac disease. In the light of a knowledge of the successive stages through which the mammalian heart passes 
in its evolution from its primitive tubular state to the completely divided four-chambered organ conducting a double circulation, the most 
bizarre combinations of defects can usually be interpreted quite simply, as due to early arrests of development, marked it may be by 
ingenious structural adaptations of growth. The critical period in the human subject lies between the fifth and eighth weeks of embryonic 
life, i.e., before the cardiac septa are formed, and while the complex processes of torsion, involution, readjustment and fusion are taking 
place at the base, interruption of which is the source of most of the graver anomalies. The figures shown opposite have been selected with 
a view to illustrating this particular stage in development, which is of particular interest in view of the modern phylogenetic theories on the 
ontogenesis of the graver cardiac defects. 

For a complete survey of this subject see the accounts by T. Walmsley, Quain’s Anatomy, 1929, IV, Pt. Ill: 3-20; 37-41; 59-62; 97-108; Cunningham’s 
Anatomy , 6th ed., 1931, 26-34; 1040-1046; also Keith, Human Embryology and Morphology, 1921; Tandler’s article in Keibel and Mall, Embryology, 1912, II; 
534-570; and that by D. Waterston on “The development of the heart in man,” Tr. Roy. Soc. Edin., 1912, 62: 257. 


Fig. 1.—Microphotograph of a transverse section through the heart of a 
10-mm. pig embryo. Note the sinus venosus above on the right, emptying 
into the right auricle between the valvulae venosae dextra et sinistra; the 
large auricles pouching forward on either side above and completely divided 
by the septum primum which has united below with the auricular ventricular 
cushions (septum intermedium) and is perforated above by the foramen ovale. 
The septum secundum has not yet developed. The large ventricular part 
with a spongy musculature and bifid apex lies below. The interventricular 
septum is incomplete, leaving a large defect at its upper border between the 
right and left chambers; the mitral and tricuspid ostia are still lined by embry¬ 
onic endocardial cushions (rudimentary valves). (From a serial section made 
by Alton Maturin of the Harvard Medical School.) 

Fig. 2.—Reconstruction models of the interior of the hearts of two turtle 
embryos at different stages of development, showing the gradual division of 
the conus into three separate vessels, the reptilian right and left aortae and 
the pulmonary artery. 

a. From a 9-mm. turtle embryo (earlier stage). The third and fourth 
pairs of aortic arches emerge at the top of the model, and just below these 
are the conal ridges, which have not yet met and are seen as deep grooves 
leaving high crests between each which will later form the lumina of the three 
separate vessels seen in Fig. 2b below. In the bulbar region the right ridge 
predominates and twists spirally to assume a left central position. (From 
the models by J. L. Bremer, PI. 5, Fig. 12; PI. 6, Fig. 11. Reference under 
Fig. 4 d.) 

b. From a turtle embryo of 16.8 mm. Here division has been completed 
by union of the conal ridges (Fig. 2a above) and the three vessels are seen 
emerging separately from the bulbar region below. 

Fig. 3.—Early stages in the development of the heart of Lacerta agilis. 

1. Heart tube from embryo of 0.4 mm., showing curve to right between 
the fixed cranial (arterial) end above and the caudal (venous) end below. 

2 and 3. Heart tube from embryo of 0.6 mm., ventral and lateral aspects. 

The tube is now S shaped from the turning up of the venous end. I.I.A.B., 
first left aortic arch. 

4 and 6. The same. From an embryo of 0.9 mm. Venous end rising 

posteriorly on the left. In the lateral view the sinus venosus (-|—b) is seen 
receiving the duct of Cuvier ( D.C.s) above and the omphalo-mesenteric vein 
( V.o.m.s .) below. 

6 and 7. The same. From an embryo of 1.2 mm. The auricle (At.) is 
seen in the lateral view (7) to be rising above the ventricular part of the heart. 


8. Heart and truncus arteriosus (T.art.) from embryo of 2 mm. The 
auricle has now risen above the ventricle to the left of the bulbus cordis. Note 
the deep bulbo-auricular (S.b.aur.) and bulbo-ventricular (Kn.F.) clefts. 

9. The same, with a slice removed at the line marked XY in 8, showing 
the interior. Note distal ( d.B.W. II and IV) and proximal (p.B.W.A. and B.) 
bulbar swellings at level of aortic cusps and conus oiifice, radial trabecular 
system ( r.T.S .), endocardial cushions ( E.K.) at auriculo-ventricular orifice, 
and torsion of bulbar cavity. (From Greil, “Anatomy and development of 
the heart and truncus arteriosus in reptiles,” Morphol. Jahrb., 1903, 31: 128- 
130; 304, PI. VI.) 

Fig. 4.—Reconstruction models or casts of interior of the embryonic heart 
showing the progressive torsion that takes place clockwise or dextral at its 
arterial and sinistral at its venous end, and the gradual differentiation of 
chambers, development of septa and division of great trunks. 

a. Model of cavities of heart of chick embryo of 60 hours incubation. 
Flattened S-shaped tube curving to right with venous end entering on the 
left posteriorly and the aortic arches arising from the bulbar enlargement at 
right upper angle. 

b. The same at 4 days 12 hours. The auricles have risen and pouch 
forward. Septum primum appears as a cleft above posteriorly on the left 
leaving a large ostium primum (seen as a solid mass) below. The auriculo- 
ventricular cushions (seen as cavity) separate the mitral and tricuspid orifices 
(seen as wax lines). The bulbar end has undergone a marked clockwise 
torsion toward the median line before giving off the aortic arches above. The 
ventricle has been cut away below and is represented by projections indi¬ 
cating clefts between the trabeculae. 

c. The same at 6 days incubation. The auricles are greatly enlarged with 
corrugated surfaces (indicating clefts) and divided below by the septum 
primum which has met the auriculo-ventricular cushions (seen as cavity). 
The still undivided bulbus lies obliquely in the median line anteriorly and 
gives off the aorta and pulmonary artery. 

d. The same at 6 days 16 hours. The upper part of the auricles and the 
lower part of the ventricles have been cut away. Complete division of the 
auricles, ventricles and great trunks has now occurred. Patent foramen 
ovale in auricular septum. Note that through the spiral torsion that takes 
place in the bulbus (Fig. 2a) the pulmonary rises on the extreme left anter¬ 
iorly and the aorta on the right posteriorly. These are the normal postnatal 
relations. (Republished by permission from J. L. Bremer, “Interpretation 
of development of the heart. The left aorta of reptiles,” Amer. J. Anat., 
1928, 42: 307, PI. 2, Fig. 3; PI. 3, Fig. 6; PI. 4, Fig. 8; PI. 5, Fig. 9.) 


2 


PLATE I. DEVELOPMENT OF THE REPTILIAN AND MAMMALIAN HEART 







Fig. 4a. 


Fig. 4c. 

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Fig. 4d. 


Fig. 1. 


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Fig. 3. 


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PLATE II 


COMPARATIVE ANATOMY. FISH AND AMPHIBIAN HEART 

Much light is cast upon the development of the mammalian heart, and, incidentally, upon the period at which arrest of growth has 
taken place in cardiac anomalies of the graver sort by a comparative study of the adult fish, amphibian and reptilian organ. The truly 
extraordinary way in which these various orders in the ascending vertebrate scale mirror the successive stages through which the human 
heart passes in very early intrauterine life is one of nature’s most spectacular and impressive feats, presenting as it does a complete review 
of this organ’s evolution down to the closure of the cardiac septa in the eighth week of foetal life. The correlation of cases of cardiac 
defect with these fields of embryology and comparative anatomy becomes especially instructive in view of the fact that steps evanescent 
in the human embryo, but fixed in its phylogenetic prototype, are not infrequently revealed in the anomaly, caught in flight as it were 
at the moment of the arrest. The earlier stages of development of the human embryo where the heart is an undivided tubular organ with 
ventricular bend to the right (PL I) are thus mirrored in the primitive heart of the teleost fish, in which there are four chambers arranged 
caudo-cranially, namely, sinus venosus, common auricle, common ventricle and bulbus cordis (Fig. 1). There is no sign of subdivision 
into right and left halves, except possibly at the extreme caudal extremity, which has risen posteriorly in the higher fish orders and pouches 
forward above the ventricle (Figs. 2-3). Similar conditions prevail in the amphibian heart, except that here subdivision into lateral cham¬ 
bers is actually beginning caudally, both in the roof of the auricle and at the entrance of the sinus venosus (Fig. 5). 

The illustrations opposite of fish and amphibian hearts (Figs. 1, 2, and 5) and also those of the python heart (PI. Ill, Figs. 4a and b) are from drawings of 
specimens in the Cardiac Anomaly Collection of the Medical Museum of McGill University, made by the late W. W. Beattie and E. Shanly and published with the 
writer in “Cardiac defects in the light of comparative anatomy,” J. Tech. Meth., 1922, 8: 188. Figure 3 is from Jane Robertson’s brilliant contribution on the 
development of spiral torsion in the bulbus cordis of the higher orders of fish hearts (J. Path, and Bad., 1913-14, 18: 191—210); and Figs. 6 and 7 are from Sir 
Arthur Keith’s Schorstein Lecture on this subject, “The fate of the bulbus cordis in the human heart,” Lancet, Lond., Dec. 20, 1924, reproduced by kind permis¬ 
sion of the authors and publishers. 


Fig 1.—Order Teleostei. (Class Pisces.) Heart of angler (Lophius 
piscatorius).* 

a. Dorsal surface showing sinus venosus opening into laterally expanded 
common auricle below, large thick-walled ventricle with spongy musculature 
in median line above, narrow bulbo-ventricular neck and bulbus cordis at 
extreme upper end giving off aortic arches cranially. 

b. Ventral surface laid open to show common auriculo-ventricular orifice 
at lower end of ventricle in median line, single pair of semilunar valves at 
ower bulbar orifice. (Original drawings by W. W. Beattie and E. Shanly.) 

Fig. 2.—Order Ganoidei. Heart of bony pike (Lepidosteus osseus).* 

a. Bulbus cordis laid open to show rows of valves lining its lumen in differ¬ 
ent stages of development, the smaller, more rudimentary ones indicating 
retrogression incidental to the incipient spiral twisting that is taking place 
here. 

b. Heart showing pyramidal-shaped ventricle below giving off slender 
elongated bulbus above, ending cranially in aortic arches. Auricle juts for¬ 
ward above and behind on either side of bulbus. (Drawings by W. W. Beattie 
and E. Shanly.) 

Fig. 3.—Diagrams of the bulbus cordis of a, Elasmobranch fish; b, of 
Ceratodus; and c, Lepidosiren, the two latter being dipnoan fishes. In a 

there are four vertical rows of bulbar swellings or cusps, Nos. 1, 2, 3 and 4, 
those at the proximal extremities being numbered for comparison with b and 
c, 1, 2 B, 3 and 4 A, respectively. No spiral arrangement. 

In diagram b the swellings are asymmetrically placed and form in the 
transverse portion ( B.C.e.) a spiral valve (Sp.v.), where distal ( D ) and proximal 
(P) constrictions have occurred. 

c. Distal ridges 2 and 4 have disappeared and 3 is a solid ridge while proxi¬ 
mal ridges 1, 2 B and 3 are merely vestigial. There remains only a spiral 
valve (Sp.V.), which begins above as distal ridge 1 and ends below in proximal 
ridge 4 A. (From the article by Jane Robertson, Fig. 1 in her Plate XI.) 

* Specimen in the Cardiac Anomaly Collection of McGill University. 


Fig. 4.—Order Elasmobranchii. Heart of skate (Raia erinacea).* 

a. Ventral surface. The common ventricle curves definitely to the right 
and gives off the elongated conus lined by four horizontal rows of mucoid 
cusps arranged in vertical columns from its right upper end, while the com¬ 
mon auriculo-ventricular orifice enters it at the left upper border. The auricle 
has ascended behind and above ventricle and pouches forward on either side. 

b. Dorsal surface showing interior of common auricle expanded laterally 
with auriculo-ventricular orifice in lower left corner and sinus venosus below 
opening into its floor, between valvulae venosae a little to the right of the 
median line. 

Fig. 5.—Order Urodela. (Class Amphibia.) Heart of mud-puppy (Nec- 
turus).* 

a. Ventral view. The bulbus is a cordlike structure given off from right 
upper angle of pyramidal-shaped ventricle. Auricle to left and posteriorly 
above ventricle. B, bulbus; A, auricle; V, ventricle. 

b. Dorsal view. Auricle shows beginning division of roof, receives orifice 
of sinus venosus below on right with entrance of pulmonary vein to left of 
this. S.7., sinus venosus. (Drawings by W. W. Beattie and E. Shanly.) 

Fig. 6.—Order Elasmobranchii. Heart of shark. Showing structure of 

muscular coats and primitive structure. (From a specimen in the London 
Hospital Museum, reported by Sir Arthur Keith, l.c. under Fig. 7.) 

Fig. 7.—Diagram by Prof. Frazer of the human heart toward end of sixth 
week, showing transformation of bulbus and beginning division of aortic and 
pulmonary channels. A, right bulbar cushion; B, right cavity of bulb; C, 
aortic left posterior part of bulb; D, left bulbar cushion; E, interventricular 
septum; F, posterior and G, anterior endocardial cushions of auriculo-ventricu¬ 
lar canal; H, mitral and J, tricuspid orifices; K, right and L, left ventricles; 
M, aortico-pulmonary septum with valve cushions. Arrows show directions 
of arterial and venous blood which will be divided later by fusion of right 
and left bulbar cushions (A and D) with right end of endocardial cushion G 
to left of tricuspid orifice. (Reproduced from Sir Arthur Keith’s Schorstein 
Lecture, Lancet, Lond., Dec. 20, 1924, Figs. 1 and 16.) 


4 


PLATE II. COMPARATIVE ANATOMY. FISH AND AMPHIBIAN HEART 



2 



a 


2 



b 



c 

Fig. 3. 


Fig. 1. 


Fig. 2. 

































































PLATE III 

COMPARATIVE ANATOMY ( Continued ). REPTILIAN HEART 

A distinctive characteristic of the reptilian heart is the presence, in all orders, of a third arterial trunk, the reptilian right aorta, which, 
arising to the right of, and in close juxtaposition to, the abdominal viscera and lower extremities, coalesces some distance below the heart 
with a second large aorta, which transmits arterialized blood to the head and upper extremities (Fig. 4). The three vessels, each of which 
is supplied with a bicuspid semilunar valve, thus arise together from the base of the heart, encased usually in a tough fibrous sheath, and 
undergo at their origin a pronounced clockwise torsion from right to left upon each other, which is significant of the spiral arrangement of 
embryonic swellings in the primitive bulbus cordis, from fusion of which their three interarterial septa have been derived. The period of 
development at which these three arterial trunks are present together in the human embryonic heart is of extremely brief duration. That 
this stage does exist in it, however, is evident from the observation of embryologists and from the configuration of the normal right ven¬ 
tricle and also from the structure of various cardiac anomalies in which traces are plainly visible, fixed at the point of arrest. A compara¬ 
tive study of the successive stages in the evolution of these reptilian hearts as revealed in the turtle, python and crocodile throws a flood 
of light upon the complex processes that take place at the base of the organ in the sixth to eighth week of embryonic life, and conversely 
reveals, often with startling simplicity, the ontogenesis of many grave defects that may otherwise baffle analysis. 

The first impetus to the study of these important interrelationships of cardiac defects as revealed in the comparative embryology of the vertebrate heart 
was given by the work of Roese ( Morphol. Jahrb., 1890, 16: 2) and that of Greil on the development of the heart of Lacerta agilis (l.c. PI. I, Fig. 3). There fol¬ 
lowed the fundamental contributions of Sir Arthur Keith, whose intensive studies upon persistence of the reptilian bulbus as the main etiological factor in pul¬ 
monary stenosis and other grave cardiac anomalies cast an entirely new light on the pathogenesis of these conditions (J. Anat., Lond., 1905, 19: 14; Studies in 
Pathology, Aberdeen, 1906; Hunterian Lectures, Lancet, Lond., 1909, 2 : 359, 433, 579; Schorstein Lecture, ibid., 1924, 1: 1267). There followed the valuable work 
of Jane Robertson upon the bulbar region of the fish and its bearing upon cardiac anomalies (l.c. PI. II) and more recently Spitzer’s brilliant theory (PL IV) of 
the part taken by delayed torsion with persistence of the “right” reptilian aorta in the causation of these conditions. 

Acknowledgment is made of the kind assistance of Prof. J. S. Baxter in the revision of the descriptive text for this and the preceding plates (I and II) and 
in the preparation of the specimens shown in Figs. 2 and 3 opposite. 


Fig. 1.—Order Chelonia. Heart of snapping turtle (Chelonia mydax).* 
a, ventral; b, dorsal surface. A three-chambered heart having two com¬ 
pletely divided auricles, the right much larger than the left, which pouch 
forward above the large flattened common ventricle. The latter is incom¬ 
pletely divided dorso-ventrally by the bulbo-ventricular ridge ( B.V.R.) into 
a smaller ventral chamber, giving off the pulmonary artery, and a large dorsal 
chamber, which gives off the right aorta posteriorly from its extreme upper 
right-hand corner and, just in front of this, the left systemic aorta. All 
three vessels are united at their base by a firm fibrous outer coat and each 
has a bicuspid valve. The dorsal ventricle is itself incompletely divided into 
shallow right and left cavities by an obliquely placed apical septum derived 
from the loose muscular trabeculae of its lower wall. That on its right side, 
known as the cavum venosum, receives the venous blood from the right 
auricle, while that on the left, the cavum arteriosum, receives the oxygenated 
blood from the left auricle but gives off no vessel. The auricular septum is 
entire and ends below in two large fleshy valvular cusps which act like paddles, 
propelling the venous blood from the cavum venosum into the pulmonary 
artery and left aorta, and the arterialized blood from the cavum arteriosum 
across the interventricular communication (I.V.F.) into the right aorta to be 
distributed to the vessels of the head and neck. This arrangement corre¬ 
sponds to that of a human embryonic heart about the end of fifth week. 
A.C., anterior communication (bulbar septal defect); Au.V., auriculo-ven- 
tricular cushions; R.A., position of right auricle (removed in specimen); 
G.C., gubernaculum cordis. 

Fig. 2.—Diagram of reptilian heart. The dorsal ventricle is extended to 
right of ventral ventricle. Adapted from Walmsley ( Quain’s Anatomy, 1929, 
IV, III, p. 9). B.V.R., bulbo-ventricular ridge; R.V., ventral ventricle; 

I.V.F., interventricular foramen; C.Art. and C.Ven., cavum arteriosum and 
cavum venosum of dorsal ventricle; P.A., pulmonary artery from ventral 
ventricle; right aorta from cavum venosum; left aorta from both ventral 
ventricle and cavum venosum of dorsal ventricle above bulbar septal defect 
( A.C.). (Diagram by A. C. Cheney, Medical Art Department, McGill 
University.) 

Fig. 3.—Order Ophidia. Heart of water-snake (Matrix sitedon) in situ 
to show arrangement of great vessels.* The auricles have been pulled back 
to expose the origin of the three arterial trunks which emerge from the right 
upper angle of the ventricle as a single truncus. This is seen on close inspec¬ 
tion to have two components twisted upon each other: the aortic stem, which 
divides after 0.75 cm. into the two systemic aortae, and the pulmonary artery, 
which arises anteriorly' and to the left of this and passes directly backward, 
emerging on its right side posteriorly and curving downward to reach the lungs 
in the bdomen. The two aortae curve upward on either side and then 
downward, the left supplying the abdominal viscera, and the right, which lies 
posteriorly, giving off the large branches to the head and upper extremities 
(right aortic arch). The two trunks unite some 3 cm. below the heart into 
a single trunk (double aortic arch). The right superior 6S.F.C.) and inferior 
caval veins empty posteriorly into either end of the sinus venosus (<S.F.), 
and the large left superior cava ( Lt.S.V.C.) into the coronary sinus ( C.S .). 

* Specimen in the Cardiac Anomaly Collection of McGill University. 


(Drawing by A. G. Cheney from specimen No. 11393 presented by Prof. J. 
S. Baxter). 

Fig. 4.—The same. Heart of python (P. molurus).* a, ventral, b, dorsal 
aspect. The heart shows a distinct advance on that of the turtle in that 
torsion of the great trunks is increased and the apical septum dividing the 
dorsal ventricle into its two cava (see Fig. 2) is considerably higher and the 
interventricular foramen (I.V.F.) smaller, while the bulbo-ventricular ridge 
is likewise more developed, forming a distinct floor for the ventral ventricle 
and reducing the bulbar septal defect to a linear slit (AC). The effect is 
that of a heart with three ventricles and two completely divided auricles. In 
the lower part of the floor of the ventral ventricle is a small round communica¬ 
tion (PC) in its trabeculae with the cavum arteriosum of the dorsal ventricle, 
permitting of an arterial-venous or venous-arterial shunt between these two 
chambers. The two large valvular flaps attached on either border of the 
lower end of the auricular septum perform their paddle-like action, sending 
the venous blood from the cavum venosum toward the pulmonary artery and 
left aorta (L.Ao.) and thereafter the arterialized blood from the cavum arteri¬ 
osum through the interventricular foramen (I.V.F.) into the right aorta, 
(R.Ao). R.AV and L.AV, right and left venous ostia, SVC, superior, IVC, 
inferior vena cava. (Drawing by W. W. Beattie and E. Shanly.) 

Fig. 5.—Order Crocodilia. Heart of crocodile (Alligator mississippiensis).* 

This organ is of great interest because it reveals the final stages in closure of 
the communications that exist between the chambers in the turtle and python 
heart and thus sheds light upon the completion of the mammalian interven¬ 
tricular septum. This has been accomplished by the growth downward of 
a muscular tongue of tissue between the right and left aortae (lower border 
of aortic septum) and the further torsion of the great trunks and bulbar por¬ 
tion of the heart upon each other so that this process meets and finally fuses 
with the left border of the right auriculo-ventricular orifice. Thus the right 
aorta is placed in the now completely separated cavum arteriosum of the 
dorsal ventricle, while the pulmonary artery and left aorta remain in the 
ventral ventricle, which has now come to contain also the cavum venosum of 
the dorsal chamber. The only' communication between the two circulations 
that remains is a secondary aperture, the fora men Panizzae, which has devel¬ 
oped in the contiguous sinuses of Valsalva of the right and left aortae where 
these cross each other and which permits arterialized blood from the right 
aorta to flow at intervals into the left aortic (venous) trunk. The spiral 
twisting that has taken place in the separation of the ventricles is plainly 
visible in the convex floor of the ventral ventricle and in the twisted relations 
of the great trunks. (From a specimen in the collection of the late Prof. 
G. S. Huntington. Reproduced from the article by M. E. Abbott in Con¬ 
tributions to Medical and Biological Research, dedicated to Sir William Osier, 
1919, Fig. 5, Paul B. Hoeber, New York.) 

Fig. 6.—The aortic arches and their transformations (after Rathke). 1. 
Ceratodus. 2. Salamander. 3. Triton. 4. Frog. 5. Lizard. 6. Bird. 7. 
Mammal. (Republished by permission from the article by H. A. Harris, 
J. Anat., Lond., 1922, 57: Part I, 85.) 


6 


PLATE III. COMPARATIVE ANATOMY ( Continued ). THE REPTILIAN HEART 


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Apical Septum 



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Fig. 4. 











































































PLATE IV 


INCOMPLETE TORSION IN THE CAUSATION OF CARDIAC DEFECTS (SPITZER’S THEORY) 


In 1875, Carl von Rokitansky published his epoch-making work on the pathogenesis of cardiac septal defects. Based on an exact 
morphological study of his own great collection carried out in the light of the then new science of embryology, the facts elicited by him 
and his brilliant deductions therefrom may be said to have unlocked the door for the more exact scientific investigation of this great 
subject and definitely placed the origin of these obscure conditions in the very early weeks of embryonic life. The curious manner 
in which the graver cardiac anomalies mirror the hearts of the lower vertebrate orders had been commented upon ever since the time of 
Meckel (1812) and the correlation of these in recent years with their phylogenetic prototypes in the stage of embryonic life at which arrest 
had occurred has greatly clarified our understanding of these obscure conditions. The fundamental contribution of Sir Arthur Keith upon 
the persistence of the reptilian bulbus in the causation of pulmonary stenosis and allied anomalies ( l.c. PI. II and III) marks an important 
milestone on the line of advance in this respect and since then other workers in the same field of anatomical research have added their 
quota to our knowledge. 

It has remained, however, for Prof. Alexander Spitzer of Vienna, upon whom the mantle of Rokitansky’s genius seems to have fallen 
in our own generation, to originate a teleological theory of startling simplicity, based upon the indisputable evidence of vestigial remains 
in the right ventricle which link the human heart with the reptilian in a common ancestry. This theory has revolutionized many of our 
ideas on the ontogenesis of these defects and has supplied the most satisfactory explanation as yet available of those bizarre conditions of 
transposed great vessels and inverted ventricles that have seemed to contradict every known principle of development or evolution. 
Briefly described, it ascribes such anomalies to the arrest or delay of the clockwise torsion that normally takes place in the growth of the 
primitive embryonic heart between its fixed arterial and venous ends during the process of septation, thus leading to an apparently counter¬ 
clockwise shunting of the parts with resultant reopening of the channel of the reptilian right aorta and obliteration of the left ventricular 
vessel. Anatomical proof of the latter curious fact is supplied by the confirmation of the right ventricle in which the trabecula septomar- 
ginalis or moderator band is homologous (as observed by Tandler) with the septum between the right aorta and pulmonary artery in the 
reptilian heart; that portion of the ventricle lying between the “anterior tricuspid ledge” of the latter and the crista supraventricularis 
being identified as the “outflow channel” of the “right reptilian aorta,” which is closed in the normal human heart by the clockwise tor¬ 
sion of the bulbo-ventricular end of the primitive cardiac tube. An additional very suggestive point is seen in the persistence of the 
bicuspid pulmonary valve (normal in reptiles) in the developmental form of pulmonary stenosis. Further explanation of this very intrigu¬ 
ing and suggestive theory is to be sought in Prof. Spitzer’s own meticulously detailed and beautifully worked out monograph (reference 
under Fig. 3) or may be gathered from the writer’s summary ( Nelson’s Loose-leaf Med., 1932, 2 : 214-218), where the figures opposite also 
appear. 


Fig. 1.—Spitzer’s diagrams showing relationship of the semilunar cusps, 
bulbar swellings and septa in the reptilian and normal human heart, and in 
a malformed human heart with bicuspid pulmonary valve. 

A. Reptilian heart. Here there are three trunks, the primary septum 
aortico-pulmonale ( S.ao.p.I ) and the septum aorticum ( S.ao .) together form¬ 
ing the septum between the pulmonary artery and left aorta, but dividing 
anteriorly to enclose the right aorta ( rt.ao .). Each vessel has only two cusps 
as the primary septum aortico-pulmonale does not reach the valvular part 
of the bulbar swelling. 

B. Normal human heart. The septum aorticum has united throughout 
with the primary septum aortico-pulmonale to form the secondary septum 
aortico-pulmonale. The right aorta has disappeared. Only two great vessels 
exist and each has three cusps. 

C. Malformed heart with pulmonary stenosis and bicuspid pulmonary 
valve. The aortic septum has disappeared and the left aorta has become 
obliterated or fused with the right aorta. Only one large vessel (the 
right reptilian aorta) exists on the right anteriorly. The pulmonary 
orifice is smaller and has only two cusps because the primary aortico¬ 
pulmonary septum does not meet the valvular part of the bulbar swelling 
(III). 

I, II, III, IV, distal bulbar swellings; A, B, C, proximal bulbar swellings 
extended into bulbar region; Iv., IIv., IIIv., IVv., valvular parts of distal 
bulbar region; Is., I Is., septal parts of the distal bulbar region; S.Vt., septum 
ventriculorum; P, pulmonary artery. 

Fig. 2.—Heart in a case of persistence of the right ventricular aorta 
(Spitzer’s Type II) with rudimentary interventricular septum (cor biatriatum 
triloculare) and stenosed and bicuspid pulmonary valve. (Note the cleft ( N ) 
marking the orifice of the rudimentary conus of the obliterated left ventricular 
aorta.) C, the hypertrophied crista supraventricularis (aortico-pulmonary 
septum); S, upper border of rudimentary interventricular septum which 
separates the stenosed pulmonary ostium anteriorly from the deep niche or 
deft (N), which is the rudimentary conus of the obliterated left ventricular 
aort a. Behind and to the left of this niche is the aortic segment of the mitral 
valve. T, trabecula septomarginalis. 

From a boy aged 5 with extreme cyanosis and clubbing. (Reported by 
Mautner and Lowy under title “Transposition der Aorta oder Persistenz der 
rechtskammerigcn Aorta,” Virch. Arch., 1931, 229: 337.) 

Fig. 3. Diagrams showing division of the ventricles and relative positions 
of the arterial ostia in the normal heart and in the different types of trans¬ 
position of the great trunks as conceived in Spitzer’s phylogenetic theory of 
persistent right reptilian aorta and formation of a septum spurium in the right 

ventricle. 


a. Normal human heart. A.P., pulmonary artery; Ao., normal (left 
ventricular) aorta; Ao. rt., obliterated conus of right aorta; Mi. and Trie., 
mitral and tricuspid valves; va., vp. and vm., anterior, posterior and median 
tricuspid cusps; cr., crista supraventricularis; as. and s.p., anterior and pos¬ 
terior part of interventricular septum. 

b. T3'pe I of transposition: “ reitende ” aorta (fusion of both aortic trunks). 

c. Type II, “simple” transposition (aorta from right ventricle). 

d. Type III, “crossed” transposition, aorta arising from the right and the 
pulmonary from the left ventricle. 

e. Type IV, “mixed” transposition, the pulmonary artery and both 
auriculo-ventricular ostia in a common chamber and aorta arising transposed 
from small right ventricle. a.Tr.r., anterior tricuspid ridge; A.Sr. and 
p.S.r., anterior and posterior septal ridges; a.P.Mi. and P.P.Mi., anterior 
and posterior papillary muscles of mitral valve; p.p.Tr., posterior papillary 
muscle of tricuspid. (From A. L. Spitzer, “t)ber den Bauplan des normalen 
und missbildeten Herzens,” Virch. Arch., 1923, 243: 81-272. Diagrams 
adapted by J. Fleury.) 

Fig. 4.—Diagrams illustrating a case of “crossed” transposition (Spitzer’s 
Type III, aorta in right ventricle, pulmonary artery in left ventricle), showing 
plan of base of ventricles seen from above (after Spitzer). 

A. In the normal mammalian heart. S.vt., septum ventriculorum; Cr. + 
Tsm., crista supraventricularis + trabecula septomarginalis; Co.rt.Ao., blind 
ending of rudimentary conus of right ventricular aorta; Mi., Tri., mitral and 
tricuspid orifices. 

