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Echo-morphological correlates concerning the functionally univentricular heart in the setting of isomeric atrial appendages

Published online by Cambridge University Press:  10 January 2006

William T. Mahle ,
Norman H. Silverman ,
Gerald R. Marx  and
Robert H. Anderson
William T. Mahle
Affiliation:
Children's Healthcare of Atlanta and the Emory University School of Medicine, Atlanta, Georgia, United States of America
Norman H. Silverman
Affiliation:
Lucille Packard Children's Hospital, Stanford University School of Medicine, Palo Alto, California, United States of America
Gerald R. Marx
Affiliation:
Children's Hospital, Harvard University School of Medicine, Boston, Massachusetts, United States of America
Robert H. Anderson
Affiliation:
Cardiac Unit, Institute of Child Health, University College, London, United Kingdom
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Extract

It has long been known that the most complex combinations of cardiac malformations are those found in the setting of the so-called “splenic syndromes”.1 Many aspects of these syndromes have been controversial over recent years, not least the presence or absence of features of isomerism within the heart.2,3 Recent experience with genetic manipulation of mice, nonetheless, has now shown that it is possible to generate unequivocal evidence of cardiac isomerism, particularly in those animals which show features of right isomerism when the genes responsible for morphologically leftness are knocked out.4 Furthermore, when the crucial philosophical principle known as the “morphological method”5 is applied to the hearts of patients known to have visceral heterotaxy, it is equally clear that patients falling within these groups, when judged on the extent of the pectinate muscles relative to the atrioventricular junctions, exhibit isomerism of either the morphologically right or left atrial appendages.3

Keywords

Aspleniapolyspleniavisceral heterotaxydouble inlet ventricle
Type
Research Article
Information
Cardiology in the Young , Volume 16 , Supplement S1: Controversies and Challenges in the Management of the Functionally Univentricular Heart , February 2006 , pp. 35 - 42
Copyright
© 2006 Cambridge University Press

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