Hostname: page-component-586b7cd67f-t7fkt Total loading time: 0 Render date: 2024-11-27T01:02:24.888Z Has data issue: false hasContentIssue false

Tetralogy of Fallot: nosological, morphological, and morphogenetic considerations*

Published online by Cambridge University Press:  09 January 2014

Robert H. Anderson*
Affiliation:
Institute of Genetic Medicine, Newcastle University, Newcastle upon Tyne, United Kingdom
Diane E. Spicer
Affiliation:
All Children's Hospital, The Congenital Heart Institute of Florida, Saint Petersburg/Tampa and the University of Florida, Gainesville, United States of America
Jorge M. Giroud
Affiliation:
All Children's Hospital, The Congenital Heart Institute of Florida, Pediatric Cardiology Associates/Pediatrix Medical Group, Saint Petersburg and Tampa, Florida, United States of America
Timothy J. Mohun
Affiliation:
National Institute of Medical Research, London, United Kingdom
*
Correspondence to: Dr R. H. Anderson, BSc, MD, FRCPath, 60 Earlsfield Road, London SW18 3DN, United Kingdom. Tel: 00-44-20-8870-4368; E-mail: [email protected]

Abstract

It is timely, in the 125th anniversary of the initial description by Fallot of the hearts most frequently seen in patients presenting with “la maladie bleu”, that we revisit his descriptions, and discuss his findings in the light of ongoing controversies. Fallot described three hearts in his initial publication, and pointed to the same tetralogy of morphological features that we recognise today, namely, an interventricular communication, biventricular connection of the aorta, subpulmonary stenosis, and right ventricular hypertrophy. In one of the hearts, he noted that the aorta arose exclusively from the right ventricle. In other words, one of his initial cases exhibited double-outlet right ventricle. When we now compare findings in hearts with the features of the tetralogy, we can observe significant variations in the nature of the borders of the plane of deficient ventricular septation when viewed from the aspect of the right ventricle. We also find that this plane, usually described as the ventricular septal defect, is not the same as the geometric plane separating the cavities of the right and left ventricles. This means that the latter plane, the interventricular communication, is not necessarily the same as the ventricular septal defect. We are now able to provide further insights into these features by examining hearts prepared from developing mice. Additional molecular investigations will be required, however, to uncover the mechanisms responsible for producing the morphological changes underscoring tetralogy of Fallot.

Type
Original Article
Copyright
Copyright © Cambridge University Press 2013 

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

Footnotes

*

Presented at “The Birth of Heart Surgery: Lessons Learned from Tetralogy – Past, Present and Future” Dinner Symposium Sponsored by Johns Hopkins Medicine and All Children's Hospital, Thursday, February 21, 2013, at The Sixth World Congress of Paediatric Cardiology and Cardiac Surgery, Cape Town International Convention Centre, Cape Town, South Africa, February 17–22, 2013. A video of this presentation can be viewed at the following hyperlink: [http://www.allkids.org/wcpccs].

References

1.Fallot, ELA. Contribution à l'anatomie pathologique de la maladie bleue (cyanose cardiaque). Marseille médical 1888; 25: 7793.Google Scholar
2.Abbott, ME. Atlas of Congenital Cardiac Disease. American Heart Association, New York, 1936: 4647.Google Scholar
3.Allwork, SP. Tetralogy of Fallot: the centenary of the name. A new translation of the first of Fallot's papers. Eur J Cardiothorac Surg 1988; 2: 386392.Google Scholar
4.Van Praagh, R. Etienne-Louis Arthur Fallot and his tetralogy: a new translation of Fallot's summary and a modern reassessment of this anomaly. Eur J Cardiothorac Surg 1989; 3: 381386.Google Scholar
5.Anderson, RH, Becker, AE, Van Mierop, LHS. What should we call the “crista”? Br Heart J 1977; 39: 856859.Google Scholar
6.Merrick, AF, Yacoub, MH, Ho, SY, Anderson, RH. Anatomy of the muscular subpulmonary infundibulum with regard to the Ross procedure. Ann Thorac Surg 2000; 69: 556561.Google Scholar
7.Anderson, RH, Allwork, SP, Ho, SY, Lenox, CC, Zuberbuhler, JR. Surgical anatomy of tetralogy of Fallot. J Thorac Cardiovasc Surg 1981; 81: 887896.Google Scholar
8.Suzuki, A, Ho, SY, Anderson, RH, Deanfield, JE. Further morphologic studies on tetralogy of Fallot, with particular emphasis on the prevalence and structure of the membranous flap. J Thorac Cardiovasc Surg 1990; 99: 528535.CrossRefGoogle Scholar
9.Van Praagh, R, Van Praagh, S, Nebesar, RA, Muster, AJ, Sinha, SN, Paul, MH. Tetralogy of Fallot: underdevelopment of the pulmonary infundibulum and its sequel. Am J Cardiol 1970; 26: 2433.CrossRefGoogle Scholar
10.Becker, AE, Connor, M, Anderson, RH. Tetralogy of Fallot: a morphometric and geometric study. Am J Cardiol 1975; 35: 402412.CrossRefGoogle Scholar
11.Howell, CE, Ho, SY, Anderson, RH, Elliott, MJ. Variations within the fibrous skeleton and ventricular outflow tracts in tetralogy of Fallot. Ann Thorac Surg 1990; 50: 450457.Google Scholar
12.Anderson, RH, Weinberg, PM. The clinical anatomy of tetralogy of Fallot. Cardiol Young 2005; 15 (Suppl 1): 3847.Google Scholar
13.Anderson, RH, Aiello, VD, Spicer, DE, Jacobs, JP, Giroud, JM. Fallot's tetralogy: anatomical observations and controversies. Congenit Cardiol Today 2013; in press.Google Scholar
14.Baker, E, Leung, MP, Anderson, RH, Fischer, DR, Zuberbuhler, JR. The cross – sectional anatomy of ventricular septal defects: a reappraisal. Br Heart J 1988; 59: 339351.Google Scholar
15.Milo, S, Ho, SY, Wilkinson, JL, Anderson, RH. Surgical anatomy and atrioventricular conduction tissues of hearts with isolated ventricular septal defects. J Thorac Cardiovasc Surg 1980; 79: 244255.CrossRefGoogle Scholar
16.Abbott, ME. Atlas of Congenital Cardiac Disease. American Heart Association, New York, 1936: 23.Google Scholar
17.Mohun, TJ, Weninger, WJ. Imaging heart development using high-resolution episcopic microscopy. Curr Opin Genet Dev 2011; 21: 573578.Google Scholar