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

Published online by Cambridge University Press:  09 January 2014

Robert H. Anderson ,
Diane E. Spicer ,
Jorge M. Giroud  and
Timothy J. Mohun
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]
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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.

Keywords

Ventricular septal defectinterventricular communicationcardiac developmentterminology
Type
Original Article
Information
Cardiology in the Young , Volume 23 , Issue 6: HeartWeek 2013 , December 2013 , pp. 858 - 866
Copyright
Copyright © Cambridge University Press 2013 

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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].

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