Congenital Heart Disease A Clinical, Pathological,

Embryological, and Segmental Analysis 1st Edition

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Congenital
Heart Disease
A Clinical, Pathological, Embryological,
and Segmental Analysis
This page intentionally left blank

     
To protect the rights of the author(s) and publisher we inform you that this PDF is an uncorrected proof for internal business use only by the author(s), editor(s), reviewer(s), Elsevier and
typesetter TNQ Technologies Pvt. Ltd. It is not allowed to publish this proof online or in print. This proof copy is the copyright property of the publisher and is confidential until formal
publication.

Congenital
Heart Disease
A Clinical, Pathological, Embryological,
and Segmental Analysis

Richard Van Praagh, MD

Professor of Pathology Emeritus
Harvard Medical School
Director of the Cardiac Registry Lab Emeritus
Research Associate in Cardiac Surgery Emeritus
Emeritus Member of the Departments of Cardiology,
Pathology, and Cardiac Surgery
Boston Children’s Hospital
Boston, Massachusetts
Elsevier
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CONGENITAL HEART DISEASE: A CLINICAL, PATHOLOGICAL,

EMBRYOLOGICAL, AND SEGMENTAL ANALYSIS ISBN: 978-1-56053-368-9
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 D E D I C AT I O N

This book is dedicated to all patients with congenital heart disease and to their
physicians, surgeons, nurses, and other health care providers.
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 ACKNOWLED GMENTS

To understand the bridge that exists between the genome and the operating room, one should
study all the stages that make up this bridge. My postgraduate training (1954 to 1965) represents
my attempt to do that. The following are some of the outstanding individuals who have helped
to guide and to contribute to my studies. Also indicated are the names of my training positions
and where and when this postgraduate work was done.
Dr. Laurence Chute and his staff at the Hospital for Sick Children in Toronto, Canada, intro-
duced me to clinical pediatrics. I served as a Junior Assistant Resident in Pediatrics from 1955 to
1956.
Dr. Charles A. Janeway and his staff at the Children’s Hospital Medical Center in Boston,
Massachusetts, from 1957 to 1958. This position was known as a Senior Assistant Residency in
Pediatrics.
Dr. John Craig, who masterfully directed my basic science year in general pediatric pathology
at the Children’s Hospital Medical Center in Boston, Massachusetts, from 1956 to 1957. This posi-
tion was known as an Assistant Residency in Pathology.
Dr. MacDonald introduced me to adult internal medicine at Sunnybrook Hospital in Toronto,
Canada, from 1958 to 1959. During this year I also finished a paper on the causes of death in
hemolytic disease in a newborn—my first scientific paper. This position was known as an Assis-
tant Residency in Internal Medicine.
Dr. Helen B. Taussig and Dr. Catherine Neill taught me during my first year of clinical pedi-
atric cardiology at Johns Hopkins Hospital in Baltimore, Massachusetts, from 1959 to 1960. This
position was known as a Fellowship in Pediatric Cardiology.
Dr. Jeremy Swan, Dr. Owings Kincaid, and Dr. Alberto Barcia (a visiting radiologist from
Uruguay), and Dr. Jesse Edwards (a great pathologist) introduced me to cardiac catheterization
and angiocardiography at the Mayo Clinic in Rochester, Minnesota, from 1959-1961—one whole
year spent in this laboratory as a Fellow in Cardiopulmonary Physiology.
Dr. John D. Keith, Dr. Richard Rowe, Dr. Peter Vlad, and Dr. Stella Zacharioudaki
(my future wife and a pediatric cardiologist) at the Hospital for Sick Children in Toronto, Canada,
from 1961 to 1963. I was a Senior Fellow in Cardiology.
Dr. Maurice Lev at the Congenital Heart Disease Research and Training Center in Chicago,
Illinois, from 1963 to 1965, where I became an Assistant Professor of Pathology concentrating on
congenital cardiac morphology.
Dr. Robert DeHaan of the Carnegie Institute of Washington, where I did experimental and
observational embryology, from January 1 to June 30, 1966. My title was Visiting Scientist.
Dr. Alexander Nadas, Chief of the Department of Cardiology, Dr. Robert E. Gross, Chief of
the Department of Surgery, and Dr. Sidney Farber, Chief of the Department of Pathology—all
three invited me to become a member of the staff of Children’s Hospital Medical Center in Bos-
ton, Massachusetts. Dr. Stella Van Praagh, by then my wife, was included in this invitation.
The unfailing expert assistance of Elsevier was greatly appreciated.
Dr. Maurice Lev’s morphologic method in cardiac chamber identification has been an inspi-
ration. Dr. Jesse Edwards’ clinicopathologic correlation has been exemplary.
Dr. Richard Van Praagh founded the Cardiac Registry, where he did many of the lectures
for the medical fellows and staff, including some for only the nurses, and he also did most of the
cardiac dissections. He became Professor of Pathology at Harvard Medical School, Boston, Mas-
sachusetts, in 1974.
Dr. Stella Van Praagh was the Assistant Director of the Cardiac Registry, and her assistance
was enormously appreciated. She had the very important task of teaching our cardiac surgical
residents.
Drs. Richard and Stella Van Praagh retired in 2002. Dr. Stella Van Praagh died in 2006.
Dr. Richard Van Praagh did a “working retirement.” It is now 2021. In the last 19 years, he has
written 38 scientific papers and this book, Congenital Heart Disease. Following a Cardiac Sur-
gery Meeting in Kraków, Poland, he was invited to write a paper for his Polish colleagues that
resulted in the development of 15 new cardiovascular equations. These equations constitute a new
approach to the understanding of congenital heart disease.

