Outcomes of aortopulmonary window repair in children: 33 years of experience.

BACKGROUND: The purpose of this study was to assess the outcomes of children undergoing repair of aortopulmonary window (APW).

METHODS: We conducted a retrospective review of all children (n=43) who underwent surgical repair of APW between 1980 and 2013.

RESULTS: Median age at surgery was 40 days (range, 13 to 125). Simple APW was present in 15 of 43 patients (35%), and 28 of 43 patients (65%) patients had concomitant cardiovascular anomalies. The aorta was repaired by direct suturing in 36 patients (84%) patients and patching in 7 patients (16%). The main pulmonary artery was repaired by direct suturing in 22 patients (51%) patients and by patching in 21 (49%). Cardiopulmonary bypass was used in 42 of the 43 patients (97.7%). Single-staged repair of concomitant cardiovascular anomalies was undertaken in 26 of 28 patients (93%). Only 2 of the 28 patients (7%) underwent repair of interrupted aortic arch before APW repair. Operative mortality was 6.7% (1 of 15 patients) among patients with simple APW and 18% (5 of 28 patients) among patients with concomitant anomalies. Operative weight less than 2.5 kg was associated with mortality on univariable analysis (p=0.02). Median follow-up was 10.1 years (range, 0.17 to 24.2). There were no late deaths. Overall survival was 86% (95% confidence interval: 71.3 to 94.2) at 10 years. Freedom from reoperation was 95.3% (95% confidence interval: 86.2 to 99.9) at 10 years. At last follow-up, all patients were in New York Heart Association functional class I/II.

CONCLUSIONS: Survival beyond discharge from the hospital is associated with excellent outcomes.