B. In the anomalous heart described showing transposed aorta. Ao 

[; rt.Ao ], the transposed (right reptilian) aorta; Cr., the markedly hypertrophied 
crista aortico-pulmonaris (supraventricularis), forming spurious ventricular 
septum; a.S.v.t., the atrophic true anterior interventricular septum; and 
Co.lt.Ao, blind ending of the left ventricular aorta; a.T.l. anterior tricuspid 
ledge. (Bj r P. Freudenthal, Virch. Arch., 1927-28, 226: 640.) 

Fig. 5.—Heart showing niche of the obliterated right aorta in normal right 
ventricle. This ventricle laid open to show its interior which is of normal 
configuration except that the crista supraventricularis is heavily developed, 
thus bringing into prominence a deep crevice tying posteriorly between it and 
the infundibular tricuspid segment. This is the niche and outflow tract of 
the obliterated right aorta. 

From an infant dying three hours after birth. The ventricular septum 
was entire and the foramen ovale and ductus arteriosus were widely patent, 
the pulmonary artery forming the descending aorta through the latter channel. 
The heart lay entirety in the right thorax. Multiple somatic anomalies. 
(From a specimen in the possession of L. Minor Blackford, Atlanta, Ga., 
kindly supplied for illustration of this point in this Atlas. Drawing by 
A. C. Cheney, Medical Art Department, McGill University.) 


8 


PLATE IV. INCOMPLETE TORSION IN THE CAUSATION OF CARDIAC DEFECTS (SPITZER’S THEORY) 



Fig. 3e. 
9 


Fig. 3c. 


Fig. 5. 

































































































' 

. . 











PART II 

CLINICAL CLASSIFICATION OF CONGENITAL CARDIAC DISEASE 


Group II. 
Group III. 


Group I. No Abnormal Communication (Acyanotic Group) 

Cases of Arterial-Venous Shunt with Terminal Reversal of Flow (Cyanose Tardive) 
Cases of Permanent Venous-Arterial Shunt and Retardation of Flow (Cyanotic Group) 


PLATE V 


THE CLINICAL CLASSIFICATION OF CARDIAC DEFECTS. ILLUSTRATIVE DIAGRAMS 

The clinical significance of cardiac anomalies depends upon the pathological physiology of the altered circulation as induced by the 
defect. This may be of the nature of a simple mechanical obstruction which leads directly to undue strain at exposed points, but in which 
there is no abnormal communication between the systemic and pulmonary circulations and therefore no cause for cyanosis is present (Group 
I; Fig. 1). Or, secondly, an anomalous aperture may exist in the form of a localized, uncomplicated defect in the cardiac or aortic septa, 
through which, so long as the pressure remains physiologically higher on the left or systemic side, oxygenated blood passes into the pul¬ 
monary circulation. An arterial-venous shunt exists, until pathological conditions supervene, raising the pressure on the right side of the 
defect; then the direction of the shunt will be reversed and venous blood will flow into the arterial stream ( cyanose tardive ) (Group II; 
Fig. 2). Thirdly, if such a localized defect be complicated by associated anomalies which permanently raise the pressure on the venous 
side; or if one or more of the cardio-vascular septa be entirely absent; or if the great trunks arise transposed from reversed ventricles (Figs. 
3a, b, c), a permanent venous-arterial shunt will result with development of capillary changes and a persistent morbus coeruleus. In addition, 
a small group must be recognized in which, without any abnormal communication between the two circulations, a mechanical obstruction 
to the return of venous blood to the right heart exists, leading to retardation of flow in the capillaries, and persistent cyanosis from capillary 
stasis and increased deoxygenation at the periphery (Group III (6) below). 

On the basis of the above considerations the following classification has been formulated of cardiac anomalies of clinical significance. 

I. A cyanotic group. Cases in which no abnormal communication exists between the two circulations but in which the anomaly is liable to become the seat 
of strain. 

II. Cyanose tardive group. Cases of arterial-venous shunt with possible transient or terminal reversal of flow. 

III. Cyanotic group, (a) Cases of permanent arterial-venous shunt, with resultant capillary changes, (6) Cases of simple retardation of flow, (right-sided 
valvular lesions with all foetal passages closed). PI. XVII. 


Acknowledgment. The large series of diagrams illustrating the course of the circulation in the different individual defects which are 
used as key features heading Plates XIII to XV (Group II) and Plates XVIII to XXIV (Group III) were drawn by Garnet Jex of the 
Medical Art Department of the Army Medical Museum, Washington, D. C., for illustration of the article by M. E. Abbott and W. T. 
Dawson on “The Clinical Classification of Congenital Cardiac Disease” which appeared in International Clinics, 1924, IV, Ser. 34: 155; 
and the figures opposite were drawn by P. Lariviere of the Medical Art Department of McGill University after the diagrams by Louis 
Gross, and appeared in the writer’s article on this subject, published, ibid., 1934, III, Ser. 44 : 15. The writer’s thanks are expressed to 
both sources and to the J. B. Lippincott Company, publishers, for kind permission to reproduce these very useful diagrams here. Figures 
la, lc and 2b are reproduced from the writer’s monograph in Osier’s Modern Medicine, 3d ed., by permission of Lea and Febiger, 
publisher. 

For additional references on this subject see the writer’s articles in Osier’s Mod. Med., 1927, 4: 653-657; Lancet, Lond., July 27, 1929; Brit. Med. J., Dec. 31, 
1932; and Nelson’s Loose-leaf Med., 1932, 4: 226-229. Also D. C. Muir and J. W. Brown, “Congenital heart disease,” Brit. Med. J., 1935, 1: 966; and “Clinical 


Classification of Congenital Cardiovascular Disease,” in Heart Disease by Paul 

Fig. 1.—Diagram showing the course of the circulation under normal con¬ 
ditions and in cardiac anomalies in which there is no abnormal communication 

between the two circulations, with examples of defects which fall under this 
category (Group I). 

a. Right aortic arch with total suppression of left.* The ligamentum 
arteriosum crosses behind the trachea and oesophagus to be attached to the 
right arch, thus encircling these viscera in a vascular ring, a condition which is 
liable to give rise to pressure symptoms of dysphagia and dyspnoea. From 
a woman aged 61, dying of intestinal obstruction. (Drawing by Prof. J. G. 
Adami of specimen No. 15.11. 1 Reported by M. E. Abbott in Osier’s Mod. 
Med., 1927, 4: 791, Fig. 93. Reprinted on Plate VI, Fig. 6.) 

b. The diagram illustrating this group. P.V.R., pulmonary venous reser¬ 
voir; S.V.R., systemic venous reservoir; S.V.C., superior vena cava; I.V.C., 
inferior vena cava; C., conus of right ventricle; A., aorta and aortic vestibule; 
P.A., pulmonary artery; R.A., R.V., right auricle and ventricle; L.A., L.V., 
left auricle and ventricle. 

c. Anomalous origin of left coronary from pulmonary artery.* Heart 
showing cirsoid dilatations and sacculation of both coronaries, due to the 
blood entering the anastomotic branches of each under different pressures and 
producing the effect of an arterio-venous aneurysm. A, vascular loop the 
size of a crabapple just beyond the origin of the right coronary, giving off its 
descending branches; B , dilated transverse branch of the same; C, circumflex 
-nd D, descending branches of the left coronary. 

From a woman aged 60 who died accidentally. The heart was hyper¬ 
trophied and the seat of replacement fibrosis and fatty changes. (Drawing 
' Prof. J. G. Adami from specimen No. 15.145. 1 Reported by M. E. Abbott, 
r id., 795, Fig. 94.)' 

Fig, 2.--Diagrams of the circulation in cases of localized uncomplicated 
cardiac septal defect leading to an arterial-venous shunt with reversal of 

Specimt in the Cardiac Anomaly Collection of McGill University. 

(Continued 


. White, 1931, 310-313, The Macmillan Company, New York. 

flow. With example of a defect falling under this category (Group II 

Lettering as in Fig. 16 (cf. PI. XIII-XV). 

a. Circulation under physiological conditions in which the pressure is 
normally higher in the left ventricle causing the shunt to pass from left to 
right through the defect as occurs in compensated septal defects. 

b. Heart showing a large defect at the lower part of the interauricular 
septum (persistent ostium primum) illustrating this type of anomaly. Asso¬ 
ciated with double mitral orifice, cleavage of the anterior segment of the mitral 
valve, patent foramen ovale and bifid apex. Accidental findings in a child 
of 5 years. (Reported by M. E. Abbott, ibid., 759, Fig. 88.) 

c. Circulation when pathological conditions supervene, causing a relative 
rise of pressure in the right chambers or in the pulmonary circulation, pro¬ 
ducing a reversal of flow from right to left through the defect (indicated by 
dotted arrow) and mixture of venous blood with the arterial stream (cyanose 
tardive). 

Fig. 3.—Diagrams of the circulation in cases of permanent venous- 
arterial shunt (congenital cyanosis), Group III. A black arrow is passed 
through the patent foramen ovale. P.D.A., patent ductus. Other lettering 
as in Fig. 16. 

a. Circulation in the tetralogy of Fallot (defect at base of interventricular 
septum with dextroposition of aorta and pulmonary stenosis), in which 
venous blood passes from right to left through the defect and the pressure is 
also raised in the systemic capillaries (cf. PI. XIX). 

b. In complete absence of the interventricular septum (cor triloculare biatri- 
atum) showing free admixture of venous and arterial streams in the common 
ventricle (cf. PI. XXI). 

c. In complete transposition of great trunks with ventricular septum closed, 
foramen ovale and ductus arteriosus patent. Note that the sole pathway for 
the aerated blood from the lungs is through the foramen ovale into the trans¬ 
posed aorta (cf. PL XXIII). 

on page 14.) 


12 


PLATE V. CLINICAL CLASSIFICATION OF CARDIAC DEFECTS. ILLUSTRATIVE DIAGRAMS 




Fig. 1.—Group I. 


i 




P.V.R. 




PV.R. 


P.V.R 



Fig. 3.—Group III. 


13 









































































PLATE V ( Continued) 

DETAILS OF FOREGOING CLASSIFICATION 


A. Cardiac Defects of Clinical Significance 

Note: In the list below the figures given showing relative incidence and duration of life in the various defects are drawn from the writer’s chart of 1000 
cases analyzed (see pp. 60-61), the total number of cases of the individual lesion being taken from the last column, and the highest and average age, etc., in the 
number of cases listed as the primary lesion from the first three columns. 


I. A cyanotic group. Left-sided lesions with no abnormal communication 
between the two circulations. Such are: pericardial defect, 31 cases (average 
age in 28 cases classed as primary lesion 42 years, highest 75); congenital 
so-called idiopathic hypertrophy, 17 cases (average in 15 cases 4 months, 
highest 4 years); congenital heart block, 4 cases (average in 3 cases 7 years, 
highest 20); anomalous septa in auricles, 30 cases (average in 12 cases 22% 
years, highest 58); congenital aortic and mitral stenosis, 34 cases (average in 
17 cases 3 years, highest 27); bicuspid aortic valve, 78 cases (average in 32 
cases 33 years, highest 68); bicuspid pulmonary valve, 31 cases (age in 1 case 
classed as primary lesion 20); supernumerary aortic cusps, 4 cases (average 
in 2 cases 36 years, highest 41); supernumerary pulmonary cusps, 12 cases 
(average in 8 cases 36 years, highest 80); double mitral orifice, 10 cases (aver¬ 
age in 8 cases 37 years, highest 71); pulmonary dilatation, 9 cases (average in 
6 cases 14 years, highest 59); coarctation of aorta of adult type, 105 cases 
(average in 70 cases 33 years, highest 92—Reynaud’s case); hypoplasia of 
aorta (average in 2 cases listed 19 years); double and right aortic arch, 40 cases 
(average in 19 cases 32% years, highest 87); left coronary from pulmonary 
artery, 10 cases (average in 8 cases 36 years, highest 61); congenital arterio¬ 
venous aneurysm, 8 cases (average in 6 cases 21 years, highest 35) congenital 
rhabdomyoma, 17 cases (highest age 20 years). 

II. Cases of arterial-venous shunt with possible transient or terminal reversal 

of flow (cyanose tardive ). (1) Patent ductus arteriosus, 242 cases (average in 
92 cases 24 years, highest 66); Paul D. White’s case. (2) Persistent patency 
of foramen ovale, 290 cases (average in 40 cases 29 years, highest 70); defect 
at upper part of interauricular septum, 19 cases (average in 10 cases 34 years, 
highest 64); defect in the lower part of the interauricular septum, 36 cases 
(average in 18 cases 19 years, highest 46); premature closure of foramen ovale, 
4 cases (average in 3 cases 18 hours). (3) Localized defects of the inter¬ 
ventricular septum, maladie de Roger , 257 cases (average in 50 cases 14% 
years, highest 49); elsewhere or multiple, 28 cases (average in 5 cases 23 years, 
highest 79—Weiss’s case); aneurysm of pars membranacea, 16 cases (average 
in 7 cases 42 years, highest 60). (4) Defects of aortic septum: congenital 

aneurysm of aortic sinus of Valsalva (average in 12 cases 28 years, highest 53); 
communication between aorta and pulmonary artery (average in 10 cases 14 
years, highest 48). 

III. Cyanotic group. Lesions or combinations of lesions arranged below 
in the order of increasing depth of cyanosis and lessened duration of life. 

(a) Right-sided valvular lesions with foetal passages closed and in which there 
are stasis and increased deoxygenation in the capillaries, leading to moderate 
cyanosis with clubbing of late appearance: pulmonary stenosis with closed 


septa, 11 cases (average in 9 cases 10% years, highest 45—Hebb’s case); 
congenital tricuspid stenosis with closed septa, average age in 2 cases listed 
22 years. 

(b) Cases of permanent venous-arterial shunt with resultant capillary changes. 
Cardiovascular septal defects complicated by right-sided valvular lesions, 
complete absence of cardiac or arterial septa, “complete” or “partial” trans¬ 
position of great trunks, isolated congenital dextrocardia. 

Moderate cyanosis. Pulmonary stenosis with closed ventricular septum 
but patent foramen ovale, 20 cases (average in 16 cases 18 years, highest 
57—case by Lallemand); tricuspid atresia with defective auricular septum 
and transposition of great trunks (one case only, recorded by Hedinger in 
1915, lived 56 years); dextroposition of aorta with defect at base of ventricular 
septum without pulmonary stenosis, 121 cases (average in 10 cases 13 years, 
highest 45 years); complete absence of interventricular septum (cor biatriatum 
triloculare), 27 cases (highest age 35—case by Mann); common auriculo- 
ventricular ostium, 14 cases (average in 10 cases 1% months, highest 4% 
years). 

Marked cyanosis. Pulmonary stenosis with defect of interventricular 
septum, 20 cases (average in 85 cases 14% years, highest 59 years 9 months— 
White’s musician); pulmonary atresia with defect of interventricular septum, 
49 cases (average in 30 cases 6% years, highest 30—Bach’s case); persistent 
truncus, complete defect of aortic septum, 34 cases (average in 21 cases 
4% years, highest 25—Zimmerman’s case); complete transposition of great 
arterial trunks with defect of interventricular septum, 36 cases (average age 
in 16 other cases 1% years, highest 16—case by Keith); cor biloculare with 
transposition of great trunks (1 case was reported by Rudolf, aged 16 years; 
(vide infra). 

Extreme cyanosis. Morbus coeruleus from birth. Transposition of great 
trunks with closed ventricular septum, ductus arteriosus and foramen ovale 
patent, 38 cases (average age in 31 other cases was 1% months, highest age 
reached was 11 years—case of Emanuel); pulmonary atresia with closed inter¬ 
ventricular septum, ductus arteriosus and foramen ovale patent (highest age 
in a patient reported by Costa was 20 years, average age in 9 others 2% 
months); tricuspid atresia, 25 cases (Hedinger’s case with transposition 
reached 56, average age of 15 other cases 5 months); cor biloculare, 14 cases 
(1 case by Wood and Williams attained 15 years and 1 by Rudolf with trans¬ 
position 16 years, average age in 7 other cases 5% weeks); mitral atresia, 13 
cases (highest age 3% years—Blackmore, average in 4 other cases 5 weeks); 
aortic atresia, 14 cases (average in 12 cases 4 days, highest 15 weeks—-case 
by Simmons). 


B. Cases of No Clinical Significance 

Diverticulum of pericardium, bifid apex of heart, diverticulum of heart, acardia and ectopia cordis pectoralis (as being non-viable), true (mirror-picture) 
dextrocardia in complete situs inversus are conditions that come under this heading. Such cases are not figured here. 


GENERAL REFERENCES 

Abbott, Maude E., “Congenital cardiac disease,” monograph in Osier and McCrae’s System of Modern Medicine , 1st ed., 1908, 4: 323-425; 2d ed., 1915, 4: 
323-448; 3d ed., 1927, 4: 612-812. Also: “The treatment of congenital cardiac disease,” in Blumer-Billings-Forschheimer’s System of Therapeutics, D. 
Appleton Company, 1924, 302-362. Also: “The diagnosis of congenital cardiac disease” in Blumer’s Bedside Diagnosis, W. B. Saunders Company, 
1928, 2 : 357-574. Also: “Congenital heart disease,” in Nelson’s Loose-leaf Medicine, Thomas Nelson & Sons, 1932, 4, 207-321. 

Fleury, J., “Les affections congenitales, in Encyclopedic Medico-chirurgicale, 1934, 12. 

Herzheimer, G., Missbildungen des Herzens und der Gefasse, in Schwalbe’s Missbildungen, 1910, Part III, 339. 

Humphry, L., “Congenital diseases of the heart,” in Allbutt’s System of Medicine, 1909, 6, 276. 

I.aubry, C., and C. Pezzi, Traite des maladies congenitales du coeur, J. B. Bailliere et fils, Paris, 1921. 

Monckeberg-Bonn, J. G., “Die Missbildungen des Herzens,” Henke u. Lubarsch Handbuch, 1924, II. 

Muir D. C. and J. W. Brown, Congenital Heart Disease, Brit. Med. J., 1935, 1, 1966. 

Osier, W., “Congenital affections of the heart,” in Keating’s Cyclop. Dis Child., 1889, 2, 747. 

Peacock, T. B., On Malformations of the Human Heart with Original Cases, London, 1858; 2d ed., 1866. 

Theremin, Etudes congenitales du coeur, St. Petersburg, 1895. 

Thomson, J., “Congenital heart disease,” Oxford Medicine, 1920, 2, 371. 

Thorel, In Lubarsch-Ostertag’s Ergebnisse, 1903, Ch. II, p. 585; 1910, Ch. I, p. 585; 1910, Ch. II, p. 268. 

Yierordt, H., “ Die augeborenen Herzkrankheiten,” in Nothnagel’s System, 1898, 16, 244. 

White, Paul D., On cardiac anomalies in Chap. XII of his Heart Disease, The MacMillan Co., 2nd ed. 1936. 


14 


GROUP I 


NO ABNORMAL COMMUNICATION 
(ACYANOTIC GROUP) 


PLATE VI 


ANOMALIES OF THE AORTIC ARCH AND ITS BRANCHES 

The evanescent character of the six pairs of embryonic arches, which replace each other in rapid transition as the heart ascends in the 
thorax, supplies the basis for a well-recognized series of anomalies in which arrest has occurred at a point that is commonly mirrored in 
the hearts of the lower orders of vertebrates (see PI. Ill, Fig. 6 and Congdon, Amer. J. Anat., 1926, 37 : 499). The only embryonic arches 
that persist in their entirety in the normal human heart are the fourth left, which becomes the definitive aorta, and the third pair (carotid), 
while the right fourth arch is normally suppressed in part, its cephalic portion only persisting as the innominate. Persistence may occur of 
both fourth arches in their entire length, constituting a double aortic arch in which (a) both trunks may be of full caliber as in Figs. 4 and 5 
opposite, or (b) the left member of the pair may be stenosed or obliterated usually in the region of the foetal isthmus as in Arkin’s case (Fig. 2). 
Again, a right aortic arch may occur (a) with total suppression of the left (Fig. 6) or ( b ) the caudal or distal portion of the latter may, and 
usually does, coexist ( right aortic arch with persistent left root). Finally, if suppression of the right or left fourth arch has occurred before 
the ascent of the subclavians, one of the latter will arise from the descending arch or thoracic aorta, the right subclavian from a persistent 
right root (Fig. 8), or the left subclavian from a left root or from the ductus or pulmonary artery (Fig. 7). The clinical significance of all 
these cases, except the anomalous left subclavian, lies in the fact that a vascular ring is formed encircling and sometimes compressing the 
trachea and oesophagus with resultant dysphagia and dyspnoea. A correct diagnosis can usually be made by barium-x-ray investigation 
(Figs. 1, 3, 4). Anomalous origin of the right pulmonary from the innominate (Fig. 9) is a rare condition, explained by the author of the 
case reported below (Fig. 9) as a substitution of the right fifth arch. 

In this connection see the valuable illustrated monograph by Williams Evans of the Cardiac Department of the London Hospital entitled “The 
Course of the Oesophagus in Health and in Disease of the Heart and Great Vessels,” just published under the British Medical Research Council, Special 
Report Ser. No. 208, London, 1936. 


Fig. 1.—Roentgenogram after barium meal in antero-posterior diameter 
from a case of right aortic arch. The course of the oesophagus is visualized 
and has been outlined in white and shows a characteristic sharp curve with 
convexity to the left and behind this on the right a retro-oesophageal shadow 
of the right aorta. From a boy, aged 10, with multiple associated anomalies. 
(Case of L. M. Blackford, Amer. J. Dis. Child., 1932, 44: 823). 

Fig. 2.-—Double aortic arch with atresia of left isthmus. The aorta arises 
by a broad base and arches up to the right and posteriorly giving off a large 
left innominate and thereafter the right carotid and subclavian. The left 
subclavian is attached near its origin by a short fibrous cord (obliterated 
isthmus) to a diverticulum which projects toward it from the descending arch 
(left aortic root), the whole forming a vascular ring encircling the trachea and 
oesophagus. A long ligamentum arteriosum attached the atresic isthmus to 
the left pulmonary artery. From a man aged 50, dying of periarteritis nodosa. 
Diagnosis made before death by x-ray which showed in right oblique diameter 
the right aortic knob behind the trachea and within this the round shadow 
of the left aortic root. (From the article by A. Arkin, Wien. Arch. f. inn. 
Med., 1926, 12: 385, Case 1, PL XVI, Fig. 1.) 

Fig. 3.—Orthodiagraph of right aortic arch after barium meal in right 
oblique diameter. Note curve forward of oesophagus and right aortic knob 
behind this, which are diagnostic, tra., trachea; oes., oesophagus; ao. k., 
aortic knob. (Arkin’s Case 2, ibid., PI. XIV, Fig. 2.) 

Fig. 4.—Antero-posterior roentgenogram after barium meal in a case of 
double aortic arch with total persistence of left arch. Note that the aortic 
shadow at the level of the third and fourth dorsal vertebrae is uniformly 
constricted on all sides and is not deflected to the left as in simple right aortic 
arch (cf. Fig. 1) or in double aortic arch with atresic isthmus (Arkin’s Case 1), 
a diagnostic point. Female, aged 5 months with noisy inspiration and respira¬ 
tory distress, death from bronchopneumonia. (From the article by C. E. 
Snelling and I. H. Erb, Arch. Dis. Child., 1933, 8: 401.) 

Fig. 5.—Double aortic arch with total persistence of left arch which is of 
good caliber and completely pervious. Classic type. The ascending aorta, 
which is wider than normal, arches upward and backward on the right and 
splits at its convexity into two transverse branches of which the larger lies 
above and posteriorly and gives off the right subclavian and carotid while 
the smaller lies in front and anteriorly and gives off the two left trunks, the 
two arches enclosing an ovoid cleft 1.6 by 3 cm. long, beyond which they 
unite to form the large descending left arch 8 cm. in circumference. The 
trachea and oesophagus lie in the cleft and are flattened but not constricted. 
An accidental finding in a Greek man, aged 45, who died of gangrene of the 
lungs following removal of the tongue. (By Hamdi, Deutsch. med. Woch., 
1906, 32: 1410.) 

Fig. 6.—Right aortic arch with total suppression of left. The aorta is a 
large trunk 8 cm. in circumference, which gives off the left innominate from 
its anterior surface and curves upward and directly backward, descending on 


the right of the vertebral column to the abdomen. Just beyond the origin 
of the right subclavian on its left wall is a tent-shaped diverticulum attached 
to a long ligamentum arteriosum which passes to it from the left branch of 
the pulmonary artery behind the trachea and oesophagus, thus enclosing these 
viscera in a vascular ring. Accidental finding in a woman aged 61 years, 
dying from intestinal obstruction. (Diagrammatic sketch by Prof. J. G. 
Adami from specimen No. 15.11 1 in the McGill Museum, Reported by M. E. 
Abbott, Osier’s Mod. Med., 1927, 4: 791.) 

Fig. 7.—Right aortic arch with persistent left aortic root supplying branches 
to both lungs in a case of pulmonary atresia. The large aorta 3.5 cm. in diam¬ 
eter rises above a defect in the interventricular septum and after giving off 
the left innominate and right branches arches down behind the right bronchus. 
Just opposite the left subclavian it gives off a flaring thin-walled tube, the 
dilated patent ductus, which communicates with a branch of the atresic 
pulmonary artery and empties into the hilum of the right lung. 2.5 cm. below 
this there rises from the left wall of the thoracic aorta a thick-walled left 
aortic root which passes up to the left, giving off two branches to the right 
and then to the left lung and apparently ending as a left subclavian artery. 
a, right aortic arch; b, patent ductus; c, varicose pulmonary end of b; left 
common carotid artery; e, anomalous left subclavian;/, trachea; g, pulmonary 
branch to right lung; h, oesophagus. From a man aged 29 with cyanosis, 
and clubbing, the result of the associated anomalies. (Case of Digby Wheeler, 
and M. E. Abbott, Canad. Med. Assn. J., 1928, 19: 297.) 

Fig. 8.— Origin of right subclavian artery from descending arch of aorta, 
a, anterior; b, posterior view. The anomalous vessel originating from the 
posterior aspect of the aorta 5 mm. to the right of and below the left sub¬ 
clavian (6), ascended obliquely to the left crossing behind the oesophagus and 
trachea, which it pressed upon but did not constrict. An accidental finding 
in a woman aged 36, dying of pulmonary tuberculosis. (By Vera Dolgopol, 
J. Tech. Meth. 1934, 13: 112.) 

Fig. 9.—Absence of right pulmonary artery and replacement by anomalous 
branch from innominate and bleeding into right lung. Heart and lung of a 

well-developed somewhat cyanotic infant aged 6 days. The main pulmonary 
trunk continues directly into the descending aorta through the widely patent 
ductus and gives off, just before this, the left pulmonary artery to the lung 
of that side. The right pulmonary is replaced by a large vessel which passes 
from the innominate artery to the hilum of the right lung. This organ is 
cyanotic, cystic and microscopically shows extensive haemorrhage into the 
alveoli, due probably to the high systemic pressure acting upon the pulmonary 
capillaries in the congenital absence of the right pulmonary artery. The 
anomaly is explained as a too ‘early involution of the right sixth arch and 
the substitution in the fourth embryonic week of an anomalous artery from 
the right fifth arch, a, pulmonary trunk; b, ascending aorta; c, left pulmonary 
artery; d, patent ductus arteriosus; e, aortic arch; /, innominate artery; g, 
anomalous right pulmonary artery from innominate to right lung. (Reported 
by G. Ambrus, J. Tech. Meth., 1936, 5: 103.) 


16 


PLATE VI. ANOMALIES OF THE AORTIC ARCH AND ITS BRANCHES 



Fig. 6. Fig. 7. Fig. 9c. 


17 

































PLATE VII 


COARCTATION OF THE AORTA OF THE ADULT TYPE. HISTORIC CASES 

CULLED FROM THE LITERATURE TO ILLUSTRATE THE PATHOLOGY OF THIS LESION 

General Considerations. In this condition there is a more or less abrupt narrowing, constriction, or obliteration of the descending 
arch in the region of the insertion of the (usually obliterated) ductus arteriosus, with resultant formation of an extensive collateral circu¬ 
lation between the branches given off from the aorta above and those below the seat of coarctation. The aortic intercostal arteries play an 
important part in the development of these anastomotic channels and, by the continuous pulsation of their tortuous dilated channels against 
the adjacent compact bone, produce extensive excavations in the borders of the ribs, a conspicuous pathological change that was first described 
and figured by Meckel in 1827 (Fig. 36) and which, when present, is plainly visible by the x-rays (PI. VIII, Fig. 9). The ascending aorta 
is usually dilated and the aortic valve is frequently bicuspid. A dramatic termination by rupture of the aorta (Fig. 6), cerebral haemor¬ 
rhage or other sudden lethal event (Fig. 5) is not uncommon; or life may be prolonged to an advanced age even in cases of extreme con¬ 
striction, in the presence of a highly developed collateral circulation—as witness Reynaud’s patient, a man who lived without apparent 
inconvenience to the age of 92 (Fig. 1). There is a curious predominance in the male sex (147 out of 200 cases analyzed, i.e., 70 per cent), 
and the subjects are usually highly developed individuals, of more than average intelligence and of great bodily vigor. 

The extensive earlier literature was reviewed and illustrative cases published by J. T. King (Arch. Int. Med,., 1926, 38: 69); W. F. Hamilton and M. E. 
Abbott ( Anier. Heart J., 1928, 3: 381, 514); and L. M. Blackford (Arch. Int. Med., 1928, 41: 702). Rare instances of stricture of the aorta some distance below 
the ductus in the thoracic or abdominal aorta have been reported, by Schlesinger (Wchnsch.f. d. ges. Heilk., 1835, p. 489); Hasler (Leipzig Thesis, 1911); Costa 
(Arch. d. Pat. e. Clin. Med., 1929-30, 9: 305) and W. H. Maycock of Montreal (in press). Attention is also drawn to the valuable recent contributions by H. 
Roesler (Wien. Arch. f. inn. Med., 1928, 15: 521) and William Evans (Quart. J. Med., 1933, 16: 205); and to the highly illuminating article by Sir Thomas Lewis 
(Heart, 1933, 16: 205), in which complete blood vascular studies and electrocardiographic and x-ray tracings are presented for nine cases of coarctation, in eight 
of whom the diagnosis was made during life. See also the report of May G. Wilson (with diagnostic roentgenograms, Amer. J. Dis. Child., 1932, 44: 390); and 
that of W. E. Pierce (J.A.M.A., 1934, 103: 838, (mycotic aneurysm at seat of coarctation). 


