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Eur J Cardiothorac Surg 2007;32:202-208. doi:10.1016/j.ejcts.2007.04.022
Copyright © 2007, European Association for Cardio-Thoracic Surgery. Published by Elsevier B.V. All rights reserved

Clinical results of staged repair with complete unifocalization for pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries

Department of Cardiovascular Surgery, Heart Institute of Japan, Tokyo Women's Medical University, Tokyo, Japan

Received 5 September 2006; received in revised form 10 April 2007; accepted 12 April 2007.

^_* Corresponding author. Address: Department of Cardiovascular Surgery, Children's National Medical Center, 111 Michigan Avenue, NW, Washington, DC 20010-2970, United States. Tel.: + 1 202 884 2593; fax: +1 202 884 5572. (Email: nishibas{at}cnmc.org).

Objective: Our treatment strategy for pulmonary atresia with ventricular septal defect (VSD) and major aortopulmonary collateral arteries is a staged repair that comprises the first complete unifocalization (UF) with ‘unification’ of intrapulmonary arteries and then the definitive repair. The purpose of this study is to evaluate the outcome of our staged repair strategy with complete UF and to determine the results of our current management strategy. Methods: From 1982 to 2004, 113 consecutive patients were treated with staged repair at our institute. We evaluated the risk of definitive repair failure or death in the 3 years after definitive repair using logistic regression. Furthermore, we compared the early group (patients who underwent UF before December 1995) and the late group (patients who underwent UF after January 1996). Results: The mean follow-up interval was 8.8 years (0.8 months to 23.3 years), and Kaplan–Meier-estimated overall survival rates after first UF were 80.9, 73.8, and 69.9% at 5, 10, and 15 years, respectively. Survival in patients with an absent central pulmonary artery (PA) was significantly lower than in those with a central PA (p < 0.05), and the factor that was significantly associated with definitive repair failure or death in the 3 years after definitive repair was central PA morphology (p < 0.05). Higher mean PA pressure after UF was detected in patients with hypoplastic central PA, compared with those without hypoplastic PA (30.9 mmHg vs 23.3 mmHg, p < 0.05). In the late group, age (in years) at first UF (3.9 vs 8.4, p < 0.01), second UF (4.3 vs 9.2, p < 0.01), and definitive repair (5.8 vs 9.1, p < 0.01) was significantly younger than in early group, and the survival rate after first UF in the late group was 96.2 and 91.3% at 3 and 7 years, respectively. Systolic right ventricular pressure and the pressure ratio between the right and the left ventricles after definitive repair in the late group were significantly lower than in the early group (53.6 mmHg vs 75.0 mmHg, p < 0.01; 61.7% vs 75.9%, p < 0.05). Conclusions: Hypoplastic central PA was a significant risk factor in this disease. The overall survival was improved by our current management strategy. Improved RV pressure after definitive repair appears to affect the long-term outcome.

Key Words: Major aortopulmonary collateral arteries • Unifocalization • Staged repair • Surgery