Maude Abbott Pediatric Cardiovascular Symposium
Moderator: Glenn Taylor
Section 2 -
Maude Abbott and the Congenitally Malformed Heart
Robert H. Anderson
UCL Institute of Child Health
London , United Kingdom
In the frontispiece to her "Atlas of Congenital Cardiac Disease" , Maude Abbott pictures
the five "great scientists who have made fundamental contributions to our knowledge of congenital cardiac
disease". She chose a Frenchman, Jean-Baptiste de Senac, an Italian, Giovanni Baptista Morgagni, an
Austrian, the Baron von Rokitansky, and two physicians and scientists from the United Kingdom, Thomas
Bevill Peacock and Arthur Keith. One cannot quarrel with her choice. There can be no question, however,
but that Abbott's own contributions took our understanding of the congenitally malformed heart to new
heights. The contributions of her great scientists were important, and Rokitansky's own
Atlas  illustrates many, if not most, of the malformations we recognise today, establishing
for example the existence of congenitally corrected transposition (Fig. 1). Keith also made significant
advances, not only clarifying the disposition of the atrioventricular conduction axis , and
discovering the sinus node , but also discussing the morphogenesis of malformations involving
the ventricular outflow tracts . It was Abbott, however, who first gathered together and
synthesised the existing descriptions of congenital cardiac malformations, which still tended to appear
as isolated reports during her long career. Most importantly, she then grouped them together in
clinically meaningful fashion. We continue to describe the malformations in the fashion she suggested,
dividing them into acyanotic and cyanotic lesions, and further dividing the cyanotic group into those
with late reversal of an arterio-venous shunt, as opposed to those with a permanent right-to-left shunt,
or retardation of flow. We can also continue to learn from her Atlas, which contains an analysis of
1,000 cases, combining the autopsy and clinical findings in a table with 24 sub-sections, a format which
could serve perfectly well for current listings. In her introduction, Abbott states "the Atlas here
published is merely preliminary to a larger volume of congenital heart disease which the author has under
preparation". As far as I am aware, this textbook never materialised, despite the fact that the Atlas
was published in 1936, and Abbott lived on until 1940. Had the textbook appeared as she promised, our
appreciation of the many variations in congenital cardiac disease might have been further enhanced. As
it is, it was entirely appropriate that she should have entered the pantheon of giants in their field
included in the mural of Diego Rivera that is housed in the Institute of Cardiology in Mexico City (Fig.
Abbott rightly states that "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" . Her emphasis on the reptilian heart is also justified, albeit that she followed
the concept in vogue at that time, namely that hearts with an aorta arising from the morphologically
right ventricle, such as transposition, could be explained on the basis of persistence of the right
ventricular aorta to be found in animals such as turtles, crocodiles, and alligators (Fig. 3). We now
know that this concept, despite the plausible suggestions made by Abbott , has no basis in
either anatomic or developmental fact. There is much to be learned, nonetheless, from these fascinating
reptilian hearts, not least the four-chambered crocodilian heart, with its foramen of Pannizza and truly
septated outflow tract (Fig. 4). Certainly there is much to be learned from the development of these
hearts. The avian heart, for example, more closely related to the reptiles than to the mammals, still
retains three cushions within its developing outflow tract, albeit that eventually it becomes separated
into two rather than three arterial channels .
Congenitally Malformed Hearts
In her formidable table, Abbott provides clinical and pathological details concerning virtually all
the malformations we recognise today. Constraints of time will prevent me from listing all the gems
still to be gleaned from her writings and drawings. Instead, I will select some of the most appropriate
to modern day paediatric cardiology, while also highlighting some features of more historical interest.
Malformations of the aortic arch
Twice in the Atlas Abbott illustrates what she describes as a "long ligamentum arteriosum". She
illustrates the structure as extending from the left pulmonary artery to the descending aorta (Fig. 5).
