Outcome of Infants With Ductal-Dependent Pulmonary Atresia and Ventricular Septal Defect in a Developing Nation: A Single-Center Experience

Abstract

Objectives: The number of publications on the outcomes of treatment of ductal-dependent pulmonary atresia-ventricular septal defect in developing countries is limited. This study examines the results of surgical repair of this complex congenital heart disease at a high-volume center in Vietnam. Methods: This retrospective study included 144 patients with primary biventricular repair (group p-BVR, n = 70, 48.6%) or staged repair (group SR, n = 74, 51.4%) from April 2014 to March 2023. Concomitant augmentation of individual pulmonary arteries with autologous pericardial patches was performed in most patients who underwent the Rastelli operation (115/120 patients). Results: The overall rates of freedom from right ventricular outflow tract (RVOT) reintervention after the Rastelli operation at 1 year and 6 years were 94% (95% confidence interval [CI], 90%-99%) and 79% (95% CI, 69%-91%), respectively, with a median of 1.9 years (IQR, 0.9-5.0 years) from the Rastelli operation to the first reintervention. Fine and Gray's model for cumulative incidence across groups revealed that being in the p-BVR group was a high-risk factor for RVOT reintervention but not significant relative to being in the SR group (P = .12, SHR = 2.22, 95% CI, 0.82-6.03). The overall survival rate at 6 years was 89% (95% CI, 82%-97%) in the p-BVR group and 76% (95% CI, 66%-87%) in the SR group (P = .048). Conclusions: Concomitant pulmonary arterioplasty may contribute to or be associated with favorable reintervention rates and should be considered in patients with ductal-dependent pulmonary atresia with ventricular septal defect who undergo the primary Rastelli operation.