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The Annals of Thoracic Surgery, Vol 51, 65-72, Copyright © 1991 by The Society of Thoracic Surgeons
KS Iyer and RB Mee
Fifty-eight consecutive patients with pulmonary atresia, ventricular septal
defect, hypoplastic pulmonary arteries with arborization defects, and major
aortopulmonary collaterals were entered into a program for staged surgical
repair between January 1979 and July 1989. Prerepair preparatory procedures
were designed to (1) encourage native pulmonary artery growth by increasing
blood flow and (2) unifocalize pulmonary blood supply by transplanting or
ligating major collaterals. A total of 121 staging procedures were
performed with an overall mortality of 10.3%. One hundred thirty-four major
collaterals were either ligated or transplanted. Thirty patients eventually
underwent hemodynamic repair with an early mortality of 3.3% and late
mortality of 10.0%. Twenty-six current survivors of repair remain
clinically well after a mean follow-up of 3.6 years. Ten patients are
currently in various stages of preparation. Twelve patients (20.7%) failed
to achieve minimum requirements for repair after staging and await further
palliation or heart-lung transplantation. The principles of management have
evolved over the years and are discussed.
ARTICLES
Staged repair of pulmonary atresia with ventricular septal defect and major systemic to pulmonary artery collaterals
Victorian Pediatric Cardiac Surgical Unit, Royal Children's Hospital, Parkville, Australia.
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