vii
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 CONTENTS

SECTION I Historical Perspective 15 Infundibuloarterial Situs Equations: How Normally and

Abnormally Related Great Arteries Are Built and the
1 Brief History of the Cardiovascular System, 2 Importance of Infundibuloarterial Situs Concordance and
Discordance, 750
16 Ventricular Septal Defects, 767
SECTION II Cardiovascular Development 17 Single Ventricle, 818
2 Embryology and Etiology, 14 18 Superoinferior Ventricles, 834
19 Anomalous Infundibular Muscle Bundles, 848
20 Tetralogy of Fallot, 852
SECTION III Anatomic and Developmental 21 Absence of the Subpulmonary Infundibulum With
Approach to Diagnosis Its Sequelae Has Been Misinterpreted as Common
Aortopulmonary Trunk, 867
3 Morphologic Anatomy, 60 22 Transposition of the Great Arteries, 877
4 Segmental Anatomy, 89 23 Double-Outlet Right Ventricle, 916
24 Double-Outlet Left Ventricle, 932
25 Anatomically Corrected Malposition of the
SECTION IV Congenital Heart Disease Great Arteries, 948
5 The Congenital Cardiac Pathology Database, 106 26 What Prevents and What Permits the Embryonic Great
6 Systemic Venous Anomalies, 113 Arterial Switch?, 956
7 Pulmonary Venous Anomalies, 155 27 Infundibuloarterial Situs Equations and Analysis, 969
8 Cor Triatriatum Sinistrum (Subdivided Left Atrium) and 28 The Cardiac Conduction System, 976
Cor Triatriatum Dextrum (Subdivided Right Atrium), 234 29 The Heterotaxy Syndromes, 989
9 Interatrial Communications, 266 30 Conclusions, 1030
10 Juxtaposition of the Atrial Appendages, 320 APPENDIX 1 Understanding Normally and Abnormally
11 Common Atrioventricular Canal, 352 Related Great Arteries, 1033
12 Double-Outlet and Common-Outlet Right Atrium, 416 E-APPENDIX 2 Chronological List of Publications 1961 to
13 Tricuspid Valve Anomalies, 429 2021, 1047.e1
14 Mitral Valve Anomalies, 594 POST SCRIPTUM, 1048
INDEX, 1049

ix
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 SECTION I
Historical Perspective

