Cardiology in the Young

Table of Contents - 2006 - Volume 16, Supplement S1 (Controversies and Challenges in the Management of the Functionally Univentricular Heart)  

Controversies and Challenges in the Management of the Functionally Univentricular Heart

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

William T. Mahle a1c1, Norman H. Silverman a2, Gerald R. Marx a3 and Robert H. Anderson a4
a1 Children's Healthcare of Atlanta and the Emory University School of Medicine, Atlanta, Georgia, United States of America
a2 Lucille Packard Children's Hospital, Stanford University School of Medicine, Palo Alto, California, United States of America
a3 Children's Hospital, Harvard University School of Medicine, Boston, Massachusetts, United States of America
a4 Cardiac Unit, Institute of Child Health, University College, London, United Kingdom

Article author query
mahle wt   [PubMed][Google Scholar] 
silverman nh   [PubMed][Google Scholar] 
marx gr   [PubMed][Google Scholar] 
anderson rh   [PubMed][Google Scholar] 

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


Key Words: Asplenia; polysplenia; visceral heterotaxy; double inlet ventricle.

Correspondence:
c1 Correspondence to: William T. Mahle MD, Children's Healthcare of Atlanta, Emory University School of Medicine, 1405 Clifton Road, NE, Atlanta, GA 30322-1062, USA. Tel: +1 404 315 2672; Fax: +1 404 325 6021; E-mail: mahlew@kidsheart.com

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