Hostname: page-component-586b7cd67f-rcrh6 Total loading time: 0 Render date: 2024-11-28T03:25:07.477Z Has data issue: false hasContentIssue false
  • English
  • Français

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: [email protected]
Get access Rights & Permissions [Opens in a new window]

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

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

1. Van Praagh, S, Vangi, V, Sul, JH, et al. . Tricuspid atresia or severe stenosis with partial common atrioventricular canal: anatomic data, clinical profile and surgical considerations. JACC 1991; 17: 932–943.CrossRefGoogle ScholarPubMed
2. Webb, S, Brown, NA, Anderson, RH. Cardiac morphology at lat fetal stages in the mouse with trisomy 16: consequences for different formation of the atrioventricular junction when compared to humans with trisomy 21. Cardiovasc Res 2007; 34: 515–524.CrossRefGoogle Scholar
3. Anderson, RH, Wilkinson, JL, Gerlis, LM, Smith, A, Becker, AE. Atresia of the right atrioventricular orifice. Br Heart J 1977; 39: 414–428.CrossRefGoogle ScholarPubMed
4. Williams, HJ, Tandon, R, Edwards, JE. Persistent ostium primum coexisting with mitral or tricuspid atresia. Chest 1974; 66: 39–43.CrossRefGoogle ScholarPubMed