1
 1
Brief History of the
Cardiovascular System

Who really discovered the circulation of the blood? Although blood is admixed with air, resulting in aerated (spirituous)
much has been written,1-­223 the correct answer to this funda- blood, as opposed to venous blood. Galen contended that
mental question remains largely unknown. The early history of venous blood passes from the right ventricle into the left
the development of understanding of the cardiovascular system ventricle via invisible pores in the interventricular septum.
may be summarized chronologically as follows: He also thought that a small amount of blood passes from
In 2625 BCE, the Chinese emperor Huang Ti (Nei Ching) the right ventricle via the pulmonary artery and lungs into
realized that the heartbeat and the pulse go together. He may the left heart. So Galen had a partial understanding of the
have had some understanding of the circulation of the blood, pulmonary (or lesser) circulation.
but evidence of this is lacking. In about 1250 CE, Ibn al-­Nafis deduced the existence of the
In 1550 BCE, the ancient Egyptians knew that the heart is pulmonary (lesser) circulation.2,3 He thought that the pulmo-
the center of the vascular system. But they did not distinguish nary trunk is too large to be there only for the nutrition of the
between arteries and veins. Regarding the pulse as the “voice” of lungs, and hence he deduced that the blood of the right heart
the heart, they developed an extensive pulse lore. must flow to the left heart via the large pulmonary trunk and
In 330 BCE, Praxagoras of Cos first distinguished between the lungs.
arteries and veins. This distinction was further developed by his In 1505, Leonardo da Vinci (Fig. 1.3) concluded that the
student, Herophilus of Alexandria, Egypt, about 300 BCE. human heart has “lower ventricles” (chambers) and “upper
In approximately 330 BCE, Aristotle (Aristoteles) (Fig. 1.1) ventricles” (chambers) and that the upper chambers have “ears”
discovered the cardiovascular system. Aristotle, the tutor and (auricles). Hence the concept of Rufus of Ephesus concern-
friend of Alexander the Great, was the first to realize that the ing the auricles, which the influential Galen had opposed, was
heart sits at the center of a system of arteries and veins. Aristotle finally accepted after a lapse of some 1500 years.
understood that both the arteries and the veins arise from the In 1543, Andreas Vesalius (Fig. 1.4) of Brussels, Belgium, in
heart. Prior to that time, the veins were thought to originate his epochal De Humani Corporis Fabrica, described no open-
from the liver. Aristotle said that the heart normally has three ings in the interventricular septum but did not flatly contradict
cavities (ventricles), apparently because he did not regard the Galen’s dogma of invisible pores.
right atrium as a cardiac chamber. Instead, he interpreted the In 1547, Giambattista Canano of Ferrara described a few of
right atrium as a dilatation of the great vein—our superior and the valves in the azygos vein.
inferior venae cavae.1 In 1553, Michael Servetus (Serveto) rediscovered the pul-
In 300 BCE, Herophilus and Erasistratus of Alexandria saw monary (lesser) circulation, apparently unaware of the prior
chyliferous vessels in the mesentery of sheep. Erasistratus dis- work of Ibn al-­Nafis. Servetus also denied the permeability of
covered that the heart is a pump. He was also the first to describe the ventricular septum. At the insistence of Calvin, Servetus was
and name the tricuspid and bicuspid (mitral) valves. However, burned at the stake in Geneva, Switzerland, on October 27, 1553,
Erasistratus thought that the arteries contain air (which they do because of his unorthodox views on the Christian Holy Trinity.
at autopsy, because the blood tends to drain into the more dis- All but two copies of his 1553 book, Christianismi Restituto (The
tensible veins). This erroneous concept impeded the discovery Restitution of Christianity), were also burned. Calvin is thought
of the circulation of the blood. to have strongly disapproved of an earlier work of Servetus, De
In 25 CE, Rufus of Ephesus named what we call the base of Trinitatis Erroribus libri septum (On the Errors of the Trinity, in
the heart the “head” of the heart. He observed that on each side of seven books), published in 1531.
the head of the heart there are soft, hollow, wing-­like things that In 1555, Andreas Vesalius, in the second edition of his
pulsate; he called them the “ears” of the heart because they were Fabrica, stated that he could not understand how any blood
on both sides of the head of the heart. “Ears” in Latin is “auriculae,” could pass through the ventricular septum. But he deduced
which became auricles in medical English. So Rufus realized that no further conclusions from this observation. We (R.V.P. and
the right atrium is indeed part of the heart because it beats, and he S.V.P.)a translated much of Book VI of the Fabrica in order to
was the first to distinguish between auricles and ventricles.1
In about 190 CE, Claudius Galen (Fig. 1.2) of Pergamos aS.V.P.was my dear departed wife, Dr. Stella Van Praagh. She was born
in Asia Minor showed that arteries contain blood, not air, in Crete, Greece, and was an expert in ancient Greek. I am an old Latin
as had been previously thought. Galen believed that arterial scholar; we worked together on the translation.

