Long-Term Results for Repair of Pulmonary Atresia with Intact Ventricular Septum

Lee, Cheul; Lee, Chang Ha; Hwang, Seong Wook; Lim, Hong Gook; Kim, Woong Han; Kim, Chong Whan

Abstract

BACKGROUND: Pulmonary atresia with intact ventricular septum is morphologically heterogeneous, and the surgical outcome remains suboptimal compared to other complex congenital heart defects. We evaluated the long-term results for repair of pulmonary atresia with intact ventricular septum. MATERIAL NAD METHOD: Between January 1992 and June 2004, 38 patients underwent repair of pulmonary atresia with intact ventricular septum. The average age was 18 days (2~382 days). The average Z-value of the tricuspid annulus diameter was -3.1 (-5.6~0.8). Thirteen (36%) patients had right ventricle-to-coronary artery fistulas, and 4 (11%) patients had right ventricle-dependent coronary circulation. Average follow-up was 55 months (3 months~12.2 years). RESULT: Twenty-four patients underwent initial right ventricle (RV) decompression and 14 patients underwent systemic-to-pulmonary arterial shunt only. The average size of the tricuspid annulus of the patients who underwent RV decompression was significantly larger than that of the patients who underwent systemic-to-pulmonary arterial shunt only (Z-value -2.2 vs. -4.8, p= 0.000). There were 5 (13%) early and 1 late deaths. Early deaths occurred in 3 patients who had undergone RV decompression, and in 2 patients who had undergone systemic-to-pulmonary arterial shunt only (p=1.0). Biventricular repair was achieved in 12 (32%) patients, single ventricular repair in 8 (21%), and one and a half ventricular repair in 4 (11%) patients. Nine (24%) patients are waiting for the definitive repair. Kaplan-Meier survival at 5 and 8 years was 83.2%, respectively. CONCLUSION: Most of the deaths occurred after the initial palliation. Overall long- term survival was satisfactory. Early mortality should be reduced with careful preoperative evaluation and proper surgical strategy.