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The Journal of Thoracic and Cardiovascular Surgery, Vol 97, 746-754, Copyright © 1989 by The American Association for Thoracic Surgery and The Western Thoracic Surgical Association
AM Billingsley, H Laks, SW Boyce, B George, T Santulli and RG Williams
Between 1977 and 1988, 22 patients underwent definitive repair for
pulmonary atresia with intact ventricular septum. Fifteen underwent
biventricular repair (mean age 24 months). All had mild to moderate right
ventricular hypoplasia at the time of definitive repair. Repair consisted
of closure of the atrial septal defect with enlargement of the right
ventricular cavity and outflow tract with a patch in eight, insertion of a
valved homograft in three, and superior vena cava- pulmonary artery
connection in four. There was one operative death (7%). Seven patients had
severe right ventricular hypoplasia at the time of definitive repair and
underwent a Fontan procedure (mean age 46 months). In two a valved
connection was made to the right ventricle and in five a right
atrial-pulmonary artery nonvalved connection. There were two operative
deaths (29%). Three patients had right ventricular- coronary sinusoids: Two
of them underwent a Fontan procedure and one a biventricular repair. We
conclude that with adequate early palliation a biventricular repair may be
successfully performed for patients with mild or moderate right ventricular
hypoplasia, and the Fontan procedure may be used for those with severe
right ventricular hypoplasia.
ARTICLES
Definitive repair in patients with pulmonary atresia and intact ventricular septum
Department of Cardiothoracic Surgery, University of California, Los Angeles Medical Center 90024.
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