2
 CHAPTER 1 Brief History of the Cardiovascular System 3

Fig. 1.3 Leonardo da Vinci (1452–1519), Italian painter, sculptor, archi-

Fig. 1.1 Aristotle (384–322 BCE) discovered the cardiovascular system
tect, musician, engineer, and scientist. In 1505, he confirmed the dis-
about 330 BCE. Born in Stagira, Macedonia, he studied under Plato at
tinction between the upper cardiac chambers (“ventricles”) that have
the Academy (367–347 BCE), tutored Alexander the Great at the Mace-
auricles (ears), and the lower cardiac chambers (“ventricles”) that do
donian court (342–339 BCE), and opened his own school, the Lyceum,
not have auricles. This distinction between auricles and ventricles had
in Athens (335 BCE). His was also called the Peripatetic school because
first been made by Rufus of Ephesus in about 25 CE but had been
of his practice of lecturing in the Lyceum’s covered portico or walking
effectively opposed by Galen. (Reproduced from Hemmeter JC. The
place (peripatos). Aristotle was a one-­man university for the Athenians,
history of the circulation of the blood. In Hemmeter JC: Master Minds
the Arabs, the Jews, and later for the medieval Europeans.
in Medicine. New York: Medical Life Press; 1927:226.)

Fig. 1.2 Claudius Galen (c 130–201 CE) demonstrated that the arteries
contain blood, not air as had previously been thought. Artery means air-­ Fig. 1.4 Andreas Vesalius (1514–1564), Flemish anatomist from Brus-
containing in Greek (Greek arteria, from aer, air + terein, to keep or con- sels working in Padua, described no openings in the interventricular
tain). Galen was a Greek from Pergamos in Asia Minor. After studying in septum in his monumental work, De Humani Corporis Fabrica (1543).
Asia Minor, Greece, and Alexandria, he returned to Pergamos as physi- However, he did not flatly contradict Galen’s erroneous dogma of invis-
cian to the gladiators—where no doubt he witnessed spurting arteries. ible pores in the interventricular septum. Although Vesalius is now
From c 162 CE onward, he resided chiefly in Rome, where he became widely regarded as the father of modern anatomy and modern medi-
physician to Emperor Marcus Aurelius and wrote approximately 500 cine, he did not understand the circulation of the blood. (Reproduced
publications. Galen remained the pre-­eminent medical authority until with permission from Van Praagh R, Van Praagh S. Aristotle’s “triventric-
the publication by Andreas Vesalius (1514–1564) of De Humani Corporis ular” heart and the relevant early history of the cardiovascular system.
Fabrica (The Construction of the Human Body) in 1543. Chest 1983;84:462.)
4 SECTION I Historical Perspective