Her explanation for this lesion is unlikely to be correct, since the left-sided arterial duct, when
existing with a right-sided aortic arch, passes from the left-sided brachiocephalic artery to the left
pulmonary artery. Thus, if such a cord existed in this specimen, from the McGill museum, then we know
from the studies of Edwards and his colleagues  that it would represent an atretic left arch,
with interruption of the arterial ligament. Intriguingly, Abbott also illustrates a specimen with such
an atretic component of the left arch (Fig. 6). Her explanation for the long arterial ligament,
nonetheless, seems most unlikely, but if the specimen still exists, it is worthy of further study.
The section devoted to coarctation is comprehensive, and all of the findings are as relevant today as
when first described. Her reproduction of the picture of Meckel is particularly appropriate. She also
shows an exquisite chest radiograph provided by "Douglas Wilkinson of Birmingham , England ." Discovering
this part of the Atlas had direct relevance to me, since Dr Wilkinson was the father of James Wilkinson,
who collaborated with me in producing so many of our own investigations of development and structure of
the congenitally malformed heart
Jim still has a copy of a letter from Maude, arranging
a visit to Birmingham in 1932 (Fig. 7). Jim is currently the Senior Paediatric Cardiologist at Royal
Children's Hospital, Melbourne , and has himself made many significant contributions to our understanding
of the congenitally malformed heart .
The Arterial Valves
The coverage of abnormalities of the aortic valve is just as thorough as that for aortic coarctation.
Abbott stresses the semilunar attachments of the leaflets, and illustrates the important interleaflet
triangles. These structures tended to be forgotten in the decades that followed , but they
represent a feature which still demands attention, with uncertainty remaining as to the precise
definition of the valvar "annulus" .
The Arterial Duct
The page devoted to the arterial duct is particularly beautiful, with some excellent diagrams drawn by
her colleague, Dr Segall, to show the location of the murmur, and how its character changes with posture
(Fig. 8). Abbott points out that the allegedly typical "machinery" murmur  is heard in only
one-third of cases, basing this analysis on details gathered together from 92 previously published cases.
Deficient Atrial Septation
The writing of this section would give great problems in terms of current political correctness, since
she refers to "Mongolian idiocy", but Abbott rightly highlights the important morphological differences
between defects within the oval fossa, and those involving deficient atrioventricular septation, albeit
that like so many authors, she describes "cleavage and insufficiency of the anterior mitral segment".
From her illustrations, nonetheless, we can distinguish the difference between the normal mitral valve
seen in the setting of the deficiency within the oval fossa, and the trifoliate valve in the setting of
the common atrioventricular junction . Significantly, she does not discuss either the sinus
venosus defect, nor the defect found at the mouth of the coronary sinus.
Deficient Ventricular Septation
Considering that defects of the ventricular septum are the commonest type of congenital cardiac
malformation, her coverage of this lesion is perhaps unduly short. And she describes only defects at the
base, which we would now call perimembranous, and "other or multiple defects". She does, however,
emphasise the importance of bacterial endocarditis, also illustrating this complication in the setting of
a ruptured aneurysm of the aortic sinus of Valsalva. In subsequent sections, she illustrates and
comments on deficient ventricular septation in combination with other lesions, such as tetralogy of
Fallot and common arterial trunk. She also indicates the origin of "Eisenmenger's complex" – the name we
now use to describe overriding of the aorta in the absence of subpulmonary stenosis . As
Abbott explains, the term was coined by her "in default of a better". Present day confusion arose only
when Wood  subsequently described the "Eisenmenger reaction", accounting for the shift to
overt cyanosis in Abbott's grouping of "cyanosis tardive".
Pulmonary Atresia with Closed Ventricular Septum
Abbott shows a remarkable understanding of this lesion, along with what we now call hypoplasia of the
left heart, which is way ahead of her time. She emphasises the potential inflammatory origin, or valvar
endocarditis, pointing out that the lesion sets in "in later foetal life after the heart chambers are
Congenitally Corrected Transposition
Although listing only 4 examples of the lesion characterised by discordant connections at both
atrioventricular and ventriculo-arterial junctions, and collecting 16 examples of "partial
transposition", which we now call double outlet right ventricle , her illustration of the
lesion is exquisite, and parallels that of von Rokitansky in providing the necessary understanding of the
Double Inlet Left Ventricle
It is for her description of the so-called "Holmes Heart" that most of us now remember Maude Abbott,
not least because the heart itself is so beautifully preserved in the McGill museum, despite having been
first described by Andrew Holmes in 1824 . Intriguingly, Abbott does not describe the
chamber supporting the pulmonary trunk as the incomplete right ventricle, whilst one of her illustrations
of the commoner variant of double inlet left ventricle with discordant ventriculo-arterial connections
labels the smaller ventricular chamber as the "rudimentary left ventricle".
