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Staged repair of tetralogy of Fallot with pulmonary atresia and major aortopulmonary collateral arteries
Journal article   Open access   Peer reviewed

Staged repair of tetralogy of Fallot with pulmonary atresia and major aortopulmonary collateral arteries

Brian W Duncan, Roger B.B Mee, Lourdes R Prieto, Geoffrey L Rosenthal, C.Igor Mesia, Athar Qureshi, Om P Tucker, John F Rhodes and Larry A Latson
The Journal of thoracic and cardiovascular surgery, v 126(3), pp 694-702
01 Sep 2003
DOI: https://doi.org/10.1016/S0022-5223(03)00700-1
PMID: 14502141
Featured in Collection :   UN Sustainable Development Goals @ Drexel
url
https://doi.org/10.1016/S0022-5223(03)00700-1View
Published, Version of Record (VoR) Open

Abstract

Spring
To assess the results of a staged surgical approach for tetralogy of Fallot with pulmonary atresia, hypoplastic or absent pulmonary arteries, and major aortopulmonary collateral arteries. We retrospectively reviewed a consecutive series of these patients from a single institution. From July 1993 to April 2001, 46 consecutive patients with tetralogy of Fallot, pulmonary atresia, and major aortopulmonary collateral arteries were treated with staged surgical repair. The operative sequence usually began with a central aortopulmonary shunt followed by unifocalization of aortopulmonary collateral arteries depending on the source and distribution of pulmonary blood flow. Twenty-eight patients (61%) subsequently underwent complete repair with ventricular septal defect closure and right ventricle to pulmonary artery connection. Those patients who underwent complete repair had a median of 3 total operations (range 1-6). The ratio of the mean pulmonary artery pressure to the mean systemic blood pressure at the time of complete repair was 0.36 (range 0.19-0.58). Two of the 28 repaired patients (7.1%) required subsequent fenestration of the ventricular septal defect closure due to later development of supersystemic right ventricular pressure and right ventricular failure. Eighteen patients (39%) have undergone 1 or more staging operations and are considered good candidates for eventual complete repair. There were no hospital deaths. There was 1 late death (2.2%; 95% CI 0.4-11.3%) in a patient born prematurely who developed severe bronchopulmonary dysplasia precluding complete repair. For tetralogy of Fallot with pulmonary atresia and major aortopulmonary collateral arteries, a staged surgical approach yields low overall mortality and acceptable hemodynamics after complete repair.

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Web of Science research areas
Cardiac & Cardiovascular Systems
Respiratory System
Surgery
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