Fig. 1.—The collateral circulation in a case of extreme coarctation of the 
descending arch just below the ductus in a man dying at the age of 92. Note 
the anastomosis of the hugely dilated three upper aortic intercostals with the 
superior intercostal artery and the dilated and tortuous internal mammaries 
with the deep epigastrics. (From A. Reynaud, “Observation d’une obliter¬ 
ation presque complete de l’aorte, etc.,” J. hebd. de med., 1828, 1: 161.) 

Fig. 2.—Drawings from the injected cadaver showing details of the col¬ 
lateral circulation in v a case of atresia of the descending arch two lines 
below the obliterated ductus in a man aged 21, a butcher by trade, who 
died suddenly from rupture of a dissecting aneurysm of the dilated ascending 
aorta. 

a. Dissection of the arm and shoulder from this case showing the dilated 
and tortuous subscapular branch of the brachial artery emerging below the 
angle of the scapula to pierce the intercostal space posteriorly on its way to 
join the upper aortic intercostals. 

b. The dilated epigastric arteries coursing down interiorly on either side 
of the sternum to anastomose with the internal mammaries. (From J. 
Jordan, “A case of obliteration of the aorta with disease of the valves,” North 
of Eng. M. and S. J., 1930, 1: 101.) 

Fig. 3.—Meckel’s case of extreme coarctation of the descending arch admit¬ 
ting only a straw with enormous development of the collateral circulation 
in a Bernoise peasant, aged 29, who died six days after carrying a heavy bag 
of corn to market, from rupture of the right auricle into the pericardium. 

a. Outline drawing of the aortic arch showing gradual narrowing of the 
isthmus with marked constriction just below the insertion of the ligamentum 
arteriosum and dilatation of the ascending aorta. 

b. Dissection of the interior of the thoracic cavity to show the extraordi¬ 
narily dilated and tortuous collaterals, especially the transverse cervicals and 
suprascapular branches of the subclavian artery and the anastomosis of the 
latter with the hugely dilated and tortuous intercostals and the excavations 
made in the ribs by these, as pointed out by this author. (From A. Meckel, 
“ Verschliessung der Aorta am vierten Brustwirbel,” Arch. f. Anat. u. Phys., 
D 27, 345.) 


Fig. 4.—A sudden constriction as though by a ligature just below the 
obliterated ductus, corresponding internally to a circular diaphragm 1 mm. 
thick with a central opening admitting a fine sound. The aorta expanded 
widely below the stricture, the ascending aorta, innominate and left subclavian 
also much dilated. Persistent left superior cava. In a farmer, age 27 years, 
dying of failing compensation. (From L. M. Bonnet, “Sur la lesion dite 
stenose congenitale de 1’aorte dans la region de l’isthme,” Rev. de med., Paris, 
1903, 23: 108.) 

This article summarized all the important cases to date and presented a 
valuable review of the earlier literature. 

Fig. 5.—Complete obliteration of the descending arch half an inch in length, 

beginning just below the insertion of the obliterated ductus with huge col¬ 
lateral circulation, in an Austrian officer, who had fought through the French 
Revolution and had suffered for the last year from dyspnoea, dysphagia and 
oedema of the extremities and died suddenly. (From A. Roemer, “Eine 
vollstandige Verschliessung der Aorta bei einem 50-jahrigen Manne,” Med. 
Jahrb. d. k. k. Oesterr. Staates, 1839, 29: 208.) 

Fig. 6.—Extreme stenosis of the descending arch just below the insertion 
of the obliterated ductus with congenitally bicuspid aortic valve in a beautiful 

girl of 17, who had been apparently quite well except for occasional precordial 
distress and who became very ill with pain and loss of consciousness after rising 
suddenly in the night and walking across a cold room in bare feet to attend a 
sick relative, and died suddenly 8 hours later. Autopsy showed the ascending 
aorta greatly dilated and provided with only two cusps and presented on its 
left wall a jagged tear about 1 in. long beginning in. behind the left coronary 
orifice and running obliquely upward, through which rupture into the peri¬ 
cardium had taken place. (From A. W. Otto, “Eine Zerreissung der Aorta, 
wegen stellenweiser grosser Verengerung derselben,” in Seltene Beobachtungen 
zur Anat. Phys. u. Path., 1824, p. 66.) 

Fig. 7.—Extreme stenosis of the aorta at the level of the ductus which 
barely admitted a fine probe. The first aortic intercostals are seen dilated 
as are also the vessels of the arch. A beautifully depicted case. (From 
J. Cruveilhier, “Retrecissement par froncement de l’aorte sans alteration des 
parois,” Anat. path, du corps hurnain , 1835-42, 43, Fig. 3.) 




18 


PLATE VII. COARCTATION OF THE AORTA OF THE ADULT TYPE. HISTORIC CASES 





Fig. 2a. 


Fig. 3b. 





Fig. 5. 



Fig. 6. 






Fig. 2b. 


Fig. 4. 

19 


Fig. 7. 


A S C t> 


A$(q 

























PLATE VIII 


COARCTATION OF THE AORTA {Continued). PERSONAL CASES AND CLINICAL FEATURES 

General Considerations. The interference with the main part of the circulation by the constriction in the descending arch and the 
consequent dilatation of the collaterals above the obstruction give rise to a series of vascular signs and of x-ray findings that are of patho¬ 
gnomonic value. Such are the hypertension that invariably exists in the upper part of the body along with reduction of pressure in the 
lower extremities, the diminution or absence of the femoral or popliteal pulse to the palpating finger and the relatively slow rise of the latter 
in the sphygmographic tracing (Figs, la and b ); the disappearance over the upper part of the thorax and neck of visible pulsations in the 
tortuous and dilated collaterals, which often transmit also a fine thrill and systolic or postsystolic murmur; the erosion of the ribs (see 
Fig. 3), which is visible by x-rays in all cases in which it has occurred (Roesler’s sign); and other roentgenological features, such as 
the peculiar contour of the left border of the cardiac shadow, the widened right base and the complete absence in the left oblique diameter 
of the descending arch (Fig. 6). Multiple capillary aneurysms seen in two carefully investigated cases were reported by Roesler as of 
diagnostic value {Wien. Arch. f. inn. Med., 1928, 15: 521). 


Fig. 1.—Complete atresia of the descending arch with large collateral cir¬ 
culation, saccular aneurysm of ascending arch with impending rupture, con¬ 
genitally bicuspid aortic valve and aortic insufficiency. Cerebral death.* 

a. Anterior view, showing the enormously dilated and hypertrophied left 
ventricle laid open to reveal its interior, the sclerosed and incompetent 
bicuspid aortic valve, the dilatation of the ascending aorta, and the displace¬ 
ment of the great trunks to the extreme left of the elongated transverse arch. 

a, the narrow aortic isthmus giving off an anomalous vessel; b, the area of 
complete obliteration at the level of c, the ligamentum arteriosum; d, interior 
of pulmonary artery; e, enlarged periarterial gland. 

b. Posterior view of aortic arch and interior of dissective aneurysm. Just 
beyond the great trunks the aortic isthmus narrows rapidly to a ring of further 
constriction which coincides internally with a complete obliteration, the aorta 
above and below this point ending blindly in a shallow biconcave disc lined 
with shining healthy intima, indicating arrest of the fourth left arch at its 
junction with the sixth. The aorta below this area widens rapidly and pre¬ 
sents the dilated orifices of the upper aortic intercostals. 

From a boy of high intelligence, aged 14, who presented the characteristic 
clinical picture of coarctation with huge development of the collateral circu¬ 
lation, and died in coma setting in 12 hours before death. The brain was not 
examined, but cerebral haemorrhage was undoubtedly the cause. (Reported 
by W. F. Hamilton and M. E. Abbott, Amer. Heart J., 1928, 3: 381; 574. 
Drawings by H. Blackstock, Medical Art Department, McGill University.) 

Fig. 2.-—Coarctation of the aorta with double stricture of descending arch, 
bicuspid aortic valve, aortic, mitral and tricuspid stenosis and aortic and mitral 
insufficiency.* Sudden death. 

A. Inner and outer views of the transverse and descending arch. Just 
beyond the left subclavian it has become abruptly narrowed (upper stricture) 
and then widens to undergo again at the level of the obliterated ductus a 
second very sharp kinking which corresponds internally to a thickened intimal 
ridge or shelf, enclosing a very narrow lumen. The aorta between these two 
points is ballooned out and twisted on itself. Below the second stricture it 
widens rapidly and attains after receiving the collateral supply through the 
dilated aortic intercostals a circumference of 56 mm. 

B. Heart showing a marked simple hypertrophy of the left ventricle with 
dilatation and hypertrophy of both auricles, especially the right. The 
bicuspid aortic valve presents a low raphe behind the combined coronary seg¬ 
ments and both segments are greatly thickened and manifestly incompetent. 

From a primipara aged 34, who presented signs of cardiac insufficiency 
during her pregnancy and was successfully delivered by cesarian section in 
the eighth month of a viable baby, but died quietly in her sleep six nights later. 
(Report from the Pathological Service of the Royal Victoria Hospital, kindly 
supplied by W. H. Chase. Published by M. E. Abbott, Libman Ann. Vol., 
1932, 1: 1. Drawing by H. Blackstock, Medical Art Department, McGill 
University.) 

Fig. 3.—Roentgenograph in antero-posterior diameter of the heart and 
aortic arch from the case in Fig. 1, showing great broadening and con¬ 
vexity to right of the aortic shadow, and the characteristic contour of the left 
border with absence of aortic knob and kinking in the region of the aortic 
ist hmus, also erosion of the ribs. (From Amer. Heart J., 1928, 3 : 385, Fig. 1.) 

Fig. 4.—The dilated and tortuous internal mammary arteries as they pass 
down to anastomose with the deep epigastrium in the upkeep of the collateral 
circulation. {Ibid., p. 389, Fig. 4.) 

Fig. 5.—Vascular signs. Markings in heavy charcoal on the back of a 
living case of coarctation, indicating the position of forcible pulsations in the 


dilated and tortuous branches of the dorsalis scapulae, supra- and subscapular 
arteries. 

From a carpenter aged 58, with hypertension in the upper extremities, 
diminished femoral pulse and large collateral circulation, diagnosed during 
life from these features. (Republished by permission from the article by 
J. T. King, Arch. Int. Med., 1926, 38: 99.) 

Fig. 6.—-Roentgenograph in the left oblique diameter in Roesler’s case of 
coarctation in a man of 45, who died suddenly in his sleep. The picture shows 
the ascending aorta dilated and passing backward toward the vertebral column 
at what appears to be a somewhat higher level than usual. In the retroaortic 
and retrocardiac space below it, the bifurcation of the trachea and one bron¬ 
chus are clearly visible anteriorly, but behind these the area that is normally 
crossed by the descending arch is occupied by a clear space, the shadow of 
the descending arch being absent in this situation. (From H. Roesler, Wien. 
Arch. f. inn. Med., 1928, 16: 520, Fig. 3.) 

Fig. 7.—Polygraphic tracings from the radial and femoral arteries and 
collaterals of the back, in Roesler’s Case 2 of coarctation. 

a. The femoral curve is much lower than in the radial and arises slowly and 
falls gradually, producing an elevation with an obtuse flattened top, and the 
rise begins a little later than that of the radial. 

b. The curve in the collaterals is still shallower but the rise begins synchron¬ 
ously with that in the radial pulse. (From H. Roesler, ibid. , Fig. 5.) 

Fig. 8.—-Polygraphic tracings from the brachial and femoral arteries in a 

patient diagnosed clinically as coarctation. Note the difference in form, the 
slow up-stroke and low amplitude of the femoral as compared with the radial 
tracing. (From Laubry and Pezzi, Traite des maladies congenitales du coeur, 
1921, p. 263, Fig. 94.) 

Fig. 9.—Roentgenogram of thorax, showing marked erosion of the fourth 
to eighth ribs on both sides, in an unpublished case of coarctation, under the 
care of Douglas Wilkinson of Birmingham, England. The patient was a 
young man of 21, well built and powerful, a football and cricket player. 
Routine physical examination revealed a systolic pressure of 280 degrees with 
a diastolic so low that it could not be made out satisfactorily and no pulse 
palpable in the iliacs or abdominal aorta. Heart action was forcible but the 
organ was hardly enlarged. The diagnosis was made on the above character¬ 
istic findings. (From the copy kindly supplied by Dr. Wilkinson for this 
Atlas.) 

Fig. 10.—Dr. Libman’s case of mycotic aneurysm of the thoracic aorta just 
below the seat of coarctation with rupture into the left bronchus. 

From a girl of 12, who died with symptoms of bacteraemia of one month’s 
duration. Autopsy showed abundant streptococcus haemolyticus in the walls 
and thrombotic contents of the aneurysm, with subacute glomerulonephritis 
and embolism of femoral artery. (Reported by permission by M. E. Abbott, 
Blumer’s Bedside Diag., 1928, 3 : 376, Fig. 258. Drawing by A. Feinberg.) 

Fig. 11.—Ruptured cerebral aneurysm thought to be congenital. This 

patient was an apparently healthy young man of 24, who fell on the street in 
an epileptiform convulsion and was brought into hospital in coma and died 
a few hours later. The base of the brain was enveloped in abundant 
haemorrhage, and dissection showed this small thin-walled sac at the second 
bifurcation of the anterior cerebral artery torn across on its free surface. 
Microscopic examination showed intimal proliferation and some medial degen¬ 
eration. There was no coarctation but the case illustrates a not infrequent 
complication of this. (From C. P. Symonds, “Spontaneous subarachnoid 
haemorrhage,” Quart. J. Med., 1924-25, 18: 118, Case 2.) 


* Specimen in the Cardiac Anomaly Collection of McGill University. 


20 


PLATE VIII. COARCTATION OF THE AORTA (i Continued ). PERSONAL CASES AND CLINICAL FEATURES 




Secondary Sacculation 
beneath upper bof( 1 (r of 
ariCuryim.iAMlh ditsuAon 
Of Atilcriof wall. (d’J 


E 

Duct Art*r(o>ua 
C. - - 


Fnaispicl Aortic 
Valve. 


Dil.it ed Ascending Areh 
of Aoi-ta - 


f£--G. 



Descending ’IKoi-aew Aorta. 

A.Anomalous o^tery from dwuudtng arch to Hyperplastic glands. 

B . 3l'enosed descending Jnn i f Aorta . C, Sift of OmctXtUin. I). Raphe behind Juyeci Culp. 

E .Dilated left COYonavv d'rftry. F. Ituvd Intercostal Artery. G. Coronary Arl'criesfverysmall). 


A. COARCTATION Of THE AORTA WITH DOUBLE STRICTURE AT 
AND ABOVE ENTRANCE OF OBLITERATED DUCTUS; 

B. HEART FROM SAME CASE SHOWING BICUSPID AORTIC VALVE 
WITH LOW CALCIFIED RAPHE BEHINO COMBINED CORONARY SEG¬ 
MENTS AORTIC INSUFFICIENCY TRICUSPID AND MITRAL STEN¬ 
OSIS. CASE OF DR CHASE. 

female AGE 3 4 REF R.v.H 37^32 MUSEJM NO I039& M<GILL 


Fig. la. 


Fig. lb. 


Fig. 2. 



Fig. 3. 



Fig. 4. 



Fig. 5. 



Fig. 6. 



Fig. 10. 



Fig. 8. 



Fig. 11. 


Fig. 9 


21 

























PLATE IX 


A. BICUSPID AORTIC VALVE. B. SUPERNUMERARY AORTIC CUSPS 

A. Congenitally bicuspid aortic valve is a relatively rare condition which does not of itself produce signs or symptoms. Nevertheless 
its presence is of serious clinical significance on account of the fact that it almost invariably undergoes sclerotic changes and becomes the 
seat of a subacute bacterial endocarditis usually with lethal results (Fig. 8); or the ascending aorta immediately above the valve may 
undergo dilatation with dissection of its coats and secondary rupture into the pericardium (Figs. 6, 9, 10). It is important therefore to 
differentiate between a congenital union and a postnatal inflammatory fusion. The macroscopic features distinctive of a congenital origin 
were first pointed out by Sir William Osier in 1880 and again in 1886 in a careful morphological and statistical study of 16 personal cases; 
the microscopic criteria however, which alone can furnish positive evidences in doubtful cases, were first established by Sir T. Lewis and 
R. T. Grant (1923) in their brilliant histological investigation of the architecture of (a) the normal and ( b ) the conjoint segments. In 
it they show that the normal reversal of the annulus fibrosus with the elastica of the aortic media which takes place at this area is absent 
in the abortive commissure, the annulus remaining submerged beneath the elastica which may pass over superficially in an unbroken layer, 
or undergoes a confused whorling. Similar observations on the structure of the commissure were recorded by Gross and Kugel ( Amer. J. 
Path., 1931, 7: 445) and recently by Bishop and Trubek (Fig. 5). 


Fig. 1.—Diagram by Lewis and Grant of the structures supporting the 
normal aortic valve. Serial reconstruction made from vertical sections taken 
through the entire breadth of the aortic lumen. The stippling represents the 
aortic media, the vertical lines the annulus fibrosus, A.F.; the heavy black 
line, F.V., indicates the fibrous layer of the valves; D.E. the deep and S.E. 
the superficial endings of the elastica of the media; M.S., membranous sep¬ 
tum; M.V., mitral valve; R.A., P. } and L.A., the right anterior, posterior, and 
left anterior cusps; A , B, and C, the commissures formed at the junction of the 
right and left anterior, right anterior and posterior, and at junction of posterior 
and left anterior cusps; R.C. and L.C., the two coronary arteries. Note that 
here the normal reversal has occurred, the annulus emerging from below and 
passing superficially to the deep elastica over the top of each commissure. 

Fig. 2.—Diagram of the structures supporting a congenitally bicuspid 
aortic valve with no trace of raphe. Serial reconstruction by these authors 
from a specimen in the Museum of University College. Lettering as in Fig. 1. 
Note that at the site of the abortive commissure the annulus fibrosus lies deep 
to the elastica, the latter sweeping superficially over the floor of the sinus 
behind the composite cusp and curving over the slight elevation produced by 
the (displaced) membranous septum. (From article by T. Lewis and R. T. 
Grant, Heart, 1923, 10: 23, 35, Figs. 1 and 2.) 

Fig. 3.—Peacock’s case of congenital bicuspid aortic valve with union of 
right anterior and posterior cusps (commissure B) and partial fusion of 
anterior segments. The aortic arch laid open to show the deformed aortic 
valve which is irregularly thickened and the sinus behind the conjoint cusp 
unequally divided by a prominent raphe. The ascending aorta is dilated 
and the descending arch is narrowed in the isthmus region but undergoes a 
bulbous dilatation after entrance of the patent ductus. Female infant, aged 
10 weeks. (Specimen in the Museum of University College, described and 
figured by Thomas B. Peacock in his Malformations of the Human Heart, 
2d ed., 1866, p. 152, PI. viii, Fig. 1. Congenital origin confirmed by Lewis 
and Grant Fig. 4 below.) 

Fig. 4.—Microphotograph of serial section taken by Lewis and Grant 
through the abortive commissure in Peacock’s case of bicuspid aortic valve 
demonstrating its congenital origin. The main ridge (r) is seen rising above 
a central space which is the normal projection upward of the ventricular 
cavity below the commissure and is lined by endocardium containing a strong 
layer of elastic tissue ( V.e ., ventricular elastica). Externally to this the 
fibrous layer of the valve is continuous with the annulus fibrosus ( an.f .), and 
the whole ridge is covered on its aortic surface with a thin layer of elastic 
tissue (S.e.) which is continuous on either side with the aortic media, and is 
itself in part covered superficially by a layer of foetal connective tissue repre¬ 
senting a much thickened subendothelial layer (F.s.). “These observations 
stamp the deformity as a congenital malformation, for they clearly show that the 
two cusps have been laid down in one sheet without subdivision.” (From the 
article by Lewis and Grant, ibid., pp. 32-33, Fig. 8.) 

Fig. 5a.—Microphotograph of a serial transverse section through the raphe 
of a bicuspid aortic valve due to inflammatory fusion, showing normal com¬ 
missural relationships. Note that the connective tissue of the annulus passes 
superficially to the aortic media to form the apex of the commissure, while 
the deep layers of the elastica pass uninterrupted below the annulus, the 
normal reversal existing. From a man aged 45 with advanced aortic stenosis 
with calcification of cusps, dying from congestive failure. 

Fig. 5b.—Transverse serial section through raphe of a congenitally bicuspid 
aortic valve showing lack of normal reversal. A layer of connective tissue (the 
abortive annulus) appears below the whorled elastic fibers of the media which 
lie superficially. Male, aged 26. Coronary cusps fused, raphe almost 
obliterated. Accidental finding in death from bullet wound. (From L. F. 
Bishop and M. Trubek, J. Tech. Meth., 1936, 16: 123, Fig. V, and 119, 
Fig. Ill C.) 

* Specimen in the Cardiac Anomaly Collection of McGill University. 


Fig. 6.—Bicuspid aortic valve with fusion of right anterior and posterior 
segments (commissure B) and huge saccular aneurysm of right anterior sinus 
of Valsalva eroding sternum and rupturing externally. Atheroma and dilata¬ 
tion of ascending aorta, aortic insufficiency.* (Reported by M. E. Abbott, 
Libman Ann. Vol., 1932, 1: 23.) 

Fig. 7.—Four of Osier’s 16 cases of bicuspid aortic valve believed to be 
congenital, showing a raphe of varying height behind the conjoint segment 
and aortic incompetency, a. Fusion of coronary segments with low almost 
obliterated raphe. The conjoint segment is defective at its right posterior 
end from ulcerative endocarditis and is here anchored to the aortic wall by a 
tendinous cord. From a blacksmith, aged 26, dying from cardiac dropsy. 

b. Fusion of coronary cusps with high raphe, the conjoint shorter than the 
single segment. Chronic infective endocarditis with calcification of vegeta¬ 
tions and multiple valvular aneurysms. From a man aged 45, dying with 
symptoms of severe aortic valvular disease.* 

c. Fusion of coronary cusps with low raphe, the fused shorter than the single 
segment. Ulcerative endocarditis with perforation of single segment. From 
a young man, aged 20, who presented symptoms of acute endocarditis but 
died suddenly of ruptured cerebral aneurysm (? congenital).* 

d. Fusion of anterior segments with low raphe and V-shaped deficiency in 
the conjoint curtain. The single cusp longer than the conjoint segment. 
From a man aged 42, dying of cardiac dropsy after 5 months’ illness. (Reported 
by William Osier with four other cases in the Montreal General Hosp. Repts. 
Clin, and Path., 1880, 1: 233, PL IX. Author’s cases I, V, II, and IV 
respectively. Republished by him with eight additional cases, Tr. Amer. 
Assn. Phys., 1886, 1: 185.) 

Fig. 8.—Congenitally bicuspid aortic valve with fusion of coronary cusps 
and almost obliterated raphe, insufficiency of segments, and infective endo¬ 
carditis and mycotic aneurysm of circumflex branch of left coronary artery 
rupturing into wall of left auricle with extensive myocardial infarction of left 
ventricle.* Associated subaortal septal defect, hypoplasia and coarctation 
of aorta. From a tall spare man, aged 34, who presented a musical systolic 
murmur (generated at the defect) but was in good health until the last year. 
Symptoms of bacterial endocarditis two months and repeated anginal attacks 
of increasing severity, in one of which he died. Blood culture was negative, 
but abundant streptococci in the vegetations and in wall of aneurysm. (From 
a case in the service of D. S. Lewis. Reported by M. E. Abbott and W. H. 
Chase, J. Tech. Meth., 1929, 12: 171.) 

Fig. 9.—Bicuspid aortic valve with calcified raphe behind fused (coronary) 
cusps and great elongation of single segment, dilatation and dissecting aneu- 
ryms of ascending aorta rupturing into pericardium. The aortic valve is 
calcified and insufficient and the heart in a state of marked eccentric hyper¬ 
trophy. The right coronary orifice is displaced high above the aortic ring.* 
(Reported by M. E. Abbott, Libman Ann. Vol., ibid., Case 2, p. 21, Fig. VI.) 

B. Supernumerary Aortic Cusps 

Fig. 10.—Fourth aortic cusp of irregular triangular shape interposed 
between the two coronary segments and united with the left of these by a 
low raphe. Saccular aneurysm of the sinus of Valsalva behind this combined 
segment with dissection of wall and impending rupture. Syphilitic mesa- 
ortitis with aortic sclerosis and insufficiency.* (Reported by M. E. Abbott, 
ibid., Case 4, p. 29, Fig. IX.) 

Fig. 11.—Anomalous aortic valve. Fourth semilunar cusp fused with the 
left anterior one, the sinus behind these being incompletely divided by a 
high raphe. The right anterior and posterior cusps show multiple fenestra¬ 
tions. No other anomalies or disease of the endocardium. From a man 
aged 21 who died of miliary tuberculosis. (Unpublished case of L. F. Bishop, 
Jr.) 


22 


PLATE IX. A. BICUSPID AORTIC VALVE. B. SUPERNUMERARY AORTIC CUSPS 






Atheromatous and calcareous wall of Aortic arch. 

False Aneurysm in ant. Mediastimum 
adherent To Sternum. 


pulmonary 


Thin wall of 
Conus Arteri 
os us. 

fti^ht Ventricle 


Fig. 1. 


Raphe 

L. Coronary A. 


L. Auricle. 


Mitral 

valve. 


Fig. 3. 



Fig. 2. 


d 

Fig. 7. 


Fig. 5. 


Fig. 6. 




Aneurysm of the 
Sinus of 
Valsalva . 


Aorta 


Calcified Raphe 
behind combined 
Anterior Cusps. 


Fig. 8. 


Fig. 9. 



Fig. 11. 


23 














































































































































































































PLATE X 


OTHER ANOMALIES OF THE ENDOCARDIUM 

In contrast to the similar lesion at the aortic orifice, a bicuspid pulmonary valve is rare as an isolated anomaly but is relatively common 
in association with other defects, being a practically constant feature of the developmental type of pulmonary stenosis in the combination 
known as the “tetralogy of Fallot” (see PL IV and XIV). Supernumerary pulmonary cusps, on the other hand, four or even five in num¬ 
ber, occur more frequently here than at the aortic orifice, but are not usually the seat of inflammatory changes so are not of much clinical 
import. Of the conditions figured opposite, subaortic stenosis (Fig. 1) does not in itself interfere with cardiac function but is nevertheless 
of serious significance on account of the fact that it is liable to become the seat of a subacute infective process as illustrated in the classic 
cases of Goldenweizer and Wiglesworth (in press). It can usually be readily differentiated from stenosis at the aortic orifice proper by the 
absence of any systemic evidence of aortic obstruction in the presence of distinctive physical signs at this area. Aberrant chordae tendineae 
(Fig. 2) are likewise of interest from the diagnostic standpoint on account of the confusing signs they are liable to produce but are unim¬ 
portant otherwise. Double mitral orifice, a rare condition usually occurring with cleavage of the anterior mitral segment (Fig. 4), is appar¬ 
ently always latent, an accidental finding at autopsy. The same applies to anomalous network in the auricles (Fig. 3), although this has 
sometimes led to a lethal termination through becoming the seat of origin of embolism from thrombi lodged in their meshes. Congenital 
tricuspid insufficiency, on the other hand, is an extremely serious condition terminating in early infancy with generalized cyanosis. This 
properly belongs with the cyanotic group (III). The two chief anatomical types recognized are shown in Figs. 5a, b, c, and 6. Congenital 
aortic and mitral stenosis of inflammatory origin are rare left-sided lesions not shown in this Atlas. 

Important additional references on the conditions figured opposite are the following:_On subaortic stenosis: Thursfield and Scott, Brit. J. Child. Dis., 1913, 
10: 104; Goldenweizer, Med. Oborz. Mosk., 1912, 87 : 319; C. Sternberg, Verh. d. deutsch. path. Gesell., 1930, 25: 238. Aberrant chordae in ventricles: H. Huchard, 
Rev. de med., 1895, 13, 113; Goforth, J.A.M.A., 1926, 86 : 1612; Costa, Clin. Med. Jtal., 1930, 56: 572. Anomalous network in right atrium: Chiari, Zieg. Beitr., 
1897, 22: 1; W. W. Yater, Arch. Path., 1929, 7: 418; Helwig, Amer. J. Path., 1932, 8 : 73. 


Fig. 1.—Subaortic stenosis. In the rare and interesting condition here 
shown, the aortic ostium was not narrowed and its valves were completely 
competent, but a short distance below these a thick fibrous ridge, 2 cm. wide 
by 1.5 cm. high, projected into the cavity of the ventricle, traversing the base 
of the anterior mitral segment and forming a complete ring lining its interior. 
The left ventricle was hypertrophied, but the heart was otherwise normal. 
Young man aged 19 in good health since childhood. Admitted in second week 
of typhoid fever and died some months later of purulent pleurisy. Physical 
examination showed a systolic thrill in the third right interspace and a very 
long loud humming systolic murmur heard at all ostia but maximum over the 
upper sternum. Under observation in hospital the systolic vibration over the 
aortic area and great vessels became stronger and a short diastolic murmur 
developed at the apex. Coarctation was erroneously diagnosed. (Reported 
by J. H. Lindman, Deutsch. Arch. f. klin. Med., 1880, 25: 510, PI. VI.) 

Fig. 2.—Aberrant chordae tendineae in left ventricle producing a loud 
musical murmur audible 15 feet away from the patient. A cor bovinum of 
aortic insufficiency laid open to show the interior of the right ventricle, and 
an anomalous chorda, which, arising from one of the papillary muscles of the 
mitral valve, was attached high up on the ventricular surface of its aortic 
segment half an inch below the aortic cusp. Two other fine tendinous cords 
traversed the ventricle and joined the first at right angles, while a second 
passed to be attached to the ventricular wall close to the point of insertion 
of the first. From a laborer aged 40 years, who presented, in addition to the 
classic signs of aortic incompetency, a prolonged diastolic thrill over the third 
and fourth left interspaces, and a loud musical diastolic murmur maximum at 
the same area but plainly heard over the whole thorax and 15 to 24 feet from 
the chest wall. These signs diminished with the onset of congestive failure 
a few weeks before death. (Reported by W. F. Hamilton, Montreal Med.J., 
July, 1899.) 

Fig. 3.—Anomalous chordae (“network of Chiari”) in right auricle. A 

system of fine tendinous threads attached to the lower border of the auricular 
septum, to the apex and body of the auricular surface of the tricuspid segment 
and to the right wall of the auricle just below the Eustachian valve. Probably 
vestigial remains of the valvula venosa dextra. (From F. C. Helwig, Amer. 
J. Path., 1932, 8: 73.) 

