Natural History of Pulmonary Atresia With Intact Ventricular Septum and Right-Ventricle-Dependent Coronary Circulation Managed by the Single-Ventricle Approach

In patients with PA/IVS-RVDCC, early mortality appears related to coronary ischemia at the time of BTS. Single-ventricle palliation yields excellent long-term survival and should be the preferred management strategy for these patients. Those with aortocoronary atresia have a particularly poor prognosis and should undergo cardiac transplantation.