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J Thorac Cardiovasc Surg 2003;126:694-702
© 2003 The American Association for Thoracic Surgery

Surgery for congenital heart disease

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

^a Department of Pediatric and Congenital Heart Surgery , Cleveland, Ohio, USA
^b Department of Pediatric Cardiology, the Division of Pediatrics, The Children’s Hospital at The Cleveland Clinic, Cleveland, Ohio, USA

Received for publication December 2, 2002; revisions received March 10, 2003; revisions received March 25, 2003; accepted for publication April 11, 2003.

^* Address for reprints: Brian W. Duncan, MD, Pediatric and Congenital Heart Surgery, Cleveland Clinic Foundation M/41, 9500 Euclid Avenue, Cleveland, OH 44195, USA
duncanb{at}ccf.org

OBJECTIVE: 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.

METHODS: We retrospectively reviewed a consecutive series of these patients from a single institution.

RESULTS: 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.

CONCLUSIONS: 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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