Fig. 4.—Double mitral orifice. An enlarged heart laid open to show the 
auricular surface of the mitral valve. Its anterior segment is occupied about 
its middle by a perfectly formed secondary mitral orifice, formed by an ovoid 
cleft 2 cm. long, supplied with two delicate cusps which were attached by 
short but perfectly formed chordae to a group of papillary muscles situated 
high up on the anterior wall of the ventricle. The whole is an exact replica 
of the larger “primary” mitral orifice, and the two evidently functioned 
together. The segments of the latter valve are attached to a powerful group of 
papillary muscles springing from the left wall of the ventricle. An accidental 
finding in an old man aged 71, who died after amputation of the thigh for 

* Specimen in the Cardiac Anomaly Collection of McGill University. 


gangrene of the leg. The renal artery and vein were double on both sides, a 
suggestive combination. (From J. Cohn, Uber doppelte Atrio-ventricular- 
ostium, Konigsberg, 1896, p. 19, PI. I.) 

Fig. 5.—Congenital tricuspid insufficiency due to incomplete differentiation 
of septal cusps.* 

a. Electrocardiogram from this case. Tracing shows a regular sinus 
rhythm with rate of 100. Right-axis deviation is marked. Pi and P 2 of good 
amplitude, P 3 is frequently inverted, if 2 is low. A deep Q 3 is present. The 
R-T segment is slightly elevated in leads I and II. Ti and T 2 are upright. 
Impression: Increased right auricular activity and right ventricular strain. 
(Reading by G. Nicolson.) 

b. The heart laid open to show interior of the hugely dilated right chambers 
and the anomalous tricuspid valve. The marginal and infundibular segments 
are well developed and are attached by delicate chordae to well-formed 
papillary muscles, but the septal cusp is incorporated on its ventricular surface 
with the underlying myocardium without the intervention of these structures, 
and it carries on its auricular surface a mass of irregularly formed papillary 
endocardial outgrowths of gelatinous appearance arranged in rows along its 
free border and extending along the other cusps, as well as on the free borders 
of the pulmonary segments. The foramen ovale and ductus arteriosus are 
widely patent and the inferior cava has a triple orifice. 

c. X-ray of this heart in the antero-posterior diameter, showing enormous 
enlargement of the cardiac shadow, which extends to the axillary borders and 
above to the second rib. 

From a cyanotic male infant aged 7 days, who presented a rough pre- 
svstolic thrill with accompanying presystolic and long rough systolic murmurs 
heard with maximum intensity over the center of the precordium a little to 
the left of the midsternal line. At autopsy the heart lay transversely against 
the diaphragm and completely filled the anterior aspect of the thorax, the 
hugely dilated right auricle and ventricle lying to the right and left of the 
auriculo-ventricular sulcus which ran obliquely downward in the median 
line of the body. The tricuspid orifice measured 7.5 cm., the pulmonary 
2 cm. in circumference. Microscopic examination of the endocardial nodules 
on the tricuspid and pulmonary cusps showed these covered with a single 
layer of endothelium and to consist of an abundant fibrillary matrix con¬ 
tinuous with the endocardium and showing no sign of inflammation or hyper¬ 
plasia. (From an unpublished case under care of Graham Ross in the Royal 
Victoria Hospital, Montreal, from the autopsy service of Prof. Horst Oertel. 
Drawing by P. Lariviere, Medical Art Department, McGill University.) 

Fig. 6 .—Ebstein type of congenital tricuspid insufficiency. A membranous 
sheet containing multiple large fenestrations is stretched across the orifice 
and displaced below the ring posteriorly, so that a part of the right ventricle 
lies in the cavity of the auricle, resulting in great dilatation of both chambers. 
(Case of J. Bassen of Yale. Reported by M. E. Abbott, Blumer’s Bedside 
Diag., 1928, 3: 482.) 


24 


PLATE X. OTHER ANOMALIES OF THE ENDOCARDIUM 



Fig. 1. 


Fig. 2. 




Fig. 5b. 


Fig. 5c. 


Fig. 6. 


25 




































































































































































PLATE XI 


A. HYPERTROPHY OF THE HEART IN INFANTS. B. CONGENITAL RHABDOMYOMA 

A. Hypertrophy of the Heart in Infants. The term “congenital idiopathic hypertrophy” has long been applied to a now well-recog¬ 
nized condition occurring in infancy and early childhood, characterized by great cardiac enlargement in the absence of any sign of myo¬ 
cardial disease or other intra- or extracardiac etiological factor, a pure or primary hypertrophy of the muscle fibers alone existing. This 
concept of the pathology of this condition has been gradually changing as a result of investigations into the history of the recorded cases 
and the more careful histological examination of the myocardium in those recently presented. Thus Stoloff, in 1928 in a statistical survey 
of the 34 cases in the literature at that date, eliminated all but 17 of these from the “idiopathic” class as presenting a definite etiology, 
and Ivugel and Stoloff in 1933 got only 15 apparently primary hypertrophies from 52 cases analyzed. This number must undoubtedly be 
further reduced in the light of Putschar’s and Pompe’s recognition in 1933 of the cardiac type of glycogen-storage disease as a cause of 
congenital cardiomegaly; and also of the findings recorded in Kugel and Stoloff’s recent monograph of definite inflammatory changes in the 
hearts of seven cases of massive cardiac enlargement occurring in children varying from 3 months to 6^ years in age. The latter authors 
express their belief that with our increasing knowledge of the causes of cardiac hypertrophy in children and with the help of a systematic 
microscopic investigation of the myocardium, in every case brought to autopsy, the old concept of the “idiopathic” nature of this lesion 
will eventually completely disappear. Congenital hypertrophy of the heart, presumably present at or before birth as in Simmonds’ and 
Oberndorffer’s early cases remains, however, a definite clinical entity, characterized by great cardiac enlargement, pallor and lassitude, the 
sudden onset of dyspnoea and cyanosis and a tendency to sudden dramatic exitus. The cases figured opposite are examples of some of 
the different pathological conditions that may underlie these phenomena. That shown in Fig. 1 (cardiomegaly glycogenica) was published 
by Paul White in 1931, two years before Putschar’s article appeared. 

B. Congenital rhabdomyoma (Fig. 2) is shown here in proximity to the latter case as featuring the suggestion brought forward by some 
authors that a similar perverted or delayed glycogen metabolism might be at work in both conditions. 

In addition to the references cited below see the following: A. So-called primary congenital hypertrophy: Simmonds, Munch, med. Woch., 1899, 46: 108; 
Oberndorffer, Monatschr. f. Kindheilk., 1914, 13: 357; M. Steiner and M. Bogin, Amer. J. Dis. Child., 1930, 39: 1255; E. G. Stoloff, Amer. J. Dis. Child., 1928, 
36: 1204; M. Kugel and E. G. Stoloff, Amer. J. Dis. Child., 1933, 46: 229. Cardiomegaly glycogenica: W. Putschar, Beit. z. path. Anat. Zieg., 1932-33, 90: 223; 
J. C. Pompe, Ann. d’anat. path., 1933, 10: 23; E. M. Humphreys and K. Kato, Amer. J. Path., 1934, 10: 589; A. B. Ellis, Proc. Roy. Soc. Med. Sect. Dis. Child., 
1934, 34: 282, 1330. Cardiac hypertrophy in infancy in coarctation of aorta: H. D. Levine, Amer. J. Dis. Child., 1934, 48: 1072. Hypertension in a boy 
of 2 years; H. B. Taussig and D. Remsen, Bull. Johns Hopkins Hosp., 1935, 67: 183. B. Congenital rhabdomyoma: L. Berger and A. Vallee, Ann. d’anat. path., 
1930, 7: 797; S. Farber, Amer. J. Path., 1930, 7: 105; W. M. Yater, Arch. Ini. Med., 1931. 


Fig. 1.—Congenital so-called idiopathic hypertrophy, proved by micro¬ 
scopic investigation to be the seat of von Gierke’s glycogen storage (cardio¬ 
megaly glycogenica). The liver, which was also enlarged, showed diffuse 
vacuolization of its cells. 

a. Roentgenograph from this case showing great enlargement of the cardiac 
shadow, which fills the left chest merging with the shadow of pneumonic 
consolidation of the left lung. 

b. The heart itself, showing great increase in size especially in its left 
chambers and a rounded globular contour. At autopsy it occupied one full 
third of the thorax and weighed 175 gm. (normal 34 gm.). Microscopic 
examination by Tracy Mallory showed the muscle cells twice as large as 
normal at this age, with large vacuoles surrounding the nucleus in each muscle 
cell, which did not react to fat stains but showed carmine-stained granules 
when treated later with Best reagents (see Humphrey and Kato, Amer. J. 
Path., 1934, 10: 599). 

From a girl aged 7 months with an interesting familial history of congenital 
heart disease, who from the sixth month had had spells of quick breathing 
accompanied by cyanosis, and became markedly cyanotic 2 days before death, 
with high temperature and signs of lobar pneumonia. There was marked 
precordial prominence, and the electrocardiogram (the only one reported on 
in glycogenic cardiomegaly) showed sinoauricular tachycardia, rate 140, and 
normal axis deviation. (Reported by Sprague, Bland and White, Amer. 
J. Dis. Child., 1931, 41: 877. Fig. h reproduced from Heart Disease, by Paul 
White, by permission of The Macmillan Company, New York.) 

Fig. 2.—Congenital rhabdomyoma of the heart with multiple nodules in 
the myocardium of the right ventricle. From an infant aged 9 months which 
died in convulsions. At autopsy the tumors showed the characteristic histo¬ 
logical structure, and there was tuberous sclerosis of the brain and kidneys. 
(Unpublished case in the service of K. Terplan of Buffalo. Specimen No. 
14.142 4 in the Cardiac Anomaly Collection of McGill University.) 

Fig. 3.—Congenital hypertrophy of the heart in anomalous origin of the left 

coronary artery. 

a. Electrocardiogram from this case showing low voltage and normal axis 
deviation with a late and deep inversion of the T waves of the coronary type, 
especially well seen in leads I and II. The latter feature yields definite 


diagnostic evidence in this combination of the anomalous origin of the left 
coronary, and the tracing is of great interest in that connection. 

b. The heart laid open to show the cavity of the right ventricle. The orifice 
of the anomalous coronary is seen above the left pulmonary cusp. The 
curious arterio-venous dilatation shown in PI. IV, Fig. 3 was in process of 
development, but is not visible in the picture. 

c. Seven-foot x-ray film of the chest, showing the diffuse cardiac enlarge¬ 
ment, most marked in the region of the left ventricle. 

From a female child aged 3 months, well until 2 weeks before death, when 
paroxysmal attacks with difficult respiration, transient loss of consciousness 
and signs of severe shock set in. Death of respiratory failure some hours 
after one of these attacks. No cyanosis except on prolonged crying. The 
heart weighed 91 gm., left ventricle 11 mm. thick, left coronary from pul¬ 
monary artery, all branches dilated. Microscopic examination showed 
increase in number of muscle fibers, large vascular spaces with intervening 
fibrosis with myofibrillary degeneration at deeper levels and some vacuoliza¬ 
tion of individual cells. (Republished by permission from E. F. Bland, 
Paul D. White, and Jos. Garland, Amer. Heart J., 1933, 8: 787, Figs. 1, 2 
and 4.) 

Fig. 4.—Dilatation and hypertrophy of the heart with myocardial degener¬ 
ation and fibrosis, in an infant aged 8 months. 

a. The x-ray of this case shows a greatly enlarged cardiac shadow which 
reached to the axillary border on the left and the midclavicular line on the 
right. Shadow at the base also widened especially on left. 

b. Anterior view of the enlarged heart. At autopsy it weighed 90 gm. 
(normal 32 gm.) and was distinctly dilated as well as hypertrophied in all its 
chambers with all valves normal. 

c. Microphotograph of the myocardium showing focal areas of myocardial 
degeneration and a few lymphocytes around fibrous areas in the myocardium. 
Some of the muscle fibers showed atrophy and degeneration with replacement 
fibrosis. There were no areas of suppurative infiltration. A few muscle cells 
showed vacuoles giving a glycogen reaction. 

d. Electrocardiogram showing sinus tachycardia rate about 150 beats per 
minute. QRS of low amplitude in lead I. T waves of low amplitude. 
(An unpublished case in the service of Maurice Kugel from the same series 
reported, Amer. J. Dis. Child., 1930, 39: 1255, and kindly supplied by him 
for publication in this Atlas.) 


26 


PLATE XI. A. HYPERTROPHY OF THE HEART IN INFANTS. B. CONGENITAL RHABDOMYOMA 



Fig. 4a. 


Fig. 4b. 
27 


Fig. 4d. 












































































PLATE XII 


ANOMALIES OF THE CORONARY SINUS 

The three remarkable cases figured opposite require a word of elucidation from the standpoint of development. The coronary sinus 
is derived from the transverse portion and left horn of the sinus venosus, which receives the left superior vena cava of later foetal life, and 
its proximal part persists in the normal human heart while the distal portion of the left superior vena cava undergoes involution but can 
still be traced in the tiny vestigial oblique vein of Marshall. Persistence of this entire trunk may occur as shown in Fig. 3. Further, the 
pulmonary veins originate as a single trunk in the center of the sinus venosus, but subsequently, on the shifting of this cavity to the right 
of the embryonic heart, come to lie in the posterior wall of the primitive auricle just to the left of the valvula venosa sinistra; and an arrest 
at this point, so that the common pulmonary vein remained in connection with the sinus venosus, woidd result in the very rare anomaly 
shown in Fig. 1. Lastly, Bela Halpert’s case, shown in Fig. 2, is an exquisite example, unique in the literature, of a true congenital arterio¬ 
venous aneurysm of the coronary circulation, the result of an anomalous anastomosis in the primitive vascular network that encircles the 
cardiac onlage in very early embryonic life. 

Other references bearing on the subjects in this plate are as follows: Pulmonary veins enter coronary sinus: D. Nabarro, J. Anat. and Path., 1902-03, 37: 383. 
Persistent left superior vena cava entering coronary sinus in complete absence of right: S. A. Habershon, Tr. Path. Soc. Lond., 1876, 27: 79; A. Dietrich, Virch. 
Arch., 1913, 212: 119; H. Schultz, Virch. Arch., 1914, 216: 35; Gruber, Virch. Arch., 1885, 99: 492 (see also PL XXIV, Fig. 7, and PL XXV, Fig. 2c). 


Fig. 1.—Both pulmonary veins empty into coronary sinus as common 
trunk. Patent foramen ovale. 

a. Anterior view, showing interior of the greatly dilated and hypertrophied 
right auricle and ventricle, the huge orifice of the coronary sinus 1.8 cm. 
across and the patent foramen. The right chambers occupied the entire 
anterior aspect of the heart, the pulmonary artery was greatly dilated, the 
left ventricle was small and the aorta hypoplasic. 

b. Posterior view, showing entrance of left into right pulmonary vein and 
of this into the hugely dilated coronary sinus. In this remarkable case the 
pulmonary veins had retained their primary connection with the sinus ven¬ 
osus as represented by the coronary sinus (see above), and the entire volume 
of aerated blood passed into the right auricle and thence, mixed with venous 
blood from the great veins, through the foramen ovale to the systemic circu¬ 
lation, a venous-arterial shunt existing (Group III). The subject was a 
female child aged 2 years 4J^ months, with precordial bulging and systolic 
murmur and thrill, maximum at the fourth left interspace, who presented no 
cyanosis at birth but developed paroxysmal attacks at 9 months and profound 
terminal cyanosis 5 days before death. (From an unpublished case in the 
service of A. F. deGroat and Harvey Thatcher, Little Rock, Ark.* The only 
other case on record was reported by Nabarro, l.c. above.) 

Fig. 2.—Anomalous communication of right coronary artery with coronary 
sinus by arterio-venous anastomotic loop with cirsoid aneurysmal dilatation 
of arterial trunk. The vessels in the coronary sulcus have been dissected out 
and extended. The right coronary artery expands immediately after its 
origin from the right aortic sinus of Valsalva into a series of huge vascular 
loops, atheromatous at the kinks, which projected wormlike above the epi- 

* Specimen in the Cardiac Anomaly Collection of McGill University. 


cardial fat, and passed backward to the level of the posterior longitudinal 
sinus, where it emptied by an opening 5 mm. across into a thinner walled 
arterio-venous loop with convexity to the left, which encircled and opened 
into the lower border of the greatly dilated coronary sinus. This structure 
measured 2.5 cm. across and received also the dilated vena cava magna behind 
and the tiny oblique vein of Marshall above before emptying into the right 
auricle by a small orifice 3 mm. in diameter. Thebesian valve absent. The 
left coronary artery (seen at extreme left) was slightly dilated and atheroma¬ 
tous, but otherwise normal. 

From a man aged 54, dying from carcinoma of pylorus, who presented no 
clinical manifestations of cardiac disorder. The heart was hypertrophied 
and subepicardial fat increased. (Reported by Bela Halpert, Heart, 1930, 
16: 129, and published by his permission from the original drawings.) 

Fig. 3.—Persistent left superior cava emptying into the greatly dilated 
coronary sinus with absence of right superior cava.* 

a. Anterior view, showing the large anomalous trunk coursing down from 
above and merging with the greatly dilated coronary sinus. 

b. Interior of right auricle, showing huge mouth of coronary sinus 4 cm. in 
diameter and absence of all trace of orifice of right superior cava. 

c. Interior of left auricle showing closed foramen ovale and entrance of 
pulmonary veins. 

From a man aged 23, who died of cerebral abscess following staphylococcus 
sinusitis. No cardiac symptoms or other anomalies. (From an unpublished 
case in the service of Lt. Col. Foucar, Walter Reed Hospital, Washington, 
D. C.) 


28 


PLATE XII. ANOMALIES OF CORONARY SINUS 



I .v.c. 



Fig. la. 


Fig. lb. 


Fig. 2. 




Fig. 3c. 


Fig. 3a. 


29 
























.» 




















. 
























, 








■ 


































• ■ 




* 




* 








. 

- 

. 


' 




✓ 

















GROUP II 

CASES OF ARTERIAL-VENOUS SHUNT WITH TERMINAL REVERSAL OF FLOW 

(CYANOSE TARDIVE) 


PLATE XIII 


PATENT DUCTUS ARTERIOSUS 

In persistent patency of the ductus arteriosus it is generally assumed that a continuous leakage takes place from the aorta (in which 
the pressure is normally much higher than in the pulmonary artery) into the latter, an arterial-venous shunt existing; and that these relative 
pressures are maintained, in spite of the open communication between the two circulations, until some cause for respiratory obstruction 
sets in, such as long-continued crying, spasmodic coughing, etc., which raises the pressure in the pulmonary circulation and causes a tem¬ 
porary reversal of flow through the canal with the sudden appearance of a transient cyanosis, which passes off with the exciting cause. 
Absence of cyanosis except as a transient or terminal phenomenon is therefore the characteristic clinical picture of these subjects, who 
usually present a tall slender build and tendency to pallor significant of aortic hypoplasia. The converse may, however, be true, for in 
some cases fluctuation in the intra-arterial pressures seems to occur very readily, so that oxygen unsaturation of the circulating blood is 
raised close to its threshold value and the subjects may be of a rather stocky build with high-colored complexion and a bluish tinge of the 
lips on slight exertion. That the course of the blood is from left to right through the canal under otherwise normal conditions is evident 
from the pathology of these cases, for the pulmonary artery is practically always dilated and the left ventricle is usually hypertrophied more 
than the right. Moreover, in the infective processes that so frequently supervene (Fig. 4 b) the pulmonary end of the ductus with the 
immediately adjacent tissues is always the initial seat of the vegetative inflammatory lesion, a very suggestive feature, as indicating that 
this point has been the seat of strain in the continuous passage of a left to right shunt through the defect. Characteristic physical signs 
are usually present, but vary according to the length of the canal and the shape of its aortic orifice. The familiar train-in-a-tunnel machin¬ 
ery murmur, beginning just after the first sound and continuous throughout the greater part of the cardiac cycle, localized over the first, 
second and third interspaces with maximum intensity at the second, with accompanying systolic or continuous thrill, which when present 
is pathognomonic, occurs in only about a third of the cases. In a number of others it is replaced by a double murmur of which the systolic 
element is apparently generated at the ductus, but the diastolic is probably that of a pulmonary insufficiency (Laubry and Pezzi). In 
over 30 per cent of the 92 cases analyzed by the writer, the murmur was systolic in rhythm but usually began just after the first sound, 
was of rather harsh character prolonged throughout systole, and of somewhat irregular localization with maximum intensity at the pul¬ 
monary area or lower over the precordium, even in some cases at the apex. A systolic murmur appears to be the rule in infants and very 
young children, in whom a continuous murmur is rare. Pulmonary accentuation is usually present. Other confirmatory signs are figured 
opposite. 


Fig. 1.—Diagram by W. T. Dawson showing the course of the normal 
circulation after the ductus arteriosus has closed in postnatal life. The 
venous and arterial circulations are here entirely separate. (From Abbott 
and Dawson, Internat. Clin., 1924, 4: 162.) 

Fig. 2.—The same, showing the foetal circulation. Here the ductus is 
open and, the pressure being highest in the right heart, the venous blood 
passes from it into the pulmonary artery and thence through the patent ductus 
into the descending aorta to the lower extremities, a venous-arterial shunt 
existing. (From Abbott and Dawson, l.c .) 

Fig. 3.—The same, showing the circulation in persistent patency of the 
ductus. The pressure being highest in postnatal life on the systemic side, 
oxygenated blood passes from the aorta through its open lumen into the 
pulmonary artery, an arterial-venous shunt existing (shown by black arrow), 
until such time as a change in the relative pressures sets in, when a reversal 
of flow takes place (shown by the broken arrow) and venous blood flows into 
the arterial stream. (From Abbott and Dawson, l.c.) 

Fig. 4.—Patent ductus arteriosus with acute infective endocarditis origi¬ 
nating at pulmonary end of ductus, mycotic aneurysm of pulmonary artery 
and embolic abscesses in the lungs in pneumococcus septicaemia. 

a. The thoracic aorta laid open from behind to show the widely patent 
ductus admitting a penhandle, and slight coarctation of the descending arch. 

b. Heart and lungs laid open to show the interior of the right ventricle and 
the greatly dilated pulmonary artery of the right lung. The latter is riddled 
with abscess cavities, and the pulmonary lumen is occupied by a huge throm¬ 
botic mass of vegetations which block the orifice of the ductus and extend 
downward toward the pulmonary cusps, which are free and healthy. The 
aorta (seen extended above) is hypoplasic. 

From a slender delicately built girl of 19 who presented a continuous 
machinery murmur with systolic accentuation and thrill with maximum inten¬ 
sity in the second left interspace, x-ray cap and “Gerhardt” dullness without 


any sign of valvular disease, and who died after some weeks of great prostra¬ 
tion, chills and septic temperature with pneumococci in the blood stream. 
Both the patency and the location of the infective process were diagnosed 
intra vitam from the classic picture presented. Microscopic examination 
showed the initial lesion situate at the pulmonary orifice of the ductus and 
extending along its lumen and that of the pulmonary artery with abundant 
pneumococci in the vegetations and in the embolic infarcts in the lungs. 
(Reported by W. F. Hamilton and M. E. Abbott, Tr. Assn. Amer. Phys., 
1914, 29 : 294. Drawing of Fig. b above by J. H. Atkinson from the specimen 
in the Cardiac Anomaly Collection of McGill University.) 

Fig. 5.—Roentgenograph of the heart in a case of patent ductus, showing 

large pulmonary arc (x-ray cap), widening of pulmonary vessels at hilum 
and hypertrophy of both sides of the heart. The patient was a woman of 
26 who presented the classic physical signs of this lesion and complete paralysis 
of the left recurrent laryngeal nerve from pressure upon this of the dilated 
ductus. Diagnosis made during life by W. S. Thayer. Death from multiple 
ruptures of the right ventricle. (Reported by Kate C. Mead, J.A.M.A., 
1910, 55: 2205. Republished by permission.) 

Fig. 6.—Heart-signs record from a case of patent ductus with continuous, 
murmur, showing low-pitched character, systolic and diastolic accentuation 
area of maximum intensity, zone of transmission, and variation at different 
areas of the precordium, in the dorsal recumbent, lateral recumbent and sit¬ 
ting postures, with point of maximum intensity of accompanying thrill. (Origi¬ 
nal diagram made for this Atlas by H. N. Segall. See his articles, Amer. 
Heart J., 1933, 8: 533; Canad. Med. Assn. J., 1932, 27: 632.) 

Fig. 7.—Electrocardiogram from a case of patent ductus showing normal 
curves. (From Clinical Electrocardiography by Sir Thomas Lewis, 1934.) 

Fig 8 . —Orthodiagraphic tracings from six cases of patent ductus, showing 
enlarged pulmonary arc (x-ray cap of Zinn) and cardiac enlargement. (From 
Th. and F. M. Groedel, Deutsch. Arch. f. klin. Med., 1911.) 


32 


PLATE XIII. PATENT DUCTUS ARTERIOSUS 


1 


1 




SCHEME OF FOETAL CIRCULATION 
PATENT FORAMEN OVALE - - PATENT DUCTUS ARTERIOSUS. 


Normal conditions. 


Fig. 2. 


I 



Arterial-venous shunt. 

Fig. 3. 


I 



Fig. 4a. 



.AORTA 
IfcTURNED 
% UP 


AREAS ' 

OF 

COLLAPSE W 


RIGHT' LUTIG 
LAID OPEll 


IRRKGUTA5 
v' ■ vfc jS 

CONSOLIDATE 


’AT I Oil i 


AREA OF** - 
SOFTEllIHG 


Fig. 4b. 



Fig. 8a. 




Jljc ' ... 


Fig. 7. 



Fig. 8b. 



Fig. 8c. 


33 
























































































































PLATE XIV 


DEFECTS OF THE INTERAURICULAR SEPTUM AND PATENT FORAMEN OVALE 

These cases may be divided, from the clinical standpoint, and for convenience of discussion, into two main categories, which are illus¬ 
trated by the diagrams opposite, namely, defects at the upper part of the interauricular septum, with which are included cases of primary 
persistent patency of the foramen ovale (Figs. 1-5), and defects at the lower part of this (persistent ostium primum Figs. 6-8). The latter 
cases are always associated with cleavage and insufficiency of the anterior mitral segment, and so do not produce a distinctive clinical 
picture but are of especial interest from their frequent association with mongolian idiocy (Fig. 8). Large defects at the upper part of this 
septum, on the other hand, are among the most interesting and significant chapters in the entire range of congenital cardiac disease. For 
these cases demonstrate, more conclusively than any others of the cyanose tardive group, the dynamic effects of a persistent arterial-venous 
shunt upon the heart and circulation. Moreover, they supply, in the great dilatation of the pulmonary artery and the eccentric hyper¬ 
trophy of the heart with hypoplasia of the aorta that invariably supervene, direct anatomical proof that the course of the anomalous 
current is from left to right through the defect until such time as a failing left heart induces a reversal of flow with signs of congestive 
failure and the sudden onset of a “late cyanosis.” A classic example of this type is shown in Fig. 2. So pronounced are the changes in 
all cases of large auricular septal defects that a definite clinical entity is established, which can be diagnosed with a fair degree of certainty 
in the light of modern roentgenological findings, especially in cases in which physical signs of the defect are localized over the midpre- 
cordium or midsternum, and a venous pulse is perceptible in the neck and liver. H. Roesler {Arch. Int. Med., 1934, 54: 339) points out 
that, in the cases of massive right-sided cardiac enlargement in which dilatation predominates over hypertrophy, the silhouette of the right 
heart as seen by x-rays extends usually far into the left chest with the huge pulmonary arc above on the left and the aortic knob diminished 
or absent; and that for the same reason the electrocardiogram shows only a moderate right predominance in the presence of extreme right 
heart enlargement. 

Acquired valvular lesions, especially mitral insufficiency and stenosis, are very commonly associated with these defects and the latter 
combination forms the well-recognized syndrome known as mitral stenosis with interauricular insufficiency, of which the classic example 
shown opposite (Fig. 5) was reported by the writer a year before Lutembacher described this condition as a clinical entity. 

In two cases of paradoxical embolism through a patent foramen ovale presented by W. W. Beattie (Fig. 3), this author made the 
important observation that, in cases of valvular patency, this phenomenon can take place only when a previous pulmonary embolism 
has reduced the pressure in the right auricle. 

(For bibliography see page 38.) 

Fig. 1.—Diagram showing the course of the circulation in patent foramen 
ovale and defect at the upper part of the interauricular septum. The pressure 
being highest under physiological conditions in the left auricle, the direction 
of the shunt is from left to right through the defect (indicated by the solid 
black arrow) until a pathological rise of pressure sets in in the right auricle 
leading to a reversal of flow (broken arrow). (From the article by W. T. 

Dawson and M. E. Abbott, Internal. Clin., 1924, 4: 164, Fig. 6.) 

Fig. 2.—Huge defect at upper part of interauricular septum above and 
posterior to closed foramen ovale. Pulmonary dilatation and atheroma with 
insufficiency and calcification of cusps and hypoplasia of aorta. Eccentric 
hypertrophy of right chambers.* 

a. Left heart laid open to show: a, the narrow hypoplasic aorta; b, the cut 
edge of the dilated pulmonary artery; c, the narrow aortic vestibule; d, the 
relatively small left auricle; e, the auricular septal defect, size of half a dollar; 

/, the closed foramen ovale; g, the defective auricular septum; h, the mitral 
valve; i, the interior of left ventricle. 

b. The greatly dilated and hypertrophied right chambers laid open to show: 
a, the huge defect in the interauricular septum; b, orifice of inferior vena cava; 
c, complete absence of Eustachian valve or annulis ovalis; d, the dilated 
coronary sinus; e, the dilated sinus of the right ventricle;/, orifice of superior 
vena cava; g, the defective inter-auricular septum; h, passage into conus of 
right ventricle. 

c. The base of the pulmonary conus showing the greatly dilated artery with 
thickened, calcareous and insufficient cusps, the hypoplasic aorta and the 
dilated right auricle above and behind this. (Drawing by A. Cheney.) 

From a woman aged 64, who had worked hard as a charwoman and had had 
perfect health until six months before death. Since then transient cyanosis 
and malaise. Admitted semicomatose with marked cyanosis and oedema and 
died before examination of the chest could be made. (Reported by M. E. 

Abbott and J. Kaufmann, J. Path, and Bad., 1910, 14: 525, Case 1.) 

Fig. 3.—Widely patent foramen ovale with antemortem adherent thrombus 
in right auricular appendix, which became the source of paradoxical embolism. 

The foramen measured 3 by 2 cm. The right chambers were greatly dilated 
and there was a small associated defect at base of the interventricular septum. 
a, foramen ovale; b, auricular septum. 

From a man aged 49, who became short-winded at 39 and developed deep 
cyanosis with oedema shortly before death from oedema of the glottis. (From 
W. \V. Beattie, “Paradoxical embolism associated with two types of patent 
foramen ovale,” J. Tech. Meth., 1925, 11: 64.) 

