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Atrioventricular septal defects with severe left ventricular hypoplasia—clinical findings and surgical options

Published online by Cambridge University Press:  19 August 2008

Carlos J. Troconis ,
Roberto M. Di Donato ,
Bruno Marino ,
Ugo Vairo  and
Carlo Marcelletti
Carlos J. Troconis
Affiliation:
Departments of Pediatric Cardiology and Cardiac Surgery, Bambino Gesú Hospital, Rome
Roberto M. Di Donato*
Affiliation:
Departments of Pediatric Cardiology and Cardiac Surgery, Bambino Gesú Hospital, Rome
Bruno Marino
Affiliation:
Departments of Pediatric Cardiology and Cardiac Surgery, Bambino Gesú Hospital, Rome
Ugo Vairo
Affiliation:
Departments of Pediatric Cardiology and Cardiac Surgery, Bambino Gesú Hospital, Rome
Carlo Marcelletti
Affiliation:
Departments of Pediatric Cardiology and Cardiac Surgery, Bambino Gesú Hospital, Rome
*
Dr. Roberto Di Donato, Department of Cardiac Surgery, Bambino Gesu Hospital, Piazza Sant' Onofrio 4, 00165 Rome, Italy. Tel. 6–65191, Fax 6–65192296
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Summary

Right ventricular dominance was observed in 22 (6.6%) out of 332 patients with atrioventricular septal defects. Their mean age was 2.24 ± 2.73 months. Chromosomal anomalies were seen in only three patients (Down's syndrome in two and trisomy nine in one). Seventeen patients underwent surgical treatment. A pulmonary artery banding was performed in eight of them with one long-term survivor who underwent a successful staged orthoterminal repair. A Norwood-type of palliation was attempted in four without success. Biventricular repair was performed in four additional patients with two mid-term survivors, in one of whom the patch used for closure of the atrial septal defect was fenestrated. One patient underwent successful heart transplantation at seven months of age. Pathophysiological problems and surgical options are discussed.

Keywords

Atrioventricular septal defectright ventricular dominance
Type
Original Articles
Information
Cardiology in the Young , Volume 2 , Issue 1 , January 1992 , pp. 53 - 55
Copyright
Copyright © Cambridge University Press 1992

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