Although we can find fault with some of her descriptions, and not surprisingly our understanding has
moved on since the appearance of her Atlas, the beginner in paediatric cardiology today could do much
worse than to read thoroughly the works of Maude Abbott. She was truly the giant who provided the firm
foundations of our modern-day knowledge.
- Abbott ME. Atlas of Congenital Cardiac Disease. New York: The American Heart Association, 1936.
- von Rokitansky CF. Die Defecte der Scheidewande des Herzens. Vienna: Pathologisch-Anatomisch Wilhelm Braumuller, 1875.
- Keith A, Flack M. The auriculo-ventricular bundle of the human heart. Lancet 1906; 2:359-364.
- Keith A, Flack M. The form and nature of the muscular connections between the primary divisions of the vertebrate heart. J Anat Physiol 1907; 41:172-189.
- Keith A. Fate of the bulbus cordis in the human heart. Lancet 1924; 2:1267-1273.
- Qayyum S, Webb S, Brown NA, Anderson RH, Verbeek FJ, Richardson MK. Septation and valvar formation in the outflow tract of the embryonic chick heart. Anat Rec 2001; 264:273-283.
- Stewart JR, Kincaid OW, Edwards JE. An Atlas of Vascular Rings and Related Malformations of the Aortic Arch System. Springfield Ill: Charles C. Thomas 1964.
- Anderson RH, Wilkinson JL, Arnold R, Becker AE, Lubkiewicz K. Morphogenesis of bulboventricular malformations. II. Observations on malformed hearts. Br Heart J 1974; 36:948-970.
- Anderson RH, Arnold R, Wilkinson JL. The conducting system in congenitally corrected transposition. Lancet 1973; 1:1286-1288.
- Wilkinson JL, Arnold R, Anderson RH, Acerete F. 'Posterior' transposition reconsidered. Br Heart J 1975; 37:757-766.
- Wilkinson JL, Acerete F. Terminological pitfalls in congenital heart disease. Reappraisal of some confusing terms, with an account of a simplified system of basic nomenclature. Br Heart J 1973; 35:1166-1177.
- Anderson RH. Demystifying the anatomic arrangement of the aortic valve. Editorial Comment. Eur J Cardio-thorac Surg 2006; 29:1006-1007.
- Sutton JPIII, Ho SY, Anderson RH. The forgotten interleaflet triangles: A review of the surgical anatomy of the aortic valve. Ann Thorac Surg 1995;59:419-427.
- Tynan M. The murmur of the persistently patent arterial duct, or "The Colonel is going to a dance". Cardiol Young 2003; 13:559-562.
- Anderson RH, Ho SY, Falcao S, Daliento L, Rigby ML. The diagnostic features of atrioventricular septal defect with common atrioventricular junction. Cardiol Young 1998; 8:33-49.
- Oppenheimer-Dekker A, Gittenberger-de Groot AC, Bartelings MM, Wenink ACG, Moene RJ, Van der Harten JJ. Abnormal architecture of the ventricles in hearts with an overriding aortic valve and a perimembranous ventricular septal defect ("Eisenmenger VSD"). Int J Cardiol 1985; 9:341-355.
- Wood P. The Eisenmenger syndrome or pulmonary hypertension with reversed central shunt. Br Med J 1958; 2:701-709.
- Wilkinson JL. Double outlet ventricle. In: Anderson RH, Baker EJ, Macartney FJ, Rigby ML, Shinebourne EA, Tynan M, (eds). Paediatric Cardiology Second Edition. Edinburgh: Churchill Livingstone, 2002: 1353-1382.
- Holmes AF. Case of malformation of the heart. Trans Med-Chir Soc Edinb 1824; 1:252-259.