Fig. 4.—Orthodiagraph from a case of mitral stenosis with patent foramen 
ovale, showing characteristic mitral configuration with great enlargement of 
pulmonary arc and right heart and absence of aortic knob. (Reported by 
W. Dressier and H. Roesler, Ztsch. f. klin. Med., 1930, 112: 42, Fig. 3.) 

Fig. 5.—Large gaping foramen ovale with calcified lower border and fenes¬ 
trated annulus ovalis and acquired button hole mitral stenosis, great dilata¬ 
tion and hypertrophy of right ventricle, widening of pulmonary and slight 
coarctation of aorta.* a, pocket in wall of left auricle. 

From a married woman aged 38, who had borne one child. Menses set in 


at 21 and disappeared at 28. Acute rheumatism at 14. Repeated attacks of 
failing compensation since age of 28. Right hemiplegia at 30 (crossed embol¬ 
ism?). Admitted in congestive failure with auricular fibrillation, anascarca 
and severe dyspnoea. Examination showed a presystolic murmur and faint 
thrill at fourth left interspace near sternum. Terminal cyanosis. (Case of C. 
F. Martin. Reported by M. E. Abbott, Int. Assn. Med. Mus. Bull., 1915, 
5: 129. Also in Blumer’s Bedside Diag., 1928, 2: 399, Fig. 254.) 

Fig. 6.—Diagram of the circulation in defect at the lower part of the inter¬ 
auricular septum {persistent ostium primum). The remarks made under 
Fig. 1 regarding the direction of shunt and terminal reversal of flow apply here 
also. (From Dawson and Abbott, ibid., p. 165.) 

Fig. 7.—Large crescentic defect at lower border of interauricular septum 
with cleavage of anterior mitral segment, hypertrophy and dilatation of both 
auricles but especially right and right ventricle, dilatation of pulmonary artery 
and hypoplasia of aorta.* 

The heart is laid open to show the interior of the left chambers: a, the dilated 
left auricle; b, the two parts of the cleft anterior segment of the mitral valve; 
c, the closed foramen ovale; d, the auricular septal defect persistent ostium 
primum. 

From a strong well-developed man of 35, who died of perforative appendi¬ 
citis. Confused heart sounds were heard at the apex. No cyanosis. 
(Reported by M. E. Abbott and J. Kaufmann, J. Path, and Bad., 1910, 
14: 525, Case 2.) 

Fig. 8.—Persistent ostium primum with cleavage of anterior mitral segment 
and deformity of tricuspid septal cusp. No cyanosis.* 

The auricular septum presents a valvular patency of the foramen ovale 
and ends below in a crescentic free border which forms the upper boundary 
of a defect 3 by 2 cm. large, the lower border of which is formed by the over¬ 
lapping upper halves of the completely divided anterior mitral segment. 

From an infant which presented a peculiar murmur over the precordiuin 
and was the subject of mongolian idiocy. Death at 10 months from broncho¬ 
pneumonia. (Case of Keith Gordon. Reported by M. E. Abbott, Int. Assn. 
Med. Mus. Bull., 1924, 10: 111, Case 2.) 

Fig. 9.—Premature closure of the foramen ovale with generalized anasarca 
of the foetus and pulmonary artery joining descending aorta through widely 
patent ductus.* 

The valvula foraminis ovalis forms a distended membrane bulging into 
the left auricle and above it is seen the crescentic border of the closed foramen 
ovale. 

From a male foetus, stillborn in the eighth month of the mother’s second 
pregnancy, which was characterized by great hydramnios. The entire body 
was the seat of an enormous generalized oedema and the right auricle was dis¬ 
tended. The foramen was seen to be occupied by a thin translucent mem¬ 
brane crossed by numerous fine trabeculae, which apparently closed it 
completely. 

(From William Osier, “Cases of cardiac abnormalities,” Montreal Gen. 
Hosp. Clin, and Path., 1880, 1: 177.) 


* Specimen in the Cardiac Anomaly Collection of McGill University. 


34 


PLATE XIV. DEFECTS OF INTERAURICULAR SEPTUM AND PATENT FORAMEN OVALE 



Fig. 2a. 


Fig. 7. 



Fig. 2c, 


Fig. 5 

35 


Fig. 9, 
































PLATE XV 


A. DEFECTS OF INTERVENTRICULAR SEPTUM. B. DEFECTS OF AORTIC SEPTUM 


A. Localized defects of the interventricular septum are usually situate at the base of the heart just anterior to the pars membranacea 
and open on the side of the right ventricle behind the septal tricuspid segment. Less commonly the defect lies more anteriorly and opens 
into the conus of the right ventricle (bulbar septal defect, Fig. 9) ; or in a few rare instances it may lie in the lower part of the septum, as 
is normal in the heart of the python (PI. Ill, Fig. 4), and as occurred in the remarkable case of E. Weiss {Arch. Int. Med., 1927, 39: 705). 
In uncomplicated cases of defects in the first of these locations {maladie de Roger), this is usually quite a small opening which transmits 
an arterial-venous shunt of blood into the right ventricle with considerable force, the current impinging upon the opposite wall of the latter 
and often producing there a patch of fibrosis. Such a defect in this location has little or no effect upon the general circulation. The 
physical signs, however, are usually distinctive, consisting of a holosystolic rather harsh murmur maximum in the third or fourth left inter¬ 
space, accompanied in about a third of the cases by a distinct thrill (Fig. 2). The electrocardiogram reveals in some cases a partial heart 
block (Fig. 7), indicating that interference with the bundle fibers has occurred. Cyanosis is absent except as a transient or terminal 
feature, and is even then rare. The clinical picture is thus that of absence of symptoms in the presence of distinctive physical signs in an other¬ 
wise normal individual, and the significance of this lesion lies not in the functional effect of the defect, but in the great frequency with which 
a subacute bacterial endocarditis develops along its margins or on the opposite wall of the right ventricle (Fig. 6). 

B. Defect of the aortic septum may be located above the valves or, more commonly, it occupies the right aortic sinus of Valsalva and 
leads into the pulmonary conus either as a direct communication or as a thin-walled aneurysmal sac which undergoes rupture in later life 
(Fig. 9). A bulbar septal defect below the aortic cusps is not infrequently associated. The physical signs are characteristic of the arterial- 
venous communication above the cusps and the danger of infective endarteritis is very great. 


A. See on this subject; H. Roger, “Communication congenital du coeur par inocclusion du septum interventriculaire,” Bull, de I’acad. de Med., 1879, 8 : 1074; 
E. Dupre, Bull. Soc. Anat. de Paris, 1891, 5: 404; LeHoux, “La cyanose tardive” ( maladie de Roger), Paris Thesis, 1902; Hart, Virch. Arch., 1905, Bd. 181, 57; 
J. G. Monckeberg, “Ueber das Verhalten des at.rio-ventricular System in Cortriloculare Biatriatum,” Studien z. Path., u. Entwickelung, 1920, 2: 448; E. Weiss, 
“Large defect in lower part of interventricular septum with impaired conduction and terminal cyanosis,” Arch. Int. Med., 1927, 39: 705. 

B. See the article by M. E. Abbott on “Clinical and developmental study of a case of ruptured aneurysm of the right anterior aortic sinus of Valsalva,” 
Contrib. Med. Biol. Research, Sir William Osier Anniversary Volume, Paul B. Hoeber, 1919, 2: 899 (Figs. 9a, b and c) for full bibliography on this subject to that 
date; also articles by Hektoen, “Large defect in septum between pulmonary artery and aorta, the heart normally developed. Tr. Path. Soc., Chicago, Nov. 12, 
1900; C. Goehrung, J. Med. Research, 1920-21, 42: 49; T. G. Moorhead and E. C. Smith, Irish J. Med. Sci., 1923, p. 545, Fig. 1. 


A 

Fig. 1.—Diagram showing the course of the circulation in localized, uncom¬ 
plicated defects at the base of the interventricular septum (maladie de Roger). 

The black arrow passing from left to right through the defect shows that the 
shunt is arterial-venous under the normal conditions of a relatively higher 
pressure in the left ventricle. The dotted arrow in the opposite (right to left) 
direction indicates the possibility of a (terminal) reversal of flow. (From 
Abbott and Dawson, Internat. Clin., 1924, 4: 166.) 

Fig. 2.—Heart-signs record from a case of maladie de Roger. Graphic 
representation of the holosystolic murmur and accompanying thrill with 
maximum intensity at the fourth left interspace near sternal border; also 
variations of the murmur, in intensity, pitch and relation to first sound, at 
the pulmonary and aortic areas, at apex, along the right sternal border and 
posteriorly at left angle of scapula. (Diagram and record made for this 
Atlas by H. N. Segall. Reference under PI. XIII, Fig. 6.) 

Fig. 3.—Small ventricular septal defect just anterior to the undefended 
space (maladie de Roger), view from left ventricle.* The anomalous opening 
admits a knitting needle and has sclerosed thickened margins. Heart was 
not enlarged and weighed 230 gm. From a female aged 20, who died acci¬ 
dentally (from burning). Specimen 14.122 2 in the Medical Museum, pre¬ 
sented by John McCrae. (Republished from the article by M. E. Abbott in 
Osier’s Mod. Med., 1927, 4: 690, Fig. 62.) 

Fig. 4.—Large ventricular defect at base admitting a slate pencil. View 
from right ventricle.* From an infant aged 49 days. The right ventricle 
was slightly hypertrophied and the pulmonary artery dilated. Specimen 
No. 14.122 3 , presented by John McCrae. (Republished, ibid., p. 689, Fig. 61.) 

Fig. 6.—Orthodiagraphic drawing from a young man aged 17. Diagnosed 
clinically as ventricular septal defect. Second (pulmonary) arc is slightly 
increased. Right auricular border pulsated very strongly and synchronously 
with left border. Ventricles slightly enlarged symmetrically. (From 
A. Dietlin, Herz und Gefdsse im Roentgenbild, 1923, Fig. 115, p. 225.) 

Fig. 6.—Ventricular septal defect (maladie de Roger) with acute bacterial 
endocarditis of tricuspid segments adjacent to and screening the defect. 
Staphylococcus septicaemia. View looking into right ventricle showing the 
small circular orifice of the defect, with smooth margins lying just behind the 
infundibular tricuspid segment, the ventricular surface and chordae tendineae 
of which are loaded with thrombotic vegetations. The endocardium else¬ 
where appears healthy. Apex slightly bifid. 

From a previously healthy boy of 2k£ years, who presented a moderately 
long, slightly harsh precordial systolic murmur, maximal to left of lower 
sternum attended by a systolic thrill over midsternum. No cyanosis or 
clubbing. Developed suddenly a severe sore throat with epistaxis and high 
temperature and two weeks later admitted to the Good Samaritan Hospital, 
Boston, in profound toxaemia, leucocytes 40,000. S. Albus in pure culture 
in blood stream. Diagnosis of Roger’s disease with infective endocarditis 
made intra vitam. (Published by courtesy of Paul D. White. Art drawing 
made for this Atlas by Muriel McLatchie, Massachusetts General Hospital.) 

* Specimen in the Cardiac Anomaly Collection of McGill University. 


Fig. 7.—Electrocardiogram showing partial heart block in a case of maladie 
de Roger. From a boy aged 10 years. The tracing shows a sinus rhythm 
with a partial A-V block of 2:1 degree. The auricular rate is 78, the ven¬ 
tricular 38 per minute. There is no delay in bundle branch conduction or in 
the electrical axis deviation. The contour of the deflection is normal. (Trac¬ 
ing and interpretation by G. Nicolson.) 

B 

Fig. 8.—Diagram showing course of the circulation in defect of the aortic 
septum. Note that the anomalous communication between the aorta and 
pulmonary artery is directly above the valves and that an arterial-venous 
shunt (indicated by black arrow) exists. (From Abbott and Dawson, 
Internat. Clin., 1924, 4: 166.) 

Fig. 9.—Congenital aneurysm of right aortic sinus of Valsalva rupturing 
into conus of right ventricle with associated bulbar septal defect and extensive 
subacute infective endocarditis with aortic and pulmonary insufficiency, 
hypoplasia of aorta and dilatation of pulmonary artery.* 

a. View of interior of right ventricle showing long tubular aneurysm opening 
into conus just below pulmonary cusps with bulbar septal defect immediately 
below this, and probes passed through both orifices. Luxuriant vegetations 
of infective endocarditis clothing margins of the defects, opposite wall of the 
conus and the thickened pulmonary segments. (Drawing by J. H. Atkinson.) 

b. Interior of left ventricle and aortic valve showing the probes passed 
through the defects in the right ventricle emerging in the sinus of Valsalva 
above and in base of left ventricle below the right aortic cusp, with thickening 
and insufficiency and recent vegetative endocarditis of all segments. 

From a tall spare man aged 36, intelligence above average, in perfect health 
until nine years before death, when after a severe strain he suddenly developed 
symptoms of cardiac insufficiency, never well since. On examination, heaving 
precordial impulse and very superficial diastolic thrill of maximum intensity 
in second and third left interspaces transmitted to left midaxillary and right 
nipple line, and very loud rough continuous murmur with diastolic accentu¬ 
ation, maximum in third left interspace, heard 2 in. from chest wall and over 
entire thorax. Low red cell count and leucocytosis, high septic temperature 
with occasional chills. Slight oedema but never cyanosis. (From a case in 
the service of W. F. Hamilton reported by M. E. Abbott in Contrib. to Med. 
and Biol. Research Sir Wm. Osier Memorial, P. B. Hoeber, 1919, 2: 899, 
Figs. 1 and 2.) 

Fig. 10 . —Model by Tandler showing interior of embryonic bulbus cordis 
at a stage when two points of communication existed between the great 
trunks above and below the point of fusion of the distal bulbar swellings 1 

and 3. A., aorta (fourth right arch); P., attachment of pericardium; PI., 

pulmonary artery (sixth arch); d. Bw. 1-3, distal bulbar swellings 1 and 3; 
p. Bw. A-B, proximal bulbar swellings A and B; * point at which the sound 
in the common lumen disappears, and * * point at which it reappears below in 
the common lumen of the bulbis cordis; S.a.p., septum aortico-pulmonale. 
Compare the defects above and below the right aortic cusp in Fig. 96. (From 
Keibel and Mall, Embryology, 1912, 2: 532. Reprinted by permission.) 


36 


PLATE XV. A. DEFECTS OF INTERVENTRICULAR SEPTUM. B. DEFECTS OF AORTIC SEPTUM 



B 







Fig. 6. 


Fig. 9a. 


Fig. 9b. 


' • - •• ~ " . - — 

Fig. 3. 


Fig. 4. 


Fig. 7. 


37 


Fig. 10. 


























































































MISCELLANEOUS ADDITIONAL REFERENCES 


PLATE XIV 

In addition to the articles cited under the cases described on p. 36 see also the following: Bard and Curtillet, “Contribution a l’etude de la physiologie de la 
maladie bleue; forme tardive de cette affection,” Rev. de med., 1889, 9 : 993; T. Thompson and W. Evans, “Paradoxical embolism,” Quart. J. Med., 1930, 23 : 135; 
R. Lutembacher, “De la stenose mitrale avec communication interauriculaire,” Arch. d. mal. du coeur, 1916, 9 : 235; also in Presse Med., 1925, p. 236; W. Dressier 
and H. Roesler, “Vorhofseptumdefekt Kombiniert mit Mitralstenose und aurikularem Leberpuls,” Ztsch.f. klin. Med., 1930, 112 : 421; N. W. Ingalls, “Vena cava 
superior receiving two upper right pulmonary veins and opening into both atria,” Bull. Johns Hopkins Hosp., 1907, 18 No. 193; S. McGinn and P. D. White, 
“Interauricular septal defect associated with mitral stenosis,” Amer. Heart J., 1933, 9 : 1; H. Roesler, “Interatrial septal defect,” Arch. Int. Med., 1934, 64 : 339; 
E. Lehman, “Congenital atresia of the foramen ovale,” Amer. J. Dis. Child., 1927, 33: 585; S. Gibson and A. Roos, “Open foramen ovale associated with mitral 
stenosis,” Amer. J. Dis. Child., 1935, 60: 1465; H. J. Hirschboeck, “Paradoxical embolism,” Amer. J. Med. Sci., 1935, 189: 236. 

PLATE XXI 

Persistent ostium atrio-ventriculare commune with mongolian idiocy: F. D. Gunn and J. M. Dieckmann, Amer. J. Path., 1927, 3 : 595; G. M. Robson, Amer. 
J. Path., 1931, 7: 299; J. G. Monckeberg, Centr.f. allg. Path. u. path. Anat., 1923, 33: 144; M. E. Abbott and K. Gordon, J. Tech. Meth., 1924, 10: 115; Cassel, 
Berl. klin. Woch., 1917, 54: 159; F. P. Mall, Amer. J. Anat., 1912, 13: 249; H. Schleussing, Arch. f. klin. Med., 1925, 254: 579; Dublizhaza, Zurich Thesis, 1906. 
Cor triloculare biatriatum with anomalous septum cutting off rudimentary right chamber: T. B. Peacock, Tr. Path. Soc. Land., 1855, 6: 117; H. Chiari, Cent, 
f. d. med. Wissensch., 1880, p. 186; J. D. Mann, Brit. Med. J., 1905, 1 : 614; A. H. Young, J. Anat. and Phys., 1907, 41 : 190; Marchand, Deutsch. Path. Ges., 1908, 
12: 174; E. S. Mills, J. Med. Research, 1923, 44: 257. With rudimentary left chamber: D. Kornblum, Amer. J. Path. 1935, 11: 803, also, S. K. Ngai, Amer. J. 
Path., 1935, 11: 309. 


PLATE XXIII 

See under PL II, III and IV for further discussion of this subject and additional references. Also C. Rokitansky, Die Defeckte der Scheidewande des 
Herzens, Vienna, Braumiiller, 1875; A. Spitzer, Roux’s Arch. f. Entwick., Part I, 1919, 45: 686; Arch.f. path. Anat., 1923, 243: 81; Ztsch. f. d. ges. Anat. (Abt. 1), 
1927, 84: 30; K. Katsujo, Amer. J. Dis. Child., 1930, 39: 363; P. F. Shapiro, Arch. Path., 1930, 9: 54; W. Feldman and A. Chalmers, Brit. J. Dis. Child., 1933, 
30: 27. 


PLATE XXV 

Congenital dextrocardia: S. L. Brimblecombe, Brit. Med. J., 1920, 2: 891; S. A. Reinberg and M. E. Mandelstam, Radiology, 1928, 11: 240; A. Spitzer, 
Virch. Arch., 1929, 27: 226; H. Roesler, Wien. Arch. f. inn. Med., 1930, 19: 505; Corrected transposition: see Rokitansky, PI. XXIII; Lochte, Zieg. Beit., 1871, 
24; Tonnies, Gottingen Thesis, 1884. Congenital heart block: E. P. Carter and J. Howland, Bull. Johns Hopkins Hosp., 1920, 31: 351; P. D. White, R. S. Eustis 
and W. J. Kerr, Amer. J. Dis. Child., 1921, 22: 299; J. G. Wilson and R. T. Grant, Heart, 1926, 12: 295; H. Davis and R. M. Stecher, Amer. J. Dis. Child., 
1928, 36: 115; G. B. Fleming and M. M. Stevenson, Arch. Dis. Child., 1928, 3: 221; G. Nicolson, H. I. Shulman and D. L. Green, Amer. J. Dis. Child., 1929, 
37: 580; W. M. Yater, Amer. J. Dis. Child., 1929, 38: 112; W. M. Yater, C. W. Barrier and P. E. McNabb, Ann. Int. Med., 1934, 7: 1263; W. M. Yater, W. G. 
Ledman and V. H. Cornell, J.A.M.A., 1934, 102: 1660. 


38 


GROUP III 


CASES OF PERMANENT VENOUS-ARTERIAL SHUNT AND RETARDATION OF FLOW 

(CYANOTIC GROUP) 


PLATE XVI 


SYMPTOMATOLOGY OF CONGENITAL CYANOSIS 

Congenital cyanosis, or morbus coeruleus, is the name given to the symptom complex which invariably develops when the oxygen 
unsaturation of the circulating blood is permanently raised above the “threshold value” at which this becomes perceptible in the capil¬ 
laries (placed by the calculation of Lundsgaard and van Slyke at 6.7 volumes per cent). As was pointed out by these authors in their 
fundamental contribution to the elucidation of this subject, the reduced oxygenation of the blood and corresponding cyanotic hue are the 
result of various “influencing” and “modifying” factors, the most powerful of these in this condition being a permanent venous-arterial 
shunt through the defect and a retardation of flow in the capillaries (Lundsgaard’s alpha and D factors, Fig. 8B), which act singly, or in 
combination with each other or with other elements, to produce the cyanosis of congenital heart disease. For a clear understanding of 
the clinical features presented, however, it is essential to remember that all such factors have been in operation from very early intrauterine 
life, and by their continuous action lead inevitably to capillary changes that must have an important bearing upon the peculiar symptoma¬ 
tology of this condition, which develops as life proceeds and serves to distinguish it from the cyanosis of acquired heart disease. Redisch 
and Roesler in their valuable differential study by the capillary microscope of the skin changes in these two sets of cases establish a definite 
histological picture of each (Figs. 4, 6) and express the belief, as does Wollheim, that the dilatation, stasis, congestion and abundant neo¬ 
formation of capillaries shown in congenital cyanosis are themselves the dominating factor in the production of its symptomatology. 
These authors, moreover, make the pregnant suggestion that the absence of oedema, which is such a striking feature of the advanced stages 
of morbus coeruleus, may perhaps be explained by the above changes in the capillary loops, the skin acting by means of these expanded 
thick-walled channels of the subpapillary plexus as a reservoir for the stagnating and retarded blood. Certainly these patients, especially 
persons who have attained early adult life, and in whom therefore these insidious capillary changes have been in operation over many 
years, present the hallmarks of deficient oxygenation in a form that is significant of a predominating capillary factor, and that is in sharp 
contrast to the cyanosis seen in the last stages of cardiac decompensation. The bluish-violet discoloration of the skin and mucous mem¬ 
branes, deepening on slight muscular exertion to a spectacular purplish hue, the suffused conjunctivae, clubbing of nose, fingers and toes, 
dyspnoea and dyspnoeic attacks, and, in advanced cases, the cyanosis retinae and polycythemia sometimes amounting to twelve or thirteen 
million, with absence of oedema, together present a unique clinical picture that, once seen, can never be forgotten and that is in itself 
pathognomonic of the underlying circulatory and structural as well as tissue change. 


(See page 46 for bibliography on this subject.) 

Fig. 1.—Clubbing of fingers and toes in congenital cyanosis. Note the 
discoloration and bulbous enlargement of terminal phalanges especially the 
thumbs and great toes, with broadening, shortening and convexity of the 
nails and thickening of nailbeds. From a cyanotic child in the Medical 
Service of the Montreal General Hospital. Cast made by Hortense Douglas. 
(Reproduced here by permission from a color print made of this, published in the 
writer’s monograph in Nelson’s Looseleaf Med., 1932, 4: PI. II facing p. 238.) 

Fig. 2.—The cyanotic facies as displayed in a highly gifted patient with 
the tetralogy of Fallot who survived into late middle life. This is a portrait 
of the notable American composer, Henry Gilbert, who presented the hall¬ 
marks of congenital cyanosis from his earliest childhood, but in spite of his 
crippling heart condition led an active artistic life and made an important 
contribution to contemporary music. Examination 14 months before death 
showed a rather florid cyanosis, with marked clubbing of the extremities and 
dyspnoea on exertion, eyes alert but somewhat suffused, sight excellent. A 
loud blowing systolic murmur maximal at the left fourth interspace not 
transmitted into back or neck vessels with slight accompanying thrill. Red 
cells 7,700,000. Death followed 8 days after onset of left haemiplegia. At 
autopsy the right ventricle was greatly hypertrophied and there was a huge 
circular defect 2 cm. across, above which the large aorta arose dextroposed 
from both ventricles. Pulmonary artery bicuspid and hypoplasic and 
emerged anteriorly and to the left from stenosed infundibulum. (From Paul 
D. White and Howard Sprague, “The Tetralogy of Fallot. Report of a case 
in a noted musician who lived until his 60th year,” J.A.M.A., 1929, 92 : 787.) 

Fig. 3.—Cyanosis retinae. Painting by H. Blackstock of this condition 
in a cyanotic boy aged 3^2 years. (Reproduced by permission from a color 
print published by W. B. Saunders Company in the writer’s article in Blumer’s 
Bedside Ding., 1928, 2: Fig. 311.) 

Fig. 4.—Pathological changes in the capillaries in the cyanosis of decompen¬ 
sation in acquired heart disease (capillary microscope). From the article by 
Redisch and Roesler, see Fig. 6.) 

Fig. 5.—Capillaries of the nailbeds seen under the microscope in a case of 

congenital cyanosis. Note the tortuosity and thickening of both venous and 
arterial ends of the capillary loops and their proximity to each other owing to 
formation of new capillaries. From a cyanotic girl of 12 with the tetralogy of 
Faliot and dying of subacute bacterial endocarditis of all valves. (Reported 
by S. D. Leader and M. A. Kugel, J. Pedriat., 1934, 4: 595.) 

Fig. 6.— Pathological changes in the capillaries in congenital cyanosis as 
seen under the capillary microscope. The capillary loops show great thick¬ 
ening especially’ of their venous ends, involving their convexity and sometimes 


their arterial part, and are twisted upon themselves and crowded together 
owing to the presence of many new formed anastomotic twigs, the whole 
presenting a marked contrast to that in the pathological capillary picture of 
acquired heart disease shown in Fig. 4 above. (From Redisch and Roesler, 
“Kapillarstudien,” Wien. Arch. f. inn. Med., 1929, 16: 463.) 

Fig. 7.—Diagrams by Lundsgaard and van Slyke showing graphically the 
circulation under normal conditions and under the effect of the different 
“influencing factors” that produce cyanosis. 

a. The circulation in normal resting individuals, when the blood leaves the 
lungs 0.95 per cent or 19 volumes per cent saturated (indicated by stippled 
area) and 0.5 or 1 volume per cent oxygen unsaturated. 

b. In a case of incomplete aeration in the lungs due to pneumonic consolida¬ 
tion, lowered oxygen tension, etc., showing the blood emerging from these 
incompletely saturated (l factor). 

c. In a case of venous-arterial shunt where the blood passes from right to 
left through a cardiac septal defect and venous blood enters the arterial stream 
(alpha factor). 

d. In a case of retardation of flow where deoxygenation is abnormally high 
in the peripheral capillaries at various parts of the surface of the body. (D 
factor.) 

Fig. 8.—A. Diagram showing the influenceon C, the mean capillary oxygen 
unsaturation, of T, the variations in the total oxygen combining power of the 
blood: of D, the oxidation rate during the passage of blood through the 
capillaries; of X, the fraction of total haemoglobin passing unoxygenated 
through unaerated parts of the lungs; and of a, the fraction of venous blood 
passing unaerated through a cardiac defect into the arterial stream. Note 
that normal value of oxygen unsaturation in the capillaries is here shown to 
be 3.5 volumes per cent, and that the threshold value at which raised oxygen 
unsaturation becomes visible is 6.7 volumes per cent. 

B. Diagram showing the influence on C, mean capillary oxygen unsatura¬ 
tion, of simultaneous variations in D and a (the two factors that commonly 
act in combination in the graver cases of congenital cyanosis). (From C. 
Lundsgaard and D. D. van Slyke, “Cyanosis” Medicine, 1923, 2: 1, Figs. 
12-16, 18. Reproduced by permission.) 

Note to Reader: For the clear understanding of these important diagrams 
these authors’ original monograph should be consulted, or the writer’s discus¬ 
sion of this in Blumer’s Bedside Diag., 1928, 2: 438-448.) 

Fig. 9.—Electrocardiogram from the cyanotic patient with tetralogy of 
Fallot shown in Fig. 2, taken March 8, 1927. Leads I, II and III, normal 
rhythm, rate 66; right axis deviation, index —18, angle +140 degrees. P 
wave, lead II, 5 mm. amplitude, 0.15 second duration. Diphasic T wave in all 
leads. (From the article by P D. White and H. B. Sprague, J.A.M.A., 
1929, 92: 787, Fig. 2.) 


40 


PLATE XVI. SYMPTOMATOLOGY OF CONGENITAL CYANOSIS 



Fig. 3. 




tissue 


Tissue 

Capillaries 

a 


tissue 


Tissue 

Capillaries 

b 


> 

5 

Jo 



Usual threshold value of C for 
appearance of cyanosis. Accord¬ 
ing to variations in what we term 
modifying factors this threshold 
i can be higher or lower. 

1 normal values. Brackets In¬ 
dicate variations ordinarily found 
in normal conditions. 


T o 
D = 0 
> = 0.0 
— 0.0 


5 io is 20 *530 j n volumes per cent. 
10 20 ' 


0.5 

0.5 


1.0 “ fractions of total hemoglobin 


1.0 


blood flow 


Fig. 5. 



Lung tissue 


Lung capillaries 




EU 


Fractional total hemoglobin 
In reduced form 
Fractional total hemoglobin 
in oxygenated form 


Fig. 7. 



0123456789 10 
D In volumes per cent. 
B 


Reduced hemoglob¬ 
in passing from the 
veins to the arter¬ 
ies through unae¬ 
rated parts of 
lungs or defective 
heart without tra¬ 
versing the lungs. 


Usual threshold 
for production of 
cyanosis. 


Reduced hemoglobin 
formed by deoxydatlon 
of blood as it passes 
through the tissue cap¬ 
illaries (D). 

Hemoglobin passing In 
reduced form from veins 
to arteries through ae¬ 
rated parts of the lungs 
(h = 0.05). 



Fig. 8, 

41 


Fig. 9 





























































































































































































PLATE XVII 


PULMONARY CONUS STENOSIS AT LOWER BULBAR ORIFICE 

ALL CARDIA SEPTA CLOSED 

The two cases figured opposite are examples of that relatively small group in which retardation of flow leading to stasis and increased 
deoxygenation in the capillaries (Lundsgaard’s D factor), without any complicating venous-arterial {alpha) shunt, was apparently the sole 
cause of the congenital cyanosis that existed. These cases are, however, remarkable in that the seat of the stenosis was not at the pul¬ 
monary valve but some distance below this at the “lower bulbar orifice” forming a capacious separate chamber with a small orifice in its 
floor. In Case 2, two such openings existed, which were the sole means of transit of the venous blood to the lungs, both foramen ovale 
and ductus arteriosus as well as the interventricular septum being completely closed. In both cases, cyanosis was moderate and came on 
late in life, in Case 2 associated with great oedema. 

