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Cor triatriatum or divided atriums: which approach provides the better understanding?

Published online by Cambridge University Press:  13 May 2014

Tara Bharucha ,
Diane E. Spicer ,
Timothy J. Mohun ,
David Black ,
G. William Henry  and
Robert H. Anderson
Tara Bharucha
Affiliation:
Department of Paediatric Cardiology, University Hospital Southampton NHS Foundation Trust, United Kingdom
Diane E. Spicer
Affiliation:
Department of Pediatric Cardiology, University of Florida, Gainesville, Florida, United States of America Congenital Heart Institute of Florida, St Petersburg, Florida, United States of America
Timothy J. Mohun
Affiliation:
Division of Developmental Biology, MRC National Institute for Medical Research, London, United Kingdom
David Black
Affiliation:
Department of Paediatric Cardiology, University Hospital Southampton NHS Foundation Trust, United Kingdom
G. William Henry
Affiliation:
Emeritus Founding Editor, Chapel Hill, North Carolina, United States of America
Robert H. Anderson*
Affiliation:
Department of Paediatric Cardiology, University Hospital Southampton NHS Foundation Trust, United Kingdom Institute of Genetic Medicine, Newcastle University, Newcastle, United Kingdom
*
Correspondence to: Professor R. H. Anderson, 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 frequent, in the current era, to encounter congenital cardiac malformations described in terms of “cor triatriatum”. But can hearts be truly found with three atrial chambers? The morphological method, emphasised by Van Praagh et al, states that structures within the heart should be defined on the basis of their most constant components. In the atrial chambers, it is the appendages that are the most constant components, and to the best of our knowledge, hearts can only possess two appendages, which can be of either right or left morphology. The hearts described on the basis of “cor triatriatum”, nonetheless, can also be analysed on the basis of division of either the morphologically right or the morphologically left atriums. In this review, we provide a description of cardiac embryology, showing how each of the atrial chambers possesses part of the embryological body, along with an appendage, a vestibule, a venous component, and a septum that separates them. We then show how it is, indeed, the case that the hearts described in terms of “cor triatriatum” can be readily understood on the basis of division of these atrial components. In the right atrium, it is the venous valves that divide the chamber. In the left atrium, it is harder to provide an explanation for the shelf that produces atrial division. We also contrast the classic examples of the divided atrial chambers with the vestibular shelf that produces supravalvar stenosis in the morphologically left atrium, showing that this form of obstruction needs to be distinguished from the fibrous shelves producing intravalvar obstruction.

Keywords

Embryologypersistent venous valvessupramitral stenosispersistent left superior caval vein
Type
Review Articles
Information
Cardiology in the Young , Volume 25 , Issue 2 , February 2015 , pp. 193 - 207
Copyright
Copyright © Cambridge University Press 2014 

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