see if Vesalius understood that the blood circulates, that is, flows
in a circle. No evidence was found that he did.
In 1559, Matteo Realdo Columbo described the pulmonary
circulation and the impermeability of the ventricular septum,
claiming both as his discoveries.
In 1564, Bartholomaeus Eustacheus discovered the thoracic
duct in the horse.
In 1571, Andreas Cesalpinus (Andrea Cesalpino in Italian),
the physician and philosopher of Arezzo, first described the cir-
culation of the blood in his Peripateticarum Quaestionum libri
quinque (Of Peripatetic Questions in five books).4 Regarding the
title of his work, it should be recalled that the school of Aristotle
(see Fig. 1.1) was known as the Peripatetic school. Hence peri-
patetic questions are scientific and philosophic matters consid-
ered in the Aristotelian way.
Cesalpino stated that the blood is in constant transition from
arteries to veins in all parts of the body by means of tiny anasto-
moses that he defined as “vasa in capillamenta resoluta” (vessels
resolved into hairlike vessels). This is where our term capillary
comes from; capillus means hair (Latin).
Cesalpino coined the term circulatio, by which he meant Fig. 1.5 William Harvey of Folkestone, Kent, England, was the third
that the blood flowed in a circle: from the veins to the right “discoverer” of the circulation (in 1628), after Cesalpino (1571, 1583,
heart, then to the lungs, thence to the left heart, and then to the 1593) and Sarpi (1623). Harvey deserves the credit for having persuaded
arteries. the medical world, despite considerable opposition, that the blood does
indeed circulate.
Cesalpino also realized that the pulmonary artery is an artery
(not a vein, as Galen had said) and that the pulmonary vein is a
vein (not an artery, as Galen had stated). function correctly. Fabricius was the tutor of William Harvey at
In 1583, Andrea Cesalpino published De Plantis (Of Plants), Padua (1598–1602).
in which he confirmed his theory that the blood circulates.4 In Milan in 1622, Gaspare Aselli observed chyliferous vessels
In 1593, Andrea Cesalpino published Questionum in the mesentery of the dog and called them lactiferous vessels.
Medicarum libri II (Medical Questions in two books) in which In 1623, Paolo Sarpi, the famous theologian and canon-
he gave experimental proof of the circulation of the blood. ist of the republic of Venice, as well as a student and friend of
These were the same two experiments later used by Harvey4: Fabricius, described the function of the venous valves correctly.
1. Cesalpino observed that when a vein is first divided, dark From this understanding, he deduced the circulation of the
venous blood comes out, which then becomes lighter and blood. Unfortunately, however, the manuscripts of Sarpi in the
lighter in color, which favored the theory that the blood is library of the Servitians at Venice were destroyed in a monastery
circulating. fire in September 1769. Hence, Paolo Sarpi may be regarded as
2. Cesalpino also observed that when veins are occluded, they the second “discoverer” (or first rediscoverer after Caesalpinus)
always swell between the ligature and the capillaries, not of the circulation of the blood.
between the ligature and the heart—as they should if Galen’s In 1628, William Harvey (Fig. 1.5) of Folkestone, Kent,
concept of centrifugal venous blood flow were correct. England, described the circulation of the blood in a 72-­page
Consequently, as the American physician, linguist, and his- book titled Exercitatio Anatomica de Motu Cordis et Sanguinis
torian John C. Hemmeter4 wrote in 1927, to Andrea Cesalpino in Animalibus (Anatomic Experience Concerning the Motion of
of Arezzo “belongs the fame of being the first to have recog- the Heart and Blood in Animals), and he demonstrated the cir-
nized and demonstrated the general circulation of the blood.” culation experimentally. After the publication of his little book
In 1583, in his outstanding work De Plantis, Cesalpino stated: at Frankfurt, Harvey was accused of plagiarism by many of his
the blood is led through the veins to the heart, and is distributed contemporaries (Micanzio, Vesling, Walaens, Riolan, Bartholin,
by the arteries to the entire body (... sanguiinem per venas duci and others). Harvey is now considered to have been the third
ad cor, et per arterias in universum corpus distribui). Cesalpino “discoverer” (or the second rediscoverer, after Sarpi) of the
discovered the circulation of the blood without knowing about circulation of the blood.4 Harvey deserves the credit not for
the valves in the veins. discovering the circulation of the blood but for eventually per-
In 1598, Carlo Ruini of Bologna published a book on the suading the scientific world, over considerable opposition, that
anatomy and diseases of the horse, in which it is clear that he the blood does indeed circulate.
understood the function of the valves of the heart. In 1648, while a student in Paris, Jean Pecquet discovered
In 1603, Hieronymus Fabricius ab Aquapendente (Fabrizio the thoracic duct in humans and realized that the lactiferous
in Italian) described valves in the entire venous system in his vessels do not return to the liver but to the thoracic duct and
work De Venarum Ostiolis, but he did not understand their thence to the left subclavian vein.
 CHAPTER 1 Brief History of the Cardiovascular System 5