Stenosis of the pulmonary conus at the lower bulbar orifice, the conus a separate chamber above this, is a well-recognized form of devel¬ 
opmental pulmonary stenosis and was reported upon by Sir Arthur Keith as a remarkable illustration of persistence of the reptilian bulbus 
in the adult human heart. In all the cases described by him, however, and so far as we know in the literature, except the two presented 
here and two reported by Lafitte {Bull, de la soc. anat. de Paris, 1892, 6: 13) and Jackson (TV. Path. Soc. Land., 1893, 44: 29) a large 
ventricular septal defect was associated with some dextroposition of the aorta. In the first case shown opposite (Fig. 1) the heart was 
otherwise normal, but the margins of the constriction were fibrosed and the seat of luxuriant recent vegetations. In the second case (Figs. 
4-8) the heart was the seat of several minor associated anomalies, including a slight dextroposition of the aorta, which was of interest as 
bringing it within the range of Spitzer’s theory. A point of great importance in this second case was the extraordinary fibrosis and degen¬ 
eration of the myocardium of the right ventricle present (Fig. 8), which was ascribed by the authors to the tremendous intraventricular 
pressure that must have existed behind the stenosed orifice in the presence of closure of all the foetal passages. In curious contrast to this 
was the complete absence of such myocardial changes in the first case (Fig. 1) under apparently the same dynamic conditions, for the 
foramen ovale here was also closed. (The writer takes this opportunity of correcting a mistake in the published report of this latter (Thal- 
heimer) case to the effect that the foramen ovale was patent. See reference under Fig. 1.) 


Fig. 1.—Stenosis of pulmonary conus at lower bulbar orifice with ven¬ 
tricular septum and foramen ovale closed. Subacute bacterial endocarditis 
of pulmonary cusps and margins of conus orifice.* S. viridans infection, 
embolic glomerulonephritis. 

A heart laid open to show the interior of the pulmonary conus. The pul¬ 
monary artery is large and is supplied with three well-developed cusps, all of 
which carried polypoid vegetations, one being 1.5 cm. long and hanging free 
in the lumen of the artery. Below the pulmonary ring the conus cavity, 
3 cm. deep with muscular walls, tapers to the slitlike opening in its floor, 
which is surrounded also by large vegetations. From a young married woman 
with a history of mental and nervous disease in sibs, who presented signs of 
heart disease from childhood and a very loud systolic blow maximum over 
the pulmonary area, but who went through two pregnancies successfully and 
was delivered of two normal children. Dyspnoea on exertion with occasional 
fainting spells and moderate cyanosis developed in recent years but no 
clubbing. Death followed septicaemia with infective endocarditis of the 
pulmonary valve and conus and sero-fibrinous pericarditis. Microscopic 
examination showed marked hyperplasia of muscle fibers of right ventricle 
but no areas of fibrosis or marked changes in blood vessels. (From the 
service of Wm. Thalheimer and H. A. Holbrook of Milwaukee, Wis. Speci¬ 
men No. 14.131 r> in the Cardiac Anomaly collection of McGill University. 
Reported by W. W. Eakin and M. E. Abbott, Amer. J. Med. Sci., 1933, 
186: 860.) 

Fig. 2.—Diagram of the course of the circulation in pulmonary stenosis with 
all cardia septa closed. 

Fig. 3.—X-ray of heart of Case 1 (Fig. 1), showing widening in region of 
right auricle, small aortic knob and some enlargement in region of pulmonary 

conus. 

Fig. 4.—Lateral view of head of Case 2 showing vestigial remains of left 
external ear, low growth of hair on neck and facial expression of mental 

deficiency. Note also slitlike opening for external ear, and displaced carti¬ 
laginous nodule at tip of mastoid. 

Fig. 6.—Roentgenograph of heart of Case 2 (Fig. 7), showing enormous 
in, ■ ase in transverse diameter, right border of shadow formed by dilated right 

■ Specimen in the Cardiac Anomaly Collection of McGill University. 


auricle and left by greatly hypertrophied right ventricle, the latter extending 
almost to the left axillary border. Also broadening of shadow at the base 
on the right suggesting dilatation of the superior cava, and marked prominence 
of pulmonary arc on left border (A) due to dilated pulmonary conus. 
Aortic knob also prominent. 

Fig. 6.—Electrocardiogram from this case showing right axis deviation and 
increased amplitude of Q R S deflections. Large P deflections are seen in 
leads I and II. The P-R interval is 0.24 seconds. 

Fig. 7.—Stenosis of pulmonary conus at lower bulbar orifice with complete 
closure of all foetal passages.* 

Anterior view of heart of Case 2 showing relations of great vessels at the base 
and conus of right ventricle laid open. Note thickened endocardium lining 
triangular cavity and minute conus orifices communicating with sinus of ventri¬ 
cle, also hypoplasia of pulmonary valve and trunk and dilated ascending aorta. 

b. Interior of aortic vestibule. Note slight dextroposition of aorta and 
aneurysmal pars mcmbranacea. 

c. Right chambers laid open to show interior of sinus portion of right ven¬ 
tricle. Note massive simple hypertrophy of right ventricle and minute conus 
orifices, also anomalous chordae in right auricle. (Drawing from specimen 
No. 14.132 5 by H. Blackstock, Medical Art Department, McGill University.) 

Fig. 8.—Microphotographs from sections through myocardium of the right 
ventricle below stenosed bulbar orifice in this case, showing diffuse myocardial 
fibrosis and sclerosis of vessels with intimal hyperplasia. Upper: Mallory 
phosphotungstic acid. Lower: Elastic tissue stain. 

From a boy aged 14 with moderate cyanosis and dyspnoea from infancy 
and great anasarca of entire body in later months of life, who presented a 
congenital papilloma of right conjunctiva, bilateral cleft of the hard palate 
and congenital absence of both cars, marked increase of venous pressure, 
pronounced systolic thrill over midprecordium and a loud blowing systolic 
murmur maximum at fourth left interspace. No polycythaemia until shortly 
before his sudden death from circidatory failure when the red count was 
5,500,000. (From a case in the Children’s Memorial Hospital, Montreal, 
reported by W. W. Eakin and M. E. Abbott, Amer. J. Med. Sri-. 1933, 186: 
860.) 


42 


PLATE XVII. PULMONARY STENOSIS AT LOWER BULBAR ORIFICE. ALL CARDIAC SEPTA CLOSED 



Fig. 1. (Case 1) Fig. 3. 




Fig. 5. 


Fig. 6. 


Fig. 4. (Case 2) 


b 


Fig. 7. 


Fig. 8. 




LEFT 


VERTEBRAL ARTERY- 

S. V. C. J 


AORTA 


CONUS ORIFICE "A." 


.CON. OR 

“ B." 


PARS. MEMB. 


OV 


CLOSED FORAMEN 
EUSTACHIAN VALVE 
CONUS ORIFICE‘'B." 


CON. OR. 

■a: 


43 






















PLATE XVIII 


A. PULMONARY AND TRICUSPID STENOSIS WITH CLOSED VENTRICULAR SEPTUM. B. EISENMENGER COMPLEX 

A. Pulmonary Stenosis with Closed Septum. Apart from the rare condition of constriction at the lower bulbar orifice with all cardiac 
septa closed presented in Plate XVII, a group of cases exists of valvular pulmonary stenosis without any other complicating lesions, except, 
in most instances, a patent foramen ovale. This condition is practically always inflammatory, the result of an endocarditis running its 
course in later foetal life after septation has been completed. In these uncomplicated cases the right ventricle is always the seat of a 
marked simple hypertrophy and the right auricle dilated (Fig. 2), but the heart is otherwise normal. The tricuspid orifice is liable to be 
involved in a similar lesion of inflammatory origin (Figs. 3 and 5). Such cases are much less frequent than the developmental type of 
pulmonary stenosis with associated ventricular septal defect and dextroposition of the aorta (PI. XIX), but the circulatory conditions are 
more favorable, in that the chief (and with closed foramen ovale, the only) cause of raised oxygen-unsaturation is retardation of flow with 
increased deoxygenation in the capillaries (Lundsgaard’s D factor), a venous-arterial shunt (Z factor) being either absent, or (where the 
foramen ovale is open) present in a much smaller volume than in the large shunt that takes place through an associated septal defect in the 
so-called “tetralogy of Fallot” (PI. XIX). Cyanosis is therefore usually moderate in degree and of relatively late onset, frequently not 
appearing until after early childhood. The condition tends, too, to become progressive, though recurrent attacks of rheumatic endocarditis 
upon the thickened valve are a common event. This is well illustrated in the classic case shown in Fig. 6, in which cyanosis first appeared 
in the ninth year. Clubbing became however, a very marked feature, the capillary factor evidently predominating. For the same reason 
the average duration of life is longer than in the tetralogy, although Paul White’s famous musician (PI. XVII), who presented a classic 
example of the latter combination, broke the record for both groups by surviving into his sixtieth year. Because of the superficial location 
of the lesion and of the fact that the current is not deflected into the aorta through a septal defect, physical signs, consisting of a harsh 
systolic murmur with a pronounced thrill, maximum at the pulmonary area, are usually very marked. There may be an enlarged pul¬ 
monary arc from dilatation of the pulmonary trunk above the valve; and right predominance is a marked feature of the electrocardiogram. 

B. Eisenmenger Complex. This term has been used by the writer, in default of a better, to designate an unusual combination, which 
was first diagnosed during life by von Sehrotter and was reported by Eisenmenger ( Ztsch. f. klin. Med., 1897, 32 : 1), of ventricular septal 
defect with dextroposition of the aorta without any pulmonary stenosis or hypoplasia. This rare condition is to be distinguished from the 
much more frequent tetralogy of Fallot, in which pulmonary stenosis is an essential feature of the otherwise identical combination. The 
term “biventricular aorta” has been proposed by Blackford for those cases of reitende aorta in which this vessel rides astride the septum 
arising from both ventricles above the defect, but it does not cover others in which it springs “dextroposed” entirely from the right ven¬ 
tricle. As both these types form a single clinical group, we are still in need of a generic name, if that adopted by the writer is discarded. 
Cyanosis and clubbing in these cases are moderate and set in late and the latter feature may be absent. Physical signs are those of the 
septal defect, and the systolic murmur is usually heard in the back but not in the vessels of the neck. A classic example of this condition 
in a man aged 60 years with atheroma of the dilated pulmonary artery and calcification and insufficiency of the pulmonary valve has been 
reported lately by H. L. Stewart and B. L. Crawford ( Amer. J. Path., 1933, 9: 637). 


A 

Fig. 1.—Diagram of the circulation in pulmonary stenosis with closed 
ventricular septum and patent foramen ovale. (From Abbott and Dawson, 
Internal. Clin., 1924, 4: 172, Fig. 11.) 

Fig. 2.—Heart showing pulmonary stenosis from inflammatory fusion of 
cusps: ventricular septum closed. A classic example of this condition in an 
adult heart. The right ventricle is hypertrophied and the right auricle 
greatly dilated. (Reproduced by permission from “Les affections con- 
genitales” by J. Fleury in Encyclopedic Medico-Chirurgicale, 1934, 12: 4075, 
Fig. 293.) 

Fig. 3.—Pulmonary and tricuspid stenosis of inflammatory origin—ven¬ 
tricular septum closed.* Great hypertrophy of right ventricle and dilatation 
of right auricle. Male infant aged 4 months. Moderate cyanosis becoming 
extreme before death from bronchitis, small patency of foramen ovale. 
(Reported by Wm. Osier, Montreal Gen. Hosp. Repts. Clin, and Path., 1880, 
1: 185, PI. IV, Figs. 1, 2. Specimen No. 7422 in the Cardiac Anomaly Col¬ 
lection of McGill Museum.) 

Fig. 4.—Diagram showing circulation in tricuspid and pulmonary stenosis 
with closed septum. The pulmonary artery is here hypoplasic, the right 
ventricle greatly hypertrophied and the right auricle dilated. (Drawing by 
P. Lariviere.) 

Fig. 5.—Heart in tricuspid and pulmonary stenosis of inflammatory origin 
o pinhole pulmonary orifice surmounted by recent vegetations. Great 
rtrophy of right ventricle and auricle.* 

- The fused pulmonary cusps with raphe a,a,a; b, central pinhole orifice 
surmounted by vegetations; (2) conus of right ventricle showing a, puckering 
oi myocardium; b, thickened endocardium; (3) lower bulbar orifice formed 
by a, ci is-s supraventricularis, b, trabecula supramarginalis; (4) interventricu¬ 
lar septum. 

From a young girl aged 14 with severe dyspnoea and marked cyanosis and 
* Specimen in the Cardiac Anomaly Collection of McGill University. 


clubbing, healthy and active, until the age of nine, when cyanosis first began 
following sore throats. Precordial bulging, rough systolic thrill maximum 
at third left interspace, rough systolic murmur maximum at apex and pul¬ 
monary area. R.B.C. 7,600,000, leucocytes 22,000. Died in dyspnoeic 
attack. (Reported by M. E. Abbott, D. S. Lewis and W. W. Beattie, Amer. 
J. Med. Sci., 1923, 165 : 636, Case 1. Drawing by W. W. Beattie of Specimen 
No. 9905 in McGill Museum.) 

B 

Fig. 6.—Diagram showing the circulation in localized defect of the inter¬ 
ventricular septum with dextroposition of the aorta. (From Abbott and Daw¬ 
son, Internat. Clin., 924, 4: 170, Fig. 9.) 

Fig. 7.—Heart, x-ray and electrocardiogram from a case of interventricular 
septal defect with dextroposition of aorta and great dilatation of pulmonary 
artery (Eisenmenger complex). Death from cerebral abscess. 

a. View of exterior of heart, showing the great hypertrophy of the right 
ventricle especially of the conus and great dilatation with hyperplasia of 
pulmonary artery. 

b. Teleoroentgenogram of the heart, showing huge pulmonary arc and 
right-sided hypertrophy. 

c. The left ventricle laid open to show a large interventricular septal defect 
2 cm. across with sclerosed border, and the somewhat hypoplasic dextroposed 
aorta. A, right anterior (coronary) aortic cusp; B, ventricular septal defect. 

d. Electrocardiogram showing right preponderance. Split R and S, and 
slight delay in conduction (bundle-branch block). 

From a young man aged 21 years, with hoarseness and aphonia since age 
of 13 (from pressure of the huge pulmonary conus on recurrent laryngeal 
nerve). Slight cyanosis and dyspnoea on exertion but no clubbing. Loud 
systolic murmur over heart, maximum at pulmonary area and soft diastolic 
to left of sternum. Cerebral symptoms developed two weeks before death 
from large abscess in right front parietal region (streptococcus haemolvticus). 
The septal defect measured 2 cm. across, the pulmonary artery 8 cm. and the 
aorta 6 cm. in circumference. (From the Medical Clinic of the Clifton Springs 
Sanatarium, reported by Baumgartner and Abbott, Amer. J. Med. Sci., 
1929, 177: 639.) 


44 


PLATE XVIII. A. PULMONARY AND TRICUSPID STENOSIS WITH CLOSED VENTRICULAR SEPTUM. B. EISENMENGER 

COMPLEX 



Venous-arterial shunt. 


Fig. 1. 


Fig. 4. 


\ 


Venous-arterial shunt. 

Fig. 6. 




Fig. 7d. 


Fig. 3. 


45 




















































PLATE XIX 


PULMONARY STENOSIS AND ATRESIA WITH DEFECT OF VENTRICULAR SEPTUM (TETRALOGY OF FALLOT) 

The combination presented in this plate of pulmonary stenosis and septal defect with dextroposition of the aorta and hypertrophy of 
the right ventricle, conveniently known as the "tetralogy of Fallot,” is almost invariably present in the developmental form of this lesion 
and contrasts sharply in most respects with the inflammatory, purely valvular type of pulmonary stenosis (PL XVIL4)./' In the group 
under consideration the entire pulmonary tract is usually narrowed and hypoplasic, the pulmonary valve is bicuspid and its leaflets fre¬ 
quently fleshy in character and the conus is narrow and deformed or constricted at its lower bulbar orifice, while the wide thick-walled aorta 
rides above the large defect in the interventricular septum receiving blood through this from both ventricles. The whole appearance is 
highly suggestive of the relationships that would result from the uncovering of the right reptilian aorta and obliteration of the left in the 
delayed torsion of Spitzer’s theory (his types I and II of transposition) and is of great interest in this connection. 

As Fallot himself pointed out, this is by far the commoner type of pulmonic stenosis, and it was present in 77 per cent of the 110 cases 
of pulmonary stenosis and in 66 per cent of the 40 cases of atresia classed as the primary lesion in the writer’s chart of 1000 cases analyzed 
(pp. 60-61). It is also the most important from the clinical standpoint, because of the relative frequency with which these patients, despite 
the high degree of oxygen unsaturation which they invariably present, attain adult life, the maximum age being approximately 25 years 
and the average in 83 cases being 12 3 ^ years, if we except Lafitte’s patient who was 36, and Paul White’s musician who reached the unprec¬ 
edented age of 59 years 9 months. The raised pressure in the right ventricle behind the stenosis sends a large venous-arterial shunt into 
this dextroposed aorta through the defect, and the effect of this in the circulation is intensified by increased deoxygenation at the periphery 
due to the obstruction at the pulmonary orifice, Lundsgaard’s D and alpha factors acting together to raise oxygen unsaturation high above 
its threshold value. In addition the long standing capillary changes and the effect of the polycythemia and great hypertrophy of the right 
ventricle that develop as compensatory features combine to produce the most pronounced symptomatology of morbus coeruleus ever seen 
in adult life, with enormous clubbing of the extremities and a polycythemia varying from 7,000,000 to 12,000,000. 


On the subject of congenital cyanosis see the following references: The pathogenesis and symptomatology of congenital cyanosis and calculation of the venous- 
arterial shunt: F. P. Parkes Weber, and G. Dorner, Lancet , Lond., 1911, 1: 150; D. Christiansen and Haldane, J. Physiol., 1914, 48: 244; W. C. Stadie, J. Exp. 
Med., 1919, 30: 215; J. A. Harrop, J. Exp. Med., 1919, 30: 241; J. S. Haldane, Respiration, 1922, Yale Univ. Press; J. M. Campbell, G. H. Hunt and E. P. Poulton, 
J. Path, and Bad., 1923, 26: 234; C. Lundsgaard and D. D. van Slyke, Medicine, 1923, 2:1; J. Meakins, L. Dautrebande and W. J. Fetter, Heart, 1923, 10: 153; 
Weiss and Lowbeer, Wien. Arch. f. inn. Med., 1924, 7: 367; W. Gravinghoff, Monatschr. f. Kinderheilk., 1927, 35: 237; J. Barcroft, The Respiratory Function of 
the Blood, 1928, London; M. E. Abbott and W. T. Dawson, Internal. Clin., 1924, 4: 156; M. E. Abbott, Blumer’s Bedside Diag., 1928, 2: 432-458; D. W. Rich¬ 
ards, C. B. Riley and Hiscock, Arch. Int. Med., 1931, 47: 484; H. N. Segall, Amer. Heart J., 1933, 8: 628; I. Berconsky, Rev. Sud. Amer. de Med. el Chir., 1934, 
5: 193; P. Cossio and I. Berconsky, Arch. mal. du coeur, 1935, 28: 19. The capillary changes: A. Krogh, The Anatomy and Physiology of Capillaries, 1922, 
Yale Univ. Press; E. Wollheim, Ztschr. f. klin. Med., 1928, 108: 248; W. Rcdisch and H. Roesler, Wien. Arch. f. inn. Med., 1929, 16: 463. 


Fig. 1.—Diagram of the circulation in pulmonary stenosis with ventricular 
septal defect and dextroposition of the aorta (tetralogy of Fallot). (From 
Abbott and Dawson, Internal. Clin., 1924, 4: 173, Fig. 12.) 

Fig. 2.—Pulmonary stenosis with associated defect of the interventricular 
septum, dextroposition of the aorta and hypertrophy of right ventricle. View 
of interior of right chambers. The large aorta arises two-thirds from the right 
and one-third from the left ventricle, receiving the contents of the latter 
through the large defect (indicated by arrow). The hypoplasic pulmonary 
has only two cusps and its conus is small with narrow lower orifice. From 
a girl of 12 with marked cyanosis and clubbing. (Drawing by Fraser B. 
Gurd from Specimen 873 in the Cardiac Anomaly Collection of McGill Uni¬ 
versity. Reported by M. E. Abbott, Blumer’s Bedside Diag., 1928, 2: 469, 
Fig. 320.) 

Fig. 3.—Heart-signs record from a case of tetralogy of Fallot, showing seat 
of maximum intensity of prolonged low-pitched systolic murmur with accom¬ 
panying thrill at second left interspace, with zone of transmission and higher 
pitched (second) murmur at angle of left scapula. (Diagram supplied by 
H. N. Segall. Reference under PL XIII, Fig. 6.) 

Fig. 4.—Illustrations from C. P. Howard’s case of the tetralogy of Fallot. 

a. The electrocardiogram from this case. Shows normal rhythm rate 75, 
marked right axis deviation, high P wave and slurring and notching of R in 
lead II. 

b. View of aortic vestibule of left ventricle showing large septal defect 
and the dextroposed aorta above this, which receives blood from both cham¬ 
bers. Note the anomalous chordae in the floor of the defect, also fusion and 
recent endocarditis of the aortic cusps above it. 

c. View of conus arteriosus of right ventricle showing marked stenosis of 
his region and of pulmonary valve with hypoplasia of pulmonary artery and 

great hypertrophy of myocardium. 

From a man aged 23 with marked cyanosis from childhood and extreme 
clubbing of nose, fingers and toes. Was mentally deficient, conjunctivae 
congested, breathing labored, red cell count 10,280,000, haemoglobin 146 per 
cent, pronounced systolic thrill and rough murmur over precordium and in 
back, not in neck, maximum at second left interspace, pulmonary second 
sound faint. Blood pressure 110-60. Subject to curious epileptiform 


seizures thought to be due to thromboses in capillaries of brain and cerebral 
anoxaemia and died in one of these accompanied by frothy haemorrhage from 
the lungs (oedema). Extensive estimations were made of blood gas findings 
and calculations of the venous shunt were done by I. M. Rabinowitch. 
(Reported by H. N. Segall, Amer. Heart J., 1933, 8: 628. Specimen in the 
Collection of the Montreal General Hospital, art drawing by Hortense 
Douglas.) 

Fig. 6.—Orthodiagram from Roesler’s case of tetralogy of Fallot showing 
coeur en sabot, enlarged cardiac shadow to left of median line and prominent 
aortic knob and double apices on left border. The arrows indicate the two 
apices of the heart seen in this condition, the lower being that of the greatly 
hypertrophied right ventricle which curves upward from below, and the 
upper that of the smaller left chamber. (From the article by H. Roesler. 
Wien. Arch. f. inn. Med., 1928, 15: 507.) 

Fig. 6.—Pulmonary atresia with defect of the interventricular septum and 
widely patent ductus arteriosus. The defect is guarded on the side of the right 
ventricle by a false valve anchored to its walls by two anomalous chordae. 
From a male infant aged 13 days, cyanotic from birth and died in dyspnoeic 
attack. (From the specimen in the Cardiac Anomaly Collection of McGill 
University and reported by William Osier, Montreal Gen. Hosp. Repls., 1880, 
1: 186, Case 3.) 

Fig. 7.—Roentgenogram in right oblique diameter from a case of pulmonary 
atresia with ventricular septal defect and dextroposition of aorta (tetralogy). 

Note the characteristic coeur en sabot effect from the great hypertrophy of the 
right ventricle. From a cyanotic male infant aged one year. (Reported 
by M. E. Abbott, Blumer’s Bedside Diag., 1928, 2: 473, Fig. 325.) 

Fig. 8.—Diagram of the circulation in pulmonary atresia with aorta from 
right ventricle and ventricular septal defect. Note the aplasic left chambers 
and the huge size of the dextroposed aorta. (From Abbott and Dawson, 
Internal. Clin., 1924, 4: 174.) 

Fig. 9.—Electrocardiogram in tetralogy with high degree partial block. 

Sinus rhythm with 2:1, 1:1 partial A-V block, marked right axis deviation. 
High Pi waves especially in Lead II; QRS slurred in all leads, and deep 
Q3. (Tracing and reading from G. Nicolson.) 


46 


PLATE XIX. PULMONARY STENOSIS AND ATRESIA WITH DEFECT OF VENTRICULAR SEPTUM (TETRALOGY OF FALLOT) 



Fig. 1. 



Fig. 2. 




Fig. 6. 



Fig. 4c. 


Fig. 8. 






















































































































































PLATE XX 


PULMONARY ATRESIA WITH CLOSED VENTRICULAR SEPTUM. AORTIC, MITRAL AND TRICUSPID ATRESIA 

Pulmonary atresia with closed septum is always, like the less severe grades of pulmonary constriction with this combination (PI. 
XVIIIA), of inflammatory origin, due to a valvular endocarditis or, more frequently, to an extensive fibrosis of the myocardium of the 
underlying conus, setting in in later foetal life after the heart chambers are completely separated. The conditions of the circulation are 
here much more serious than in the presence of an associated septal defect, for, in the absence of any direct opening into the dextroposed 
aorta, the venous blood from the right auricle must pursue a circuitous path through the foramen ovale to the left chambers, and thence 
via the ductus (which is always patent) to the lungs. These are the cases of extreme cyanosis from birth, the true blue baby of morbus 
coeruleus in whom life is sustained for only a few days or weeks or occasionally months. [A curious exception to this was Costa’s case 
(Clin. Med. Ital., 1930, 61: 567), of a cyanotic youth with marked clubbing of the extremities who attained his twentieth year, in whom 
therefore some source of collateral circulation must have existed, else oxygenation could not have been maintained.] These infants usually 
present a purplish leaden hue of skin with marked dyspnoea and dyspnoeic attacks, and death may take place in one of these or suddenly 
without apparent cause. The right ventricle is aplasic and the right auricle and left chambers are enormously enlarged while an aorta of 
great size carries on the entire work of both circulations with the help of the patent ductus and foramen ovale. Such cases were described 
by Shapiro (Arch. Path., 1930, 9: 54) and subsequently by Kugel (l.c. under Fig. 3 b below). 

The opposite condition of aortic atresia with closed se-ptum these authors likewise described as truncus solitarius pulmonalis (shown in 
Figs. 5, 6 and 9 opposite and in PI. XXIII, Fig. 4a). Here the right chambers are in their turn huge and the large pulmonary artery trans¬ 
mits the blood to the general circulation through the widely patent ductus, while the aorta is reduced at its origin to a blind cord giving off 
the coronary arteries from its patent distal end and the vessels of the arch higher up, the partly aerated blood reaching these from the 
pulmonary artery in a retrograde stream through the patent ductus (Fig. 9), while the mitral orifice and left ventricle are aplasic. Cyanosis 
is extreme and the average duration of life in 12 cases was only four days. Most of these cases are due as in this type of pulmonary atresia 
to a foetal myocarditis with resultant scarring in the conus wall just below the valves (PI. XXIII, Fig. 45), as was pointed out by de Zalka 
(Frankf. Ztschr. f. Path., 1924, 30: 144). 

Mitral and tricuspid atresia are usually of developmental origin, and a septal defect is nearly always associated, producing a special 
type of cor triloculare. The roentgenogram in tricuspid atresia shows a left-sided enlargement (Fig. 8) that is characteristic and of diag¬ 
nostic value, as is the left axis deviation seen in the electrocardiogram in these “right-sided lesions” of the cyanotic group. (See on this 
subject the article by J. Rihl, K. Terplan, and F. Weiss, Med. Klin., 1929.) Mitral atresia is very rare and is usually associated with 
grave somatic defects. Donnelly’s case was, however, a true aplasia (J.A.M.A., 1924, 82: 1318). 


Fig. 1.—Diagram showing course of the circulation in pulmonary atresia 
with closed ventricular septum, patent foramen ovale and patent ductus. 

(From Abbott and Dawson, Internat. Clin., 1924, 4: 181, Fig. 20.) 

Fig. 2.—Pulmonary atresia of inflammatory origin. Ventricular septum 
closed. Ductus arteriosus and foramen ovale widely patent. Left chambers 
and right auricle dilated and hypertrophied. The large aorta rises in its 
normal position to the right and posteriorly and the small pulmonary artery 
passes up on the left to connect with the descending arch through the widely 
patent ductus (1). The interventricular groove is displaced upward on the 
right outlining the aplasic right ventricle, the left chamber forming the apex 
and anterior four-fifths of the heart. (2) The atresic pulmonary orifice. 

From a cyanotic infant dying on the ninth day. Case of Edward Bassen, 
New Haven. (Reported by M. E. Abbott, Bull. Int. Assn. Med. Mus., 1924, 
10 : 111 .) 

Fig. 3.—Congenital atresia of the pulmonary orifice, with closed inter¬ 
ventricular septum, absence of tricuspid valve, aplasia and aneurysmal dila¬ 
tation of right ventricle, patent foramen ovale and ductus arteriosus; truncus 
aorticus solitarius; single coronary artery. 

a. The paraffinized heart showing the huge truncus aorticus and enlarged 
right auricle with cystic dilatation of right ventricle, bifid apex formed by 
massive left ventricle, and pulmonary artery forming a solid cord. 

b. Roentgenogram showing the huge cardiac shadow of peculiar egg-shaped 
appearance formed by the dilated right auricle on right above and by the left 
ventricle on left below. 

From an infant cyanotic and dyspnoeic from birth with some clubbing 
and a rough systolic murmur of peculiar resonant quality, maximum over 
second left interspace transmitted to apex; dying, aged 6 months, from 
erysipelas of the vulva. At autopsy there was no trace of the tricuspid valve 
or its attachments. (From M. A. Kugel, “Two cases of truncus solitarius 
aorticus (pulmonary atresia),” Amer. Heart J., 1931, 7: 262.) 

Fig. 4.—Diagram showing circulation in aortic and mitral atresia with 
iplasia of left ventricle, etc. (From Abbott and Dawson, Internat. Clin., 
1924, 4: 182, Fig. 21.) 

Fig. 5.—Congenital atresia of aortic orifice from foetal myocarditis, ven¬ 
tricular septum entire, patent foramen ovale, persistent ductus, mitral aplasia, 
rudimentary left ventricle and auricle. From a baby boy 3 days old, the first 
of twins. Cyanosis developed on second day and did not decrease on adminis¬ 
tration of oxygen, no heart murmurs elicited. (Reported by H. R. Wesson 
and D. C. Beaver, J. Tech. Meth., 1934, 14: 86, Fig. 2.) 


Fig. 6.—Congenital aortic atresia and mitral aplasia with marked con¬ 
centric hypertrophy of left ventricle and hyperplasia of lining of mural endo¬ 
cardium. Ventricular septum entire, ductus arteriosus widely patent. 

a. The ascending aorta and its relationships with the ductus arteriosus 
and pulmonary trunk. Shows interior of small narrow aorta with occluded 
aortic valve giving off its branches and emptying through its dilated isthmus 
into the patent ductus arteriosus which connects it with the descending aorta. 
(1), anterior view; (2), posterior view. 

b. Posterior view of heart showing interior of the small left auricle and 
aplasic ventricle, in a state of marked concentric hypertrophy, lined by 
thickened mural endocardium. Apex markedly bifid, formed entirely of 
right ventricle. 

