Pulmonary Atresia with Intact Ventricular Septum, an Evolving Strategy in the Era of PDA Stenting: Single Center Experience

Abstract

Repair or palliation of pulmonary atresia with intact ventricular septum (PA/IVS) often falls into one of 4 categories: cardiac transplant, 2-ventricular circulation, 1.5 ventricle circulation, or single ventricle circulation. The optimal management strategy has been an area of much debate. We sought to review the management strategy of patients with PA/IVS at our institution to better understand what metrics can be used to guide management and initial interventions. The study aims to examine the outcomes of a single-center approach to managing patients with PA/IVS. Our cohort included 29 patients; one patient underwent a planned transplant at ten days of life (3.4%), 12 underwent repair via a two-ventricle circulation (41.4%), 7 underwent repair with 1.5 ventricle circulation (24.1%), and 7 underwent repair with single ventricle circulation (24.1%). Survival was achieved in 93.1% with two patients (6.9%) expiring. The TV annulus z-score was significantly different between the three groups, with the 1 V group having the smallest median TV annulus z-score at − 4.04 (IQR − 4.60– − 3.60) and the 2 V group having the largest median TV z-score at − 1.4 (IQR − 2.24– − 0.12). Six patients underwent late right ventricular decompression. We present a post-hoc algorithm to help guide treatment strategies for patients with PA/IVS.