In 1650, Olaf Rudbeck was a student at Uppsala University Corrected transposition of the great arteries was not discov-
when he discovered the lymphatics of the liver and found that ered and reported until 1875 by Carl von Rokitansky (1804–
they too drained into the thoracic duct. 78) of Vienna.9
In 1661, at the University of Bologna, Marcello Malpighi, Thus, the seeds of the understanding of congenital heart dis-
using a microscope, was the first to observe the motion of the ease were sown in the 19th century by Thomas B. Peacock,10
blood in the capillaries of the lung in frogs. Malpighi had dis- Carl von Rokitansky,9 E. Théremin,11 and others. It was in the
covered the postulated capillaries of Caesalpinus. 20th century that these seeds came to fruition. Many books
In 1771, Lazzaro Spallanzani, again with the aid of a micro- appeared dealing with different aspects of heart in infants and
scope, saw blood flowing in the umbilical vessels of the chick children—cardiology, radiology, echocardiography, epidemi-
embryo. This was the first direct observation of the circulation ology, surgery, pathology, embryology, and etiology. Some of
of the blood in a warm-­blooded animal. these books are listed alphabetically in Box 1.1.
Thus, the normal circulation of the blood and lymph was “Are there any good books on congenital heart disease?” I am
discovered and documented over approximately 4,400 years often asked. My answer to this important question is yes. But a
from 2625 BCE (Huang Ti; Nei Ching) to 1771 CE (Lazzaro list of “good” books on congenital heart disease is surprisingly
Spallanzani). hard to find, hence Box 1.1. I hope that this box of “old friends,”
The story of the discovery and documentation of the abnor- which is by no means complete, will serve as a helpful reference
mal circulation of the blood in infants and children is much list for students and investigators.
more recent, being little more than 330 years old. Very largely, The history of the development of our understanding of
this is the history of congenital (as opposed to acquired) heart congenital heart disease in the 20th century is clearly indi-
disease in infants and children. cated—concerning any subject—by studying these books
For example: chronologically (see Box 1.1).
The anomaly now known as the tetralogy of Fallot was first I would like to salute the investigators cited in Box 1.1, many
described by the Danish physician/priest Niels Stensen of whom are friends. They and their colleagues are largely respon-
(1648–86) in 1671,6 the patient being a macerated stillborn sible for the remarkable progress that has occurred in pediatric
fetus with cervical ectopia cordis. cardiology and cardiac surgery during the 20th century.
The malformation now known as transposition of the In addition to the books referred to in Box 1.1 that are con-
great arteries was discovered and described by Matthew cerned in part with the history of heart disease in infants and
Baillie (1761–1823) of London, England, in 1797. 7 How- children, there are also many fascinating books that focus mainly
ever, by the dawn of the 19th century, congenital heart on history. Some of my favorites include the works of Garrison,5
disease remained mostly unknown. Single left ventri- Majno,220 Lyons and Petrucelli,221 East,222 and Harris.223
cle (LV) with absence of the right ventricular sinus and Is an understanding of history important to the compre-
double-­inlet LV was not discovered and reported until hension of congenital heart disease? The brief answer is yes.
1824 by Andrew F. Holmes (1797–1860) of Montreal, Consequently, history is often an integral part of the presenta-
Canada. 8 tion concerning many types of congenital heart disease.

BOX 1.1 Some of the Books Concerning Heart Disease in Infants and Children That Have
Appeared During the 20th Century
Authors and References Bharati S, Lev M36,37
Abbott ME12,13 Bharati S, Lev M, Kirklin JW38
Adams FH, Emmanouilides GC14 Bianchi T, Invernizzi G, Parenzan L39
Adams FH, Emmanouilides GC, Riemenschneider TA15 Bourne GH40
Albou E, Lanfranchi J, Piton J-­L, LeGoubey J16 Boyd W41
Allwork SP17 Braunwald E42
Anderson RH, Shinebourne EA20 Bremer JL43
Anderson WAD18,19 Bucharin VA, Podzolkov VP44
Ando M21,22 Burakovsky VI, Bukharin VA, Bockeria LA45
Anselmi G, Munoz H, Espino Vela J, Arguello C23 Cassells DE46
Arey LB24 Castañeda AR, Jonas RA, Mayer JE, Hanley FL47
Aziz KU25 Castellanos y Gonzalez48
Bailey FR, Miller AM26 Christidès C, Cabrol C49
Bankl27,28 Clark EB, Markwald RR, Takao A50
Barratt-­Boyes BG, Neutze JM, Harris EA29 Clark EB, Takao A51
Barth LG30 Cooley DA, Hallman GL52
Becker AE, Anderson RH31 Corone P53
Behrman RE, Kliegman RM, Nelson WE, Vaughan VC III32 Cotran RS, Kumar V, Robbins SL54
Bergsma D, McKusick VA, Neill C, Rowe R, Lindstrom J, Jackson C, Rogers J33 Crupi G, Parenzan L, Anderson RH55
Berri GG34 Davies MJ, Anderson RH, Becker AE56
Beuren AJ35 Davis JA, Dobbing J57
Continued