From a male infant, aged 15 days, weighing nine pounds and deeply cyanotic 
from birth. Cardiac dulness to left axilla and subclavicular region and 
systolic murmur over upper left chest. Died suddenly. (From Children’s 
Memorial Hospital. Reported by F. W. Wiglesworth, J. Tech. Meth., 1936, 
16: 153. Drawings by P. Lariviere, Medical Art Department, McGill 
University.) 

Fig. 7.—Diagram of circulation in tricuspid atresia with defect of inter¬ 
ventricular septum. (Drawing by P. Lariviere.) 

Fig. 8.—Roentgenogram of the thorax, anterior view, in a case of tricuspid 
atresia. Showing beautifully the typical cardiac shadow in this condition, 
the enlargement being entirely in the left thorax with absence of pulmonary 
arc and wide aortic base. 

From a cyanotic male infant, aged 6 months, with shortening of right fore¬ 
arm and absence of thumb. Red cells, 6,850,000. Presented a loud systolic 
murmur, maximum over left base with marked precordial thrill. Left-axis 
deviation with very high PI and PII and deep notching of QRS complex. 
Diagnosed during life as a three-chambered heart. Tricuspid orifice absent, 
large persistent ostium primum, small defect of interventricular septum. 
(From L. M. Blackford and L. D. Hoppe, “Functionally two chambered 
heart,” Amer. J. Dis. Child., 1931, 41: 1111.) 

Fig. 9.—Atresia of ascending aorta which takes origin from coronaries. 
Pulmonary artery forms descending aorta. Patent ductus arteriosus. Left 
subclavian below ductus. A female infant aged 4 days, cyanotic at birth, 
sudden death. (From the Maternity Service of the Royal Victoria Hospital. 
Reported by M. E. Abbott, Nelson’s Loose-leaf Med., 1932, 4: 297.) 

Fig. 10.—Roentgenogram of the case seen in Fig. 6, showing marked car¬ 
diac enlargement especially at left upper border. 


48 


PLATE XX. PULMONARY ATRESIA WITH CLOSED SEPTUM. AORTIC, MITRAL AND TRICUSPID ATRESIAS 




Fig. 1. 


Fig. 4. 




Fig. 2. 



? fUtt 


34f- 


Left su/bcla 
Left coroti 
Innominate A 


Ascendinqr 

aorta- 


Pulmonary- 

artery 


^'Descending aorta 


Ductus 

arter io3cis 


-Left auricle 
For ame n oval e 


Left ventricle 





Fig. 3b. 


Fig. 6b. 


49 


Fig. 10. 























PLATE XXI 


A. PERSISTENT OSTIUM ATRIO-VENTRICULARE COMMUNE. B. COR TRILOCULARE BIATRIATUM AND 

BI YEN TRICULARE. 


A. Combined partial defect of the lower part of the interauricular (persistent ostium primum) and upper part of the interventricular 
septa, constituting the so-called 'persistent ostium atrio-ventriculare commune, forms a well-recognized group of anomalies, in which a more or 
less free communication exists between all four chambers of the heart, which functions, where the.septal defect is large, as a biloculate organ 
(“incomplete double heart”). Cleavage of the anterior mitral and deformity of the tricuspid segments is a constant feature of persistent 
ostium primum and where the interventricular septum is also largely defective a complete separation of each of these cusps into two halves 
occurs, the contiguous parts of which are continuous each with the other over the free septal border (Fig. 2). Unless the septal defect is 
very large, cyanosis is absent except as a transient or terminal feature and a loud systolic murmur over the midprecordium and at apex 
is commonly present. The condition is explained as a failure of upward growth of the ventricular septum combined with an arrest of 
fusion of the auriculo-ventricular endocardial cushions with the free lower border of the septum primum. A feature of extraordinary 
interest is the frequent combination of mongolian idiocy with this form of defect and with persistent ostium primum per se (Figs. 2, 3, 4 
opposite and PI. XIV, Fig. 8). This combination calls for explanation. 

B. Absence or rudimentary development of one or other of the cardio-vascular septa is the underlying anatomical feature of the group 
of cases which form the subject of this group and that following (PI. XXII). In these cases of biloculate and triloculate heart, the main 
factor in the production of raised oxygen unsaturation is the admixture of venous with arterial blood that takes place in the common 
chamber (alpha shunt). The degree of this venous admixture and the cyanosis that may result therefrom vary, however, according to the 
location and extent of the defect. Thus in complete absence of the interauricular septum (cor triloculare biventriculare) without associated 
anomalies, little effect will be observed, for the reason that here, just as in localized defects of the auricular septum, the shunt is arterial- 
venous with possible reversal of flow and the advent of transient or terminal cyanosis. Absence of the interventricular septum (cor trilocu¬ 
lare biatriatum) , on the other hand, leads inevitably to a free admixture of venous with the arterial blood within the common ventricle 
and to the transmission of this mixed current into the systemic circulation. Cyanosis in these cases is moderate in degree in the absence 
of associated anomalies ( alpha shunt without D factor), the signs of oxygen unsaturation setting in relatively late, with clubbing absent or 
slight, and the subjects having a fair expectation of life until the third or fourth decade (highest age on record 35 years). In a special 
group of these cases of great interest (Fig. 9 opposite), an anomalous septum cuts off a small supplementary cavity giving off the transposed 
aorta (Spitzer’s Type IV of transposition). In the Holmes’ heart, which is still unique in the literature (Fig. 6), the pulmonary artery 
arose from this chamber, and the anomalous septum was believed to be the malposed ventricular one (Ngai, l.c. below). 


(For bibliography on this subject see page 38.) 

A 

Fig. 1.— Diagram of the circulation in persistent ostium atrio-ventricular 
commune. Note the central opening communicating with all four chambers 
guarded laterally by single mitral and tricuspid segments. (Drawing by 
P. Lariviere, Medical Art Department, McGill University.) 

Fig. 2.—Persistent ostium atrio-ventriculare commune with completely 
cleft anterior mitral and septal tricuspid segments, continuous with each other 
over free border of defective interventricular septum.* Valvular endo¬ 
cardium redundant. Bifid apex. From a female infant, aged 5 months, 
product of the mother’s fourteenth pregnancy, presenting mongoloicl facies 
and systolic murmur over whole precordium and terminal cyanosis. Death 
from bronchopneumonia. (Specimen No. 11353, presented to the writer 
from the Pathological Service of the New York Hospital by Dr. Robert A. 
Moore. Drawing by P. Lariviere.) 

Fig. 3.—Roentgenograph in left oblique diameter in a case of persistent 
ostium atrio-ventriculare commune in mongolian idiocy. Shows great 
enlargement of the cardiac shadow in the transverse diameter. From a female 
infant, aged one year, dying of lobular pneumonia. Mongolian facies, flat¬ 
tened nose, bilateral internal strabismus and slant eyes with foetal type of 
detachment of retina. Heart showing persistent ostium atrio-ventricular 
commune surrounded by five distinct cusps and no other cardiac anomaly. 
(Reported by Louise Meeker, J. Tech. Meth., 1935, 14: 72, Fig. 1.) 

Fig. 4.—Persistent ostium atrio-ventricular commune in a case of mitral 
atresia and absence of transverse aortic arch.* The hypoplasic ascending 
aorta ends after giving off the innominate and left carotid, and the bicuspid 
pulmonary artery forms the descending arch through a widely patent ductus. 
From a cyanotic infant dying at 15 hours, showing stigmata of mongolian 
idiocy. Mother fifth para, aged 43 years. (From the Maternity Service 
Division of the Royal Victoria Hospital, Specimen No. 9835, reported by 
M. E. Abbott, Nelson’s Loose-leaf Med., 1932, 4: 223, Fig. 10.) 

B 

Fig. 5.—Circulation in complete absence of the interventricular septum 

triloculare biatriatum). (From Abbott and Dawson, Internal. Clin., 

1921, 4: 178, Fig. 18.) 

Fig. 6.—Dr. Holmes’ famous case of cor biatriatum triloculare with mal- 
.posec. ventricular septum cutting off a small cavity giving off the pulmonary 
artery in normal (not transposed) relations.* 

a. Anterior view of the heart laid open to show the interior of the large 
common ventricle and the small supplementary chamber which communi¬ 
cates with the large one by a diamond-shaped defect in a short muscular 

* Specimen in the Cardiac Anomaly Collection of McGill University. 


rudimentary septum and gives off from its left upper border the pulmonary 
artery (seen cut across on the opposite side of the picture). The aorta arises 
posteriorly in the median line. The right auricle is hugely dilated. 

b. Diagrammatic sketch by R. Tait Mackenzie, showing course of the 
circulation in this case, the mitral and tricuspid orifices opening into the 
common ventricle and the small chamber situated above and anteriorly giving 
off the pulmonary artery in its normal position. 

From a young man aged 22 with moderate cyanosis from childhood, increas¬ 
ing on exertion, who suffered from attacks of palpitation, shortness of breath 
and pain over heart, in one of which he died. (From Specimen No. 14.123 1 , 
reported by Andrew F. Holmes, first Dean of the Medical Faculty of McGill 
University. Tr. Edin. Med. Chi. Soc., 1824; republished by M. E. Abbott, 
Montreal Med. J., July, 1901; also in Osier’s Modern Med., 1927, 4: 702; 
Nelson’s Looseleaf Med., 1932, 4: 278.) 

Fig. 7.—Diagram of the circulation in cor triloculare biventriculare with 
arterial trunks transposed. (Drawing by P. Lariviere.) 

Fig. 8.—Cor triloculare biventriculare in a case of isolated mirror-picture 
dextrocardia with persistent omphalo-mesenteric bay and displaced right 
pulmonary veins, a. anterior, b. posterior view. 

From a cyanotic infant aged 17 days. Faint prolonged systolic murmur 
at base to right of sternum, heart greatly enlarged. The pulmonary artery 
formed the descending aorta through the widely patent ductus. Six super¬ 
numerary splenules. (Reported by Bret Ratner, M. E. Abbott and W. W. 
Beattie, Amer. J. Dis. Child., 1921, 22: 508.) 

Fig. 9.—Cor biatriatum triloculare with malposed septum cutting off a 
small separate chamber which gives off the transposed aorta. The heart 
laid open to show the interior of the large common ventricle, at the left upper 
angle of which is seen a short rudimentary septum cutting off the small sepa¬ 
rate chamber which forms a definite bulb around the base of the large aorta, 
which vessel arises from it in transposed relations and is coarctated at the 
isthmus. 

The patient was a well-developed boy aged 9 years, who presented no 
cyanosis except when in swimming and had always been active. There was 
asymmetry of the left chest with flaring of the lower ribs and precordial bulg¬ 
ing and a widely diffused heaving impulse; also a loud systolic murmur maxi¬ 
mum at the apex with middiastolic roll and occasional extrasystoles. At 
autopsy the heart weighed 700 gm., the ventricle was 22 mm. thick, the small 
chamber 3 cm. deep, and the aorta was 2 cm. in diameter at its origin but 
narrowed at the left subclavian to 8 mm. Foramen ovale patent, ductus 
closed. (From an unpublished case in the Children’s Service of the Massa¬ 
chusetts General Hospital. Reported to the writer by M. Moriarty Glendy, 
R. E. Glendy, and P. D. White. Drawing by Muriel McLatchie, Medical 
Artist of above hospital.) 


50 


PLATE XXI. A. PERSISTENT OSTIUM COMMUNE. B. COR TRILOCULARE BIATRIATUM AND BIVENTRICULARE 



Fig. 1. 



(Fig. 2. 




V S. 


Venous-arterial shunt. 


Fig. 7. 



Fig. 5. 


Fig. 6a. 


rud. car 



Aorta 


P. A 


L. V. 


Left Subclavian- 
Innominate— | 

H.Aortic Arch 
Isthmus Kor.A- 
Patent Du c.f- 

H.Pul__ 

Descending 

Apr. 


Coonon Carotid 


A.w—L. Pulmonic'. 


RIOHT SIDE 



L. Appendix. 


LEFT SIDE 


Descending Aorta— 


Fig. 8a. 


Common Carotid- 

L. Subclavian- 

Isthmus Aortae-—J-- 

L.PulmoniC' 
Ascending Aorta— 

L.Pul.V.-- 


—Innominate 


R.Aortic Arch 
—n —R.Pulmonic 



Superior V.C. 
--R.Pul.V. 


—Omphalo- 
Mesenterio 
Bay 


1—Inferior V.C, 
RIGHT SIDE 


Descending Aorta 


—Hepatic 
Radicles 


Fig. 8b. 



Fig. 4. 


Fig. 6b. 


Fig. 9. 


51 























































PLATE XXII 


A. COR BILOCULARE. B. PERSISTENT TRUNCUS ARTERIOSUS 


A. Complete absence of both auricular and ventricular septa, true cor biloculare, is a rare condition, usually associated with grave anom¬ 
alies such as persistent truncus (Fig. 7), displaced pulmonary veins, etc. As such it constitutes one of the three most extreme forms of 
morbus coeruleus that exist, the other two being pulmonary atresia and transposition of the great trunks both with closed ventricular 
septum (PI. XVIII and XXIII). Such subjects present profound cyanosis from birth and die in very early infancy. In some cases, how¬ 
ever, when a free collateral circulation or an associated transposition has provided a more favorable path for the circulation, early adult 
life may be attained. The highest age on record is that of Rudolf’s patient, a cyanotic girl with marked clubbing and dyspnoea who died 
at 16 of pulmonary tuberculosus; and that of Wood and Williams, a mulatto servant maid presenting the same picture and dying at 15 of 
purulent meningitis. In the former case the great trunks were transposed, but in the latter apparently not. 

B. Persistent Truncus Arteriosus. In this interesting condition either the aortic septum is completely absent or the single large 
arterial trunk that emerges from the base of the right ventricle presents on its interior wall a crescentic ridge indicating its beginning septa- 
tion (partial truncus, Figs. 36 and c). A ventricular septal defect is always associated, and the blood supply to the lungs is derived from 
larger branches given off from the primitive truncus well above the aortic valve. In addition a collateral circulation to the limbs is usually 
developed either from the bronchial or oesophageal arteries or through anomalous vessels from the descending aorta. As a result these 
cases fall clinically into the group of marked but not extreme cyanosis, with onset of symptoms frequently delayed until the end of the 
first year in life and with clubbing as a marked feature. The average duration in 21 cases was 4 years but the case by Carr, Goodale and 
Rockwell lived to the age of 36. A systolic murmur probably generated at the septal defect is usually present. 

In the classic type of this condition first described by Preisz, the large anomalous trunk is supplied with four semilunar cusps, and 
this is theoretically to be expected; for in embryonic life the primitive aorta has four distal bulbar swellings, two of which become sub¬ 
divided in the descent of the aortic septum, making six valvular segments, three in the aorta and three in the pulmonary artery respec¬ 
tively, so that, in the absence of septation, four valve segments should theoretically persist. Hulse indeed definitely rejected cases in which 
there are only three cusps as not being examples of true truncus. However, a number of well-authenticated cases with only three cusps 
exist, including the two reported by the writer (Figs. 5 and 6) and in Carr’s case the valve was bicuspid. A very interesting point elicited 
by recent studies, and illustrated here by the case of Allan Roos (Fig. 8a), is the embryonic condition of the fleshy and irregularly thickened 
aortic cusps. Rigidly to be excluded from this category of persistent truncus are the cases of pulmonary or aortic atresia reported under 
the title truncus solitarius aorticus or pulmonalis as the case may be, in which a large trunk functions for both circulations in the place of 
the obliterated pulmonary artery or aorta, the vestiges of which are traceable as a cord ending blindly at the heart. Such cases are readily 
differentiated from true persistent truncus for the entire configuration of the heart is different, and examination usually reveals an aplasic 
ventricle hidden in the fleshy wall of one or other ventricle behind the atresic orifice (c/. PI. XX, Fig. 9). 

A. Cor biloculare: R. D. Rudolf, Anal. Soc. Great Brit, and Ireland, 1900, Feb. 17-19; L. Rivet and L. Girard, Arch, des mal. du coeur, 1913, 6: 720; 
R. H. Wood and G. A. Williams, Amer. J. Med. Sci., 1928, 176: 242. B. Persistent truncus arteriosus: H. Preisz, Beitr. path. Anat., 1890, 7: 283; N. W. Ingalls, 
Anat. Rec., 1915, 10: 9; W. Hulse, Virch. Arch., 1918, 226: 16; W. Ivlemke, Zbl. Path., 1925, 36: 307; H. M. Zimmerman, Amer. J. Path., 1927, 3: 617; G. Pezz, 
and G. Agostoni, Arch, des mal. du coeur, 1928, 21: 18; Iv. H. Finley, Amer. J. Path., 1930, 6: 317; P. F. Shapiro, Arch. Path., 1930, 9: 54, Case I; A. Feller 
Virch. Arch., 1931, 279: 869; E. M. Humphreys, Arch. Path., 1932, 14: 671; D. C. Beaver, Arch. Path., 1933, 16: 51; F. B. Carr, R. H. Goodale and A. E. P. 
Rockwell, Arch. Path., 1935, 19: 833; A. Roos, Amer. J. Dis. Child., 1935, 60: 966. 


A 

Fig. 1.—Diagram of the circulation in complete absence of the cardiac 
septa (cor biloculare) with transposition of the great arterial trunks. (From 
Abbott and Dawson, Internal. Clin., 1924, 4: 177, Fig. 15.) 

Fig. 1.—Cor biloculare with transposition of arterial trunks, partial situs 
inversus of viscera and imperforate anus. From a male infant aged 83^ 
months. Mother fourth para, had mental illness in early weeks of pregnancy. 
Died of erysipelas following measles and otitis media. No cyanosis through¬ 
out. At autopsy heart very large, common auriculo-ventricular orifice with 
three cusps between common auricle and ventricle. Pulmonary artery lies 
transposed behind aorta, is hypoplasic, without valve. Stomach, duodenum 
and pancreas totally, liver caecum and appendix partly transposed. (Case 
in the service of E. Weiss. Reported by M. E. Abbott, Blumer’s Bedside 
Diag., 1928, 2: 494, Fig. 340.) 

B 

Fig. 3.—Feller’s diagrams showing the essential features in his three cases 
of persistent truncus. 

a. Complete persistence of the primitive truncus. Both pulmonary 
arteries arise from it before the origin of great vessels. No trace of septum 
in its interior. Three semilunar cusps, one with raphe behind it. Large 
septal defect. Male, aged one week. 

b. Persistent truncus with partial division of interior by sickle-shaped 

septum. This incompletely separates a large left anterior chamber giving off 
die pulmonary arteries from a smaller right posterior one, which represents 
io ascending aorta and gives off the innominate and left subclavian, and 
■o ' or the narrow isthmus (at), the left subclavian arteries (ss). The truncus is 
7.2 cm. wide and rises above a septal defect 2.5 cm. across and is provided 
with three large semilunar cusps with thickened edges, behind one of which 
is a raphe separating the two coronary orifices from each other. 

c. Common truncus partially divided by curving longitudinal ridge to left 
of which pulmonary arteries are given off. Four semilunar cusps numbered 
to indicate bulbar swellings from which they are derived, small septal defect. 
Female aged 2 days. S.ap. septum aortico-pulmonale; S.ip., septum inter- 
pulmonale; ps, pulmonalis sinistra; pd, pulmonalis dextra; cd, coronaria 
dextra; cs, coronaria sinistra; D.A., ductus arteriosus. (From Virch. Arch., 
1931, 279: 869; reproduced in Nelson’s Loose-leaf Med., 1932, 4: 283.) 

* Specimen in the Cardiac Anomaly Collection of McGill University. 


Fig. 4.—Diagram showing the circulation in persistent truncus arteriosus 
with defect of interventricular septum (venous-arterial shunt). (From 
Abbott and Dawson, Internal. Clin., 1924, 4: 177, Fig. 16.) 

Fig. 6.—Persistent truncus supplied with three semilunar cusps giving off 
main pulmonary arteries and accessory branches (collateral circulation) from 
ascending part of right aortic arch. Single right coronary.* The common 

trunk is 3.2 cm. wide and arises above a septal defect 1.2 cm. across. Three 
anomalous accessory branches pass from the ascending (right) arch to the 
right lung. Left innominate artery. From a cyanotic infant aged 2 months. 
(Specimen No. 10237 in McGill Medical Museum presented from Childrens’ 
Memorial Hospital.) Acc.P.A., accessory pulmonary artery. 

Fig. 6.—Large common arterial trunk rising from hypertrophied right ven¬ 
tricle above septal defect. Three semilunar cusps. Septal defect.* From 

a boy aged 5 with cyanosis and clubbing since his third year. Intelligence 
above the average, paroxysmal dyspnoea and epileptiform convulsions. 
Partial autopsy, aorta cut off too short to determine source of pulmonary 
blood supply. (From Specimen No. 1311 presented to McGill Museum by 
McKenzie Forbes. Reported by M. E. Abbott, Osier’s Mod. Med., 1927, 
4: 708, Fig. 68.) A., Persistent truncus; B., right auricles; C., defect of 

ventricular septum; D., muscular column; E., auricular septum. H.V. right 
ventricle. 

Fig. 7.—Diagram showing the circulation in cor biloculare with persistent 
truncus (intense morbus coeruleus). (From Abbott and Dawson, Internal. 
Clin., 1924, 4: 178, Fig. 17.) 

Fig. 8.—From a case of persistent truncus with partial division of lumen, 
right aortic arch, and embryonic thickening of semilunar cusps. 

a. Photomicrograph of ostium showing nodular free margin of the valve 
composed of homogeneous lightly stained myxomatous tissue with notable 
absence of elastic fibres. (Weigert’s-safranine stain; N 18.) 

b. Roentgenogram of heart showing 200 per cent enlargement (W. E. 
Anspach) and an oval density on right side above (indicated by arrows), 
probably produced by right aortic knob. 

From a female infant with moderate cyanosis of lips and nailbeds and loud 
systolic murmur over whole precordium, dying at 17 days from acute nutri¬ 
tional disturbance. (Reported by Allan Roos, Amer. J. Dis. Child., 1935 
60: 966.) 


52 


PLATE XXII. A. COR BILOCULARE. B. PERSISTENT TRUNCUS ARTERIOSUS 




II 



Fig. 4. 


Fig. 7. 



Fig. 3c. 


53 


Fig. 3b. 


Fig. 8b. 



































































PLATE XXIII 


COMPLETE TRANSPOSITION OF GREAT TRUNKS WITH CLOSED VENTRICULAR SEPTUM 


Complete or, in Spitzer’s terminology, “crossed” transposition of the great trunks is a truly extraordinary and quite inexplicable phe¬ 
nomenon, when considered from the purely ontogenetic standpoint. For it must be recognized that this anomaly, in which the aorta arises 
from the right ventricle and the pulmonary artery from the left in an otherwise normal heart, cannot be the result of a mere arrest of 
growth, and can be explained only by the operation of additional unknown factors. The only other view possible would be to regard such 
a condition as a spontaneous aberration, constituting as it does a bewildering contradiction to the fundamental principles underlying the 
evolution of the cardiac architecture to meet the needs of the double circulation. Thus, in spite of Rokitansky’s brilliant morphological 
contribution to the elucidation of this subject, it was not until it was approached from what may be termed the teleological viewpoint that 
the enquiry into this intriguing problem yielded any directly fruitful results. The comparative anatomy studies of Keith, Robertson and 
others (PI. II and. Ill) first established the phylogenetic basis of these graver cardiac defects and may be said to have pointed the way to 
further advances. Spitzer’s spectacular explanation is in line with these earlier investigations but goes a step farther, harking back to the 
remote ancestry of the race and the inheritance of suppressed primordial tendencies which the anomaly unveils. Starting from the principle 
that very early arrest may fix, in the growing embryo, certain rapidly evanescent atavistic traits characteristic of a common ancestral stem, 
which have since become submerged in the mammalian and specialized in the reptilian orders, this author has developed, with what appears 
to us to be quite irrefutable logic, his now well recognized and, we believe, generally accepted detorsion theory. Briefly stated, this claims 
that early arrest in the bulbar region of the primitive heart tube inevitably interferes with the clockwise torsion that takes place in this 
region in normal growth; that any such lack of torsion, (i.e., detorsion) will result in the obliteration of the normal human (left) aorta, and 
the persistence of the reptilian right aorta, which is evanescent in the human embryo but now appears in permanent form as the “trans¬ 
posed” vessel, standing in abnormal relation to the pulmonary artery and other right ventricular structures (PI. IV); and that this phylo¬ 
genetic survival of an atavistic structure and its development on an ontogenetic basis are the actual causative factors in this group of grave 
cardiac anomalies. 

Clinical Aspects.—In complete transposition with closed septum, which is the subject of this plate, the conditions of the circulation 
are the worst conceivable as compatible with life. The venous blood entering the right auricle is transmitted in part through the circuitous 
route of the patent foramen ovale to the left chambers and thence to the pulmonary artery and in part to the right ventricle whence it 
passes unaerated through the transposed aorta to the systemic circulation and is returned to the right auricle without aeration by the 
systemic veins; while the aerated blood returned from the lungs to the left auricle passes straight back to these organs through the pul¬ 
monary artery except for a small part which, mixed with venous blood from the right auricle, escapes through the patent ductus to the 
general circulation. A vicious circle is thus created (Fig. 5) and results in the most extreme form of congenital cyanosis that occurs. 
Except in the rare cases in which a collateral supply to the lungs develops, these subjects survive only a few days or weeks and present 
from birth the deep mulberry hue of a true morbus coeruleus. Physical signs when present are generated at the foramen ovale or patent 
ductus, which latter is, however, occasionally closed (Fig. 3b). The right heart behind the transposed aorta is always greatly hyper¬ 
trophied and a right preponderance exists. The highest age recorded is 11 years, average in 31 other cases, 1% months. 


Acknowledgment is here expressed to Dr. Morris Lev of Chicago for access 
(For bibliography on this subject see page 38.) 

Fig. 1.—Diagram showing the circulation in complete (crossed) transpo¬ 
sition of the great arterial trunks with closed ventricular septum. Morbus 
coeruleus. (From Abbott and Dawson, Internal. Clin., 1924, 4: 179, 
Fig. 18.) 

Fig. 2.—Complete (crossed) transposition of great trunks, aorta from right 
pulmonary from left ventricle. Ventricular septum entire, foramen ovale 
and ductus arteriosus widely patent. The heart and lungs of a cyanotic 
infant dying one hour after birth. The great trunks arise beside each other, 
the pulmonary to the left of the aorta, and are of about equal size. The right 
ventricle is the larger of the two and forms the apex. The ductus is widely 
patent. Heart otherwise normal. (Drawing by Louis Gross in the Cardiac 
Anomaly Collection of McGill University. Reproduced in Abbott and Daw¬ 
son, Internal. Clin., 1924, 4: 180, PI. IV.) 

Fig. 3.—Complete (crossed) transposition of great trunks, aorta and pul¬ 
monary artery from reversed ventricles. Ventricular septum entire and 
ductus arteriosus closed. Foramen ovale patent. Dilatation of aorta and 
great hypertrophy of right ventricle. 

a. The roentgenogram from this case. The shadow is obliquely globular; 
it shows a mitral configuration. There is a dilatation of the right lower arch 
extending almost to the right thoracic wall, which indicates a high degree of 
dilatation of the right auricle. Pulmonary arc not visible. Aortic shadow 
widened at base. 

b. The heart itself. Noteworthy is the huge size of the transposed aorta 
which arises in the median line and arches over to the left, giving off the great 
vessels in their normal relations but somewhat twisted back upon themselves 
to attain the median line; the distinctly smaller pulmonary trunk which hugs 
the left side of the aorta on a slightly posterior plane; the enormous hyper¬ 
trophy of the left ventricle which presents a prominent muscular shoulder 
below the deep auriculo-ventricular groove and forms four-fifths of the anterior 
surface and entire lower border and apex of the heart; the dilated right 
auricular appendix; and the position of the smaller left ventricle at the upper 
left border, as indicated by the position of the interventricular groove. 

c. The electrocardiogram from this case. The tracing shows a sinus 

rhythm with rate of 98 per minute, no conduction delay and marked right 
axis deviation. The R-T segment is elevated in leads 1 and 2. *S 2 is notched. 

Qi is deep, indicating a right ventricular event. 

From a colored infant aged 7 months, the fourth child of a 30-year-old 
mother with hypertension. Three siblings and a miscarriage preceded this 
pregnancy. Admitted acutely ill and cyanotic with labored respiration. 


to his beautiful translation of Spitzer’s great monograph (1923). 

Eyelids puffy, lungs showed dullness in both apices. Liver enlarged and 
tender. Moderate cranio-tabes. Heart was tremendously enlarged to left 
and right, the sounds forceful. A loud high-pitched, systolic murmur clearest 
at base in second to third left interspace. No thrill. Pulmonary second 
sound was decreased. Death soon after admission from bronchopneumonia. 
At autopsy the ductus was represented hy a ligamentous cord. No collateral 
vessels to the lungs were made out, but the inferior vena cava, which was greatly 
enlarged, received one of the pulmonary veins. The great hypertrophy of the 
right ventricle that existed was probably the result of this adaptation. (From 
the Pediatric Service of St. Luke’s Hospital, New York. Reported by G. 
Nicolson for publication in this Atlas.) 

Fig. 4.—Complete transposition of arterial trunks with obliteration of aortic 
orifice below cusps (aortic atresia). Ventricular septum closed. Diffuse 
myocardial fibrosis and degeneration, syphilitic? Great dilatation and 
hypertrophy of left ventricle and dilatation of pulmonary artery which forms 
descending aorta through widely patent ductus, foramen ovale widely 
patent. 

a. The enlarged heart showing the greatly dilated pulmonary artery arising 
from the left ventricle and forming the descending aorta through the widely 
patent ductus arteriosus; the base of the aorta laid open to show three rudi¬ 
mentary cusps above the closed conus orifice, the huge left and aplasic right 
ventricle, a, right auricle; b, coronary orifice; c, aortic valve; d, descending 
aorta; e, left appendix;/, mitral valve. 

b. Microphotograph of the section taken from the myocardium of the conus 
of the right ventricle just below the aortic cusps, showing perivascular cell in¬ 
filtration and extensive fibrosis which evidently caused occlusion of the orifice. 

From a deeply cyanotic male infant aged 7 days with a supernumerary finger 
on right hand. (Case of Berta Meine, Woman’s Hospital of Philadelphia. 
Reported by Abbott and Dawson, Internal. Clin., 1924, 4: Fig. V. Drawing 
of heart by Louise Brecht, Woman’s Medical College of Pennsylvania.) 

