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Atrioventricular septal defect with coexisting tricuspid atresia

Published online by Cambridge University Press:  25 January 2011

Vera Demarchi Aiello ,
Jorge Yussef Afiune ,
Samuel Menahem  and
Robert H. Anderson
Vera Demarchi Aiello*
Affiliation:
Heart Institute, University of São Paulo Medical School, São Paulo, Brazil
Jorge Yussef Afiune
Affiliation:
Heart Institute, University of São Paulo Medical School, São Paulo, Brazil
Samuel Menahem
Affiliation:
Monash Heart, Monash Medical Centre, Monash University, Melbourne, Australia
Robert H. Anderson
Affiliation:
Institute of Child Health, Great Ormond Street Hospital, London
*
Correspondence to: Dr V. D. Aiello, Laboratory of Pathology, Av. Dr Enéas C. Aguiar, 44, São Paulo 05403-000, Brazil. Tel: +55 11 3069-5252; Fax: +55 11 3069-5279; E-mail: vera.aiello@incor.usp.br
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Abstract

We describe two infants having an atrioventricular septal defect in the setting of a double inlet atrioventricular connection, but with patency of the left-sided valvar orifice and an imperforate right-sided valvar component, and a further case with atrioventricular septal defect and an imperforate Ebstein's malformation, all producing the haemodynamic effect of tricuspid atresia. We make comparisons with the arrangement in trisomy 16 mice, in whom deficient atrioventricular septation is seen at times with the common atrioventricular junction exclusively connected to the left ventricle, a situation similar to that seen in two of our infants. We also review previous reports emphasising the important theoretical implication of the findings despite their rarity.

Keywords

Double inlet left ventricleimperforate atrioventricular valveostium primum
Type
Original Articles
Information
Cardiology in the Young , Volume 21 , Issue 3 , June 2011 , pp. 299 - 302
Copyright
Copyright © Cambridge University Press 2011

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References

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