Fig. 5.—Circulation of the blood in transposition of the arterial trunks. 
The dark colored portion represents the venous blood, which is seen filling the 
entire systemic circulation, while the arterialized blood (uncolored) is confined 
to the lesser circulation. Communication through one or other of the foetal 
passages would be necessary for life. C.S., Cor sinistra; C.D., cor dextra; 
P., pulmonary artery; A., aorta; C.S., superior cava; C.J., inferior cava; V.P., 
pulmonary veins. (Adapted from Bokay, Arch. f. Kinderheilk., 1911, 66: 
333; Republished by permission of Lea & Febiger from Osier’s Mod. Med., 
1927, 4: 719.) 


54 


PLATE XXIII 


COMPLETE TRANSPOSITION OF GREAT TRUNKS WITH CLOSED VENTRICULAR SEPTUM 



Fig. 1. 



_Probe throuqh 
Patent D.A. 


L-App- 


Fig. 2. 




Fig. 3b. 


Fig. 3c. 



Fig. 4a. 


Fig. 4b. 



55 




































PLATE XXIV 


COMPLETE TRANSPOSITION OF GREAT TRUNKS WITH DEFECT OF INTERVENTRICULAR SEPTUM 

As indicated above, complete transposition, where the ventricular septum is entire (PL XXIII), is a condition of extreme gravity, only 
a small fraction of aerated blood reaching the systemic circulation and the unfortunate subjects dying in early infancy. An associated 
ventricular septal defect, on the other hand, provides distinctly improved circulatory conditions, in that a part of the venous blood from 
the right auricle passes directly through the defect into the transposed pulmonary artery and thence to the lungs for aeration; and a part 
of the oxygenated blood returned from the left auricle is transmitted an instant later through the same pathway into the transposed aorta 
and thence, mixed with unaerated blood from the right auricle, is distributed to the systemic circulation by the same shuttle-like action 
as is carried on to perfection in the heart of the turtle, with the help of its specially developed auriculo-ventricular valves. Such patients 
accordingly may live into childhood or early adult life with fairly good cardiac efficiency and only moderate cyanosis. The larger the 
septal defect the more easily can this circulatory arrangement be carried on, and when other anomalies having a like compensatory effect 
are associated, life may be sustained with fairly good cardiac efficiency until late middle life. 

In the two remarkable cases figured opposite, the auricular septum was completely closed (as in the heart of the turtle) and the ven¬ 
tricular septum was rudimentary, a virtual cor triloculare biatriatum existing; and both were subjects of associated anomalies of a very 
unusual kind illustrating the remarkable adaptations that may develop in early embryonic life for the maintenance of the circulation. In 
Case 1 (Figs. 2 a and b) the existence of such a grave cardiac anomaly in the host of an omphalositic monster is in itself a very rare occur¬ 
rence, the autosite being almost always a normal well-developed child; of interest also are the anatomical relations of the mitral and tri¬ 
cuspid segments to the arterial ostia whereby the blood has been propelled into the transposed aorta and pulmonary artery by the shuttle 
action above described; and the enormous dilatation of the pulmonary circulation that has developed in response to the double demand that 
existed for oxygenation of the tissue of the parasitic twin. 

Case 2 (Figs. 3a, b, 4, 5 and 6) presents a remarkable combination of grave cardiac anomalies unique in our experience in the litera¬ 
ture. Space does not permit here the detailed description of this, but the beautiful drawings and diagrams opposite tell their own story. 
The primary anomaly here was undoubtedly a detorsion defect that apparently involved the auricular as well as the bulbar (ventricular) 
end of the cardiac tube, so that, in addition to the “crossed” transposition of the great trunks, there existed a dextroposition of the left 
auricle behind the right. Both these chambers have thus come to lie to the right of the great vessels, and the auricular septum, which is a 
strong muscular partition (b in Fig. 3 b) completely separating the two auricles, is malposed, and lies on a plane anterior and to the right of 
the rudimentary interventricular septum, with which it is not in continuity, thus explaining the complete heart block that existed. (The 
writer is deeply indebted to Dr. C. F. Moffatt of the Royal Victoria Hospital, in whose care this patient was during life, for permission to 
present here the pictorial reproductions of this hitherto unpublished case, to Dr. W. W. Eakin for assistance in studying the clinical labora¬ 
tory data, and to Miss Harriet Blackstock for the remarkably informing drawings shown.) 

This case is possibly analogous to those of sinistro-position of the auricles published by O. Wenner, Virch. Arch., 1909, 196: 127; A. Birmingham, J. Anat. 
and Physiol., 1892-93, 26: 139; and S. K. Ngai, Amer. J. Path., 1935, 11: 309. 

Fig. 1.—Diagram of the circulation in transposition of the great trunks 
with defect of the interventricular septum and patent ductus. (From Abbott 
and Dawson, Internal. Clin., 1924, 4: 175, Fig. 14.) 

Fig. 2.—Cardiac anomaly in host (complete transposition with rudimentary 
interventricular septum) in a case of thoracopagus (dipygus) parasiticus. 

a. The body of a well developed female infant with the lower half of an 
acardiac parasitic female twin attached at its umbilicus and left thoracic 
border. Two small arterioles enter this from the host and constitute its sole 
blood supply. 

b. The heart and lungs of the host (external view). The aorta rises on 
the left anteriorly in the position normally occupied by the pulmonary artery, 
which emerges behind on the right from the left ventricle and is greatly dilated. 

The heart is a strong muscular organ, the hypertrophy being chiefly of the 
right ventricular part. The lungs are voluminous. 

From a moderately cyanotic child aged 14 months of good intelligence. 

No clubbing, palpable thrill felt and unclassified murmur heard all over whole 
precordium. Died of bronchopneumonia. The ductus arteriosus was widely 
patent and the pulmonary artery and its terminal branches in the lungs were 
widely dilated with large thin-walled venous sinuses. (From the Pathological 
Service of the Hoagland Laboratory, Brooklyn, N. Y. Reported by W. F. 

Watton and M. E. Abbott, J. Tech. Meth., 1922, 8: 165.) 

Fig. 3.—Complete (crossed) transposition of great trunks with rudimentary 
interventricular septum and dextroposed left auricle with malposed auricular 
septum and complete congenital heart block. Stenosis of conus of right 
ventricle with hypoplasia of transposed aorta and bulbar septal defect. Sup¬ 
plementary right superior cava from left auricle, and secondary (double) 
mitral orifice. Patent ductus arteriosus. Dilatation and atheroma of pul¬ 
monary artery and veins and arterio-venous aneurysm of lungs (congenital?). 

a. Interior of aortic vestibule of this case giving off the transposed and 
greatly dilated pulmonary artery. Only two of the three pulmonary cusps 
•ive visible and a short distance below these are seen the anomalous chordae 

bached to the double mitral orifice in the anterior segment of the mitral 
•.wive. 1, the pulmonary artery; 2, dilated arterioles; 3, rudimentary ven¬ 
tricular septum; 4, double mitral orifice; 5, anomalous chordae. 

b. The heart laid open to show the chambers of both auricles and the sinus 
of the right ventricle. The auricular septum has been divided to expose the 
interior of the large left auricle, which lies directly' behind the smaller right 
auricle. It receives three greatly enlarged pulmonary veins (c) and the sup¬ 
plementary right superior cava in its roof. It is closed below by a competent 
mitral valve, the anterior segment of which is widely cleft and is the seat of 


the double mitral orifice, through which the aerated blood was directed across 
the aortic vestibule and through the bulbar septal defect into the transposed 
aorta. The rounded free muscular border of the rudimentary ventricular 
septum is visible just beneath this cleft segment, and the space above it is 
crossed by the anomalous chordae of the double orifice. The hugely dilated 
atheromatous pulmonary artery (P) is seen above the heart in the median 
line, and below it the large right pulmonary vein (c). S., right superior cava 

entering right auricle; C., carotid artery; a.-a., the hypertrophied right 
auricular appendix cut across; h, the divided auricular septum. 

Male, aged 20 years, cyanosis and dyspnoea from childhood with cough on 
exertion and frequent throbbing headaches, marked clubbing with curvature 
of nails. R.B.C. 7,200,000. Definite precordial bulging and visible pulsation 
in fourth and fifth left interspaces, questionable systolic thrill over base. 
Cardiac area enlarged to right and left and above. Soft systolic murmur in 
third left interspace, pulmonary accentuation, pulse 38, complete heart block. 
Was able to do very light work, had a slight haemoptysis and a week later 
died following a sudden terrific pulmonary haemorrhage. (Drawings by 
H. Blackstock, Medical Art Department, McGill University. From Speci¬ 
men No. 9839 in the Cardiac Anomaly Collection.) 

Fig. 4.—Roentgenogram from this case shows enlargement of the cardiac 

shadow on both right and left sides, enlarged pulmonary arc and marked 
widening with pulsation of the shadows at the hilum of both lungs. (From 
the monograph by M. E. Abbott, Blumers Bedside Diag., 1928, 2: 464, Fig. 
316.) 

Fig. 5.—Diagrammatic presentation of the anterior aspect of the heart and 
great vessels in this case, with insets showing interior of the conus at different 
antero-posterior levels. 1 , the bulbar septal defect; 2, free border of the 
rudimentary interventricular septum; 3, the small communication with triple 
opening between the conus cavity and the sinus of the right ventricle; 4, 
muscular pillar; A, the hypoplasic aortic arch with patent ductus communi¬ 
cating with the greatly dilated pulmonary artery; SVC, superior vena cava 
leading into right auricle; a, supplementary right superior cava from left 
auricle; R.P.A., right pulmonary artery; R.P.V. and L.P.V., dilated right 
and left pulmonary veins; I.V.C., inferior vena cava. (Drawing by H. 
Blackstock.) 

Fig. 6.—Electrocardiogram from this case, showing complete congenital 
heart block, right ventricular preponderance and inverted T in lead 1. (From 
Blumer’s Bedside Diag., l.c. above, Fig. 317.) 


56 


PLATE XXIV. COMPLETE TRANSPOSITION OF GREAT TRUNKS WITH DEFECT OF VENTRICULAR SEPTUM 



COMPLETE TRANSPOSITION of ARTERIAL TRUNKS 

DETECT OF VENTRICULAR SEPTUM 
PATENT DUCTUS ARTERIOSUS 

l PULMONARY HYPOPLASIA J 


Fig. 1. 


Fig. 2a. 


Fig. 6. 


Fig. 3a. 


f/.B. '30. 


Fig. 3b. 


Fig. 2b. 


57 



































































PLATE XXV 


A. DEXTROCARDIA. B. CORRECTED TRANSPOSITION WITH COMPLETE CONGENITAL HEART BLOCK 

A. Dextrocardia as a part of a complete situs inversus of the viscera is not, functionally speaking, an anomalous condition, for the 
path of the circulation is merely reversed without any interference with its physiological course. Such cases are therefore of no clinical 
significance and do not come under consideration here. Mirror-picture dextrocardia with partial situs inversus of the viscera, on the other 
hand, is practically invariably associated with grave cardiac anomalies, which place the cases in the cyanotic group (Figs. 16, 2 a, 6, c and 
3a and 6 opposite). The same applies to “pure” or “isolated” dextrocardia in which there is no situs inversus of the viscera, but in which 
there is either (a) complete or (6) partial inversion of the heart chambers, or (c) in which there is no inversion of the heart chambers 
but the heart lies to the right of the median line with apex formed by the right ventricle, a condition due to early arrest (not figured 
here). This subject is fully reviewed and the above variations classified by H. Roesler with extensive bibliography. In the case shown 
opposite of mirror-picture dextrocardia with partial situs inversus of the viscera the absence of right preponderance shown in the electro¬ 
cardiogram (Fig. lc) is characteristic of cases complicated by grave cardiac anomalies. 

B. “Corrected” transposition is a rare and still incompletely understood phenomenon. Spitzer acknowledges he has never seen an 
example, and though Rokitansky’s brilliant hypothesis covered eight possible variations, only two of these had been observed by him. In 
this phenomenon a complete or crossed transposition has occurred, in that the great trunks arise in reversed relations, but the condition is 
“corrected” from the functional standpoint in that each trunk is placed in its proper ventricle. The generally accepted explanation at the 
present day is that the transposition of the great trunks has been combined with a reversal of the bulbo-ventricular bend with inversion 
of the ventricles, but this does not seem to explain entirely the facts in the light of Spitzer’s detorsion theory. We owe the privilege of 
presenting the exquisite example of this unusual anomaly (Fig. 46) to the courtesy of Dr. Allan Roos of New York, who submitted the 
specimen to the writer for verification, and the beautiful illustration is the work of Mrs. A. C. Cheney of the Medical Art Department of 
McGill University. In it we have an example par excellence of this condition in that the aorta arises to the left and anteriorly from the- 
functionally systemic tricuspid valve ventricle here placed on the left side, and the dilated pulmonary artery from the functionally pulmonic 
mitral valve ventricle which here occupies the right side of the heart. 

Complete Congenital Heart Block. —This phenomenon, which was a special feature of the symptomatology of the above case, was 
apparently explained here by the anomalous relation of the interventricular to the auricular septum, but investigations on this point are 
being carried out by Dr. Roos. It was present also in the remarkable case shown in PI. XXIII, Fig. 36, and is commonly associated, as 
was shown by Monckeberg, with defects of the interventricular septum. 

(For bibliography on the above subjects see page 38.) 

Fig. 1.—Mirror picture dextrocardia in partial situs inversus complicated 
by mitral aplasia. 

a. Roentgenogram of the heart in this case, showing the apical portion of 
the large coeur-en-sabot in the right thorax and the huge “right” auricle form¬ 
ing the left border of the cardiac shadow extending to the left midclavicular 
line. 

b. Anterior view of heart laid open to show interior of the “right” cham¬ 
bers, showing the dextroposed aorta arising in the median line and the huge 
“right” auricle left side receiving the inferior and superior cavae and the dis¬ 
placed left pulmonary veins, a, tricuspid orifice; b, orifice of coronary sinus; 
c, orifice of inferior cava. 

c. Electrocardiogram from this case shows inversion of P and T waves, no 
preponderance and the leads known as III and II under normal conditions, 
here replacing one another as leads II and III (characteristic of situs inversus). 

A well-developed baby girl aged 14 months with cyanosis from birth 
and clubbing of fingers and toes. Apex on the right side, a systolic murmur 
over base of heart. Died suddenly just after admission. The bicuspid 
and hypoplasic pulmonary artery arose transposed from the systemic ven¬ 
tricle, the mitral valve and ventricle were aplasic, “right” auricle enormously 
dilated. (From a case in the service of Graham Ross, Royal Victoria Hos¬ 
pital. Reported by M. E. Abbott and W. Moffatt, Canad. Med. Assn. J., 

1929,20:611.) 

Fig. 2.—Mirror-picture dextrocardia in situs inversus viscerum with 
multiple associated anomalies (cor biventriculare biatriatum, displaced left 
pulmonary veins, persistent left superior cava into coronary sinus). 

a. The left lung and the heart pulled over from behind and laid open to 
show interior of transposed “right” chambers. The right auricle ( R.A .) is 
hugely dilated and receives the superior and inferior cavae on its roof and 
floor, and the two left pulmonary veins ( L.P.V .) on its middle left border and 
the large coronary sinus on the right below. It is incompletely separated 
from the small left auricle ( L.A. ), which receives the right pulmonary veins 
( R.P.V .) by a defective auricular septum which presents a widely patent 
foramen ovale ( F.O .) above and the persistent ostium primum below. The 
“right” ventricle is hypertrophied and the tricuspid valve surmounted by 
endocardial excrescences. 

b. Heart everted to reveal interior of the dilated coronary sinus. This 

ei vos in its roof the persistent superior vena cava and has at its lower border 
its large crescentic orifice into the “right” auricle. 

:. The thoracic and abdominal viscera of this case in situ, showing com¬ 
plete transposition. Liver and appendix on left side, stomach on right, heart 
in median line with apex in left thorax, dilated “right” auricle receiving pul¬ 
monary veins on left and large persistent superior cava entering coronary 
sinus on right of picture. 

From a male infant aged 9 weeks, dying from volvulus of intestines, cyanosis 
marked on crying, systolic thrill, loud murmur all over precordium. Ductus 


patent, no pulmonary stenosis. (From an unpublished case in the service 
of Louis Gross, Mount Sinai Hospital, New York. Drawings by A. C. 
Cheney from specimen No. 11353 in the Cardiac Anomaly Collection, McGill 
University.) 

Fig. 3.—Cor biloculare and pulmonary atresia in mirror-picture dextro¬ 
cardia with partial inversus of viscera. 

a. Roentgenogram of this case, showing the large globular cardiac shadow 
reaching to right axillary border and single arterial trunk. 

b. Semidiagrammatic sketch of the biloculate heart giving off the large 
truncus aorticus solitarius from its left upper angle and the atresic pulmonary 
artery behind and to right of this, communicating with the aortic arch by the 
patent ductus arteriosus ( P.D.A. ). The single auriculo-ventricular orifice 
is guarded by a common valve with four cusps ( A-V.V .) leading into the ven¬ 
tricle from a single auricle, which lies on the left above and receives a common 
pulmonary vein draining both lungs. 

A male infant aged 4 months, “blue” from birth and markedly dyspnoeic, 
finger nails curved, died just after admission. Anomalous rotation of pan¬ 
creas and duodenum, situs inversus of oesophagus and stomach, absence of 
spleen. (Reported by M. A. Kugel, Amer. Heart J., 1932, 8: 280.) 

Fig. 4.—Corrected transposition of great trunks. Hypoplasia and slight 
coarctation of aorta. Anomalous insertion of anterior mitral segment in 
floor of right auricle and complete congenital heart block. 

a. The roentgenograph from this case. Shows great enlargement of cardiac 
shadow on left reaching to axillary border. Enlarged pulmonary arc. 

b. Anterior view of the lungs and heart, the latter laid open to expose 
interior of both ventricles and the lumina of the great trunks. The greatly 
dilated pulmonary artery ( P.A .) arises on the right and somewhat posteriorly 
from the mitral valve ventricle which occupies the right side of the heart but 
has the anatomical structure of the normal left chamber and receives venous 
blood from the right auricle. The aorta arises to the left and anteriorly from 
the tricuspid valve ventricle which lies on the left side and receives the aerated 
blood from the left auricle. 

c. Electrocardiogram from this case showing complete heart block. The 

auricles are beating at a regular rate of 150 per minute. The ventricular 
complexes bear no relation to the auricular ones. In lead I and the first 
complex seen in lead II, they are of the infraventricular type with a rate of 
58 per minute. Following this the ventricular rate falls to 38 and the com¬ 
plexes are of nodal type. 

From a well developed female infant aged 846 months. Occasional short 
periods of transient cyanosis, no polycythemia. Bradycardia. Died sud¬ 
denly. Liver greatly enlarged, mural endocardium thickened, one pulmonary 
cusp very small, pulmonary artery 4.5 cm. in circumference, aorta 3 cm., 
wall fused with pulmonary artery. (Unpublished case communicated by 
Allan Roos for publication in this Atlas. Drawing by A. C. Cheney.) 


58 


PLATE XXV. A. DEXTROCARDIA. B. CORRECTED TRANSPOSITION AND COMPLETE CONGENITAL HEART BLOCK 




Fig. 2a. 




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APPfnOtA 



Fig. 3a. 



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Fig. 2b. 


Fig. 2c. 


Fig. 3b. 

















Chart I. — Statistics of Congenital Cardiac Disease (1,000 Cases Analyzed) 


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INDEX 


Abbott, M. E., 12, 18, 20, 22, 34, 36, 
46, 50, 52, 54 
and W. H. Chase, 22 
and W. Dawson, 32-36, 44-56 
and J. Kaufmann, 34 
D. S. Lewis, and W. Beattie, 44, 46 
and W. Moffatt, 58 
and E. Shanly, 4, 6 
Adami, J. G., 12, 16 
Ambrus, G., 16 
Anasarca, 34, 42 
Anatomical features: 

anterior tricuspid ledge, 8 
bulbo-ventricular ridge, 6 
crista supra-ventricularis, 6, 8 
niche of right aorta, 8 
outflow tract of right ventricle, 8, 12 
septum aortico-pulmonale, 8 
trabecula septo-marginalis, 8 
Anomalous chordae in auricles, 24, 42 
in ventricles, 24, 46, 56 
Aorta, biventricular, 8 

dilatation of, 22, 46, 48, 54 
dextroposition of, 4, 40, 42, 44 
left ventricular, 8, 54 
right reptilian, 8, 46, 54 
Aortic arch, absence of, 50 
Aortic arches, transformation of, 6 
Aortic knob, 16, 42, 46, 52 
Aortic stenosis, 20 
Aortic valve, bicuspid, 18, 20, 22 
fourth cusp, 22 
Arkin, A., 16 

Arterio-venous aneurysm of pulmon¬ 
ary circulation, 56 
of right or left coronary, 12, 28 
Associated anomalies: 
absence of ears, etc., 42 
absence of spleen, 58 
absence of thumb, 48 
double renal artery, 24 
imperforate anus, 56 
mental deficiency, 42 
mongolian idiocy, 34, 50 
situs inversus, 58 
supernumerary finger, 54 
supernumerary spleens, 50 
thoracopagus parasiticus, 56 
Atresia, aortic, 48, 50 
pulmonary, 16, 46, 48, 58 

Bard and Curtillet, 34 
Bassen, E., 48 
Baxter, J. S., 6 

Baumgartner, and Abbott, 441 
Beattie, W. W., 4, 12, 34, 44, 50 
Berconsky, I., 40 
Berger, L., and A. Vallee, 26 
Bifid apex, 2, 12, 36, 48, 50, 52 
Bishop, L. F., and M. Trubek, 22 
Blackford, L. M., 8, 44, 48 
Bland, White, and Garland, 26 
Bonnet, L. M., 18 
Brecht, Louise, 54 
Bremer, J. L., 2 
Bulbar septal defect, 36, 56 
Bulbar swelling, 8, 36 

Capillary aneurysms, 20" 

Cardiac Anomaly Collection, McGill 
University, 4, 6, 12, 16, 20, 22, 
24, 26, 28, 32, 34, 36, 42, 54-58 
Cardiomegaly glycogenica, 26 
Cerebral abscess, 44 
Cerebral aneurysm, 20, 22 
Chase, W. H., 20, 22 
Circulation, course of, 32-34, 42-56 
Clubbing, 40, 44, 46, 48, 56, 58 
Coarctation of aorta, 16-22,32, 34, 58 


Cohn, J., 24 

Collateral circulation, 18-22, 34, 52 
Cor biloculare, 32, 58 
Cor triloculare biatriatum, 50, 56 
Cor triloculare biventriculare, 50, 56, 
58 

Coronary artery, displaced, 22 
single, 48 
Costa, A., 18, 48 
Cruveilhier, J., 18 
Cyanosis retinae, 40 

Dawson, W. T., 12 

and Garnet Jex, 16, 32-36, 42-56 
Detorsion, 8, 46, 54, 56 
Dissecting aneurysm, 18, 20, 22 
Diverticulum, 16 
Dolgopol, V. B., 16 
Dressier, W., and H. Roesler, 34 
Ductus, patent, 32, 46, 48, 54, 56, 58 
Dysphagia, 16, 18 
Dyspnoea, 16, 42-56 

Eakin, W. W., and M. E. Abbott, 42 
Eisenmenger complex, 44 
Embolism, paradoxical, 34 
pulmonary, 32 
Endarteritis, bacterial, 32 
Endocarditis, 22, 32, 36, 42, 44, 46 
Epileptiform convulsions, 46 
Erb, I. H., 16 
Erosion of ribs, 18, 20 
Evans, W., 16, 18 

Fallot, 46 
Fleury, J., 44 
Foetal circulation, 32 
Foetal endocarditis, 44, 46 
Foetal myocarditis, 42, 48, 54 
Foramen patent, 12, 24, 34, 44, 46, 
48, 50, 52, 54, 58 
Foramen Panizzae, 6 
Fourth aortic cusp, 22 
Fourth pulmonary cusp, 24 

Gangrene of lung, 20, 42 
Glendy, M. M. and R. E., and P. D. 
White, 50 

Glomerulonephritis, 20, 42 
Glycogen-storage disease, 26 
Goldenweizer, 24 
Groedel, Th., 32 
Gross, L., 22, 58 

Haemorrhage, cerebral, 18, 20 
from lungs, 46, 56 
into lungs, 16 
Halpert, B., 28 
Hamdi, 16 

Hamilton, W. F., 24, 36 

and M. E. Abbott, 18, 20, 32 
Harris, H. A., 6 
Hasler, 18 

Heart, “incomplete, double,” 50 
Heart block, complete, 56, 58 
partial, 36, 46 

Heart-signs record, 32, 36, 46 
Holmes, A. F., heart, 50 
Howard, C. P., 46 
Humphreys, E. M., 26 
Hydramnios, 34 
Hypertension, 20, 26, 54 
Hypertrophy, cardiac, in infants, 26 
Hypoplasia, aortic, 22, 32, 34, 36 
pulmonary, 40, 46, 48, 54 

Jordan, J., 18 

Kaufmann, J., 34 


Keith, Sir Arthur, 2, 4, 6, 8, 42, 54 
King, J. T., 18, 20 
Kugel, M., 26, 40, 48, 58 

Laubry and Pezzi, 20, 32 
Leader, S. D., and M. A. Kugel, 40 
Left auricle, dextroposition of, 56 
Left coronary from pulmonary, 12 
Left ventricular aorta, 8 
Lev, M., 54 
Lewis, D. S., 22, 44 
Lewis, Sir Thomas, 18, 32 
and R. T. Grant, 22 
Libman, E., 20 
Lindman, J. H., 24 
Lundsgaard, and van Slyke, 40 
Lundsgaard’s D factor, 42, 44, 46 
Lutembaeher, R., 34 

McCrae, John, 36 

McGinn, S., and P. D. White, 34 

McKenzie, R. Tait, 50 

Martin, C. F., 34 

Mead, K., 32 

Meakins, J. C., 40 

Meckel, A., 8, 18 

Meeker, L., 50 

Mitral atresia, 48, 52 

Mitral insufficiency, 24, 34 

Mitral orifice, double, 5, 24, 58 

Mitral, cleavage of, 12, 34, 50, 56 

Mitral stenosis, 48, 50 

Moffatt, C. F., and M. E. Abbott, 54 

Moore, R. A., 50 

Morbus coeruleus, 40, 46, 48 

Mycotic aneurysm, 20 

Nabarro, D., 28 

Network of Chiari, 24 

Ngai, S. K., 54, 56 

Niche of left and of right aorta, 8, 46 

Nicolson, G., 24, 36, 54, 58 

Oedema, 18, 34, 42 

absence of, in morbus caeruleus, 40 
Oesophagus, constriction of, 12, 16 
Omphalositic monster, 50 
Osier, Sir William, 16, 22, 34, 44, 46 
Ostium atrio-ventriculare, 50 
Ostium primum persistens, 12, 34, 50 
Otto, A. W., 18 

Papillary excrescences, 24, 58 
Paradoxical embolism, 34 
Paralysis, left recurrent laryngeal, 32 
Parkes, Weber and Dorner, 40 
Patent ductus, infective arteritis, 32 
rupture of heart in, 32 
Peacock, T. B., 22 

Persistent omphalo-mesenteric bay, 50 
Persistent left superior cava into 
coronary sinus, 28, 58 
Persistent truncus arteriosus, 52 
Phylogenetic theory, 8, 54 
Polycythemia, 40, 42, 46, 56 
Pulmonary artery, atheroma of, 36, 
44, 50, 56 

dilatation of, 8, 24, 28, 32, 34, 36, 44 
forms descending aorta, 8, 48 
Pulmonary stenosis, or atresia, 48, 58 
of conus and trunk, 16, 46 
at lower bulbar orifice, 42 
valvular, 44, 46, 48 
Pulmonary valve, bicuspid, 4, 46 
Pulmonary veins, enter coronary 
sinus, 28 

displaced into “right” auricle, 58 
Ratner, Abbott and Beattie, 50 



Reversal of ventricular bend, 58 
Reynaud, A., 18 
Rhabdomyoma, congenital, 26 
Right aortic arch, 10, 16 
Right axis deviation, 34, 40, 42, 44, 46, 
48, 50, 52, 54 

Right pulmonary, absent 16 
Right superior cava, absence of, 28 
Robertson, Jane, 4, 6, 54 
Robson, G. M., 50 
Roemer, A., 18 
Roesler, H., 18, 20, 34, 46, 56 
Rokitansky, C. von, 8, 54, 56, 58 
Roos, A., 52, 58 

Rupture, of aortic sinus of Valsalva, 36 
of ascending aorta, 18, 20, 22 

Schlesinger, 18 
Segall, H. N., 32, 36, 40, 46 
Septation, anomalous, 8, 50, 56 
Septicaemia, pneumococcus, 32 
staphylococcus, 36 
S. haemolyticus, 20 
S. viridans, 42 

Septum, auricular, closed, 6, 56, 58 
Shanly, E., 4, 6 

Shunt, arterial-venous, 12, 32-36 
venous-arterial, 28, 40, 44-56 
Snelling, C. E., 16 

Spitzer, A., 6, 8, 42, 44, 46, 50, 54-58 
Subarachnoid haemorrhage, 20 
Sprague, Bland, and White, 26 
Stewart, H. L., and B. L. Crawford, 44 
Subaortic stenosis, 24 
Subclavian, left, from patent ductus, 
or from left aortic root, 16 

Tandler, 2, 36 
Taussig, H., 26 
Terplan, K., 26 
Tetralogy of Fallot, 16, 46 
Thalheimer and Holbrook, 42 
Thatcher,.H., and A. F. deGroat, 28 
Thayer, W. S., 32 
Thompson, T., and W. Evans, 34 
Thoracopagus parasiticus, 54 
Threshold value, unsaturation, 40 
Torsion, clockwise, 2, 6 
delayed, 46, 54, 56 
Transposition, crossed, 54, 56 
corrected, 58 
“mixed,” 8, 50 

simple (dextroposition of aorta), 46 
Tricuspid atresia, 48 
Tricuspid insufficiency, 24 
Tricuspid stenosis, 44 
Turtle, heart of, 6, 56 

Vascular ring constricting, 16 
Vascular signs in coarctation, 20 

Walmsley, T., 2 

Watton, W. F., and M. E. Abbott, 56 
Weiss, E., 36 
and Lowbeer, 40 

Wesson, H. R., and D. C. Beaver, 48 
Wheeler, D., and M. E. Abbott, 16 
White, P. D., 36, 44, 46 

R. S. Eustis, and W. J. Kerr, 58 
and H. B. Sprague, 40 
Wiglesworth, F. W., 24, 48 
Wilkinson, D., 20 

Wilson, J. G., and R. T. Grant, 58 

Yater, W. M., 24, 58 

W. Ledman, and V. H. Cornell, 58 

deZalka, 48 

Zinn, x-ray cap of, 32 


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