Surgical Options for Pulmonary Atresia with Ventricular Septal Defect in Neonates and Young Infants.

A staged repair strategy, compared with primary repair, was associated with comparable overall survival with less frequent re-interventions after repair in young infants with PA/VSD. Lowering the inter-stage mortality after initial palliation by vigilant outpatient care and aggressive home monitoring may be the key to better surgical outcomes in this subset. Surgical outcomes of PA with VSD according to the surgical strategies. Patient 1 (birth weight: 2.7 kg) underwent primary Rastelli-type repair at post-natal day # 50 (body weight: 3.8 kg) using Contegra® 12 mm. The postoperative course was rocky, with long ventilatory support (10 days), ICU stay (14 days), and hospital stay (20 days). Cardiac CT scan at 9 months post-repair showed severe branch pulmonary artery stenosis, which necessitated LPA stenting at 12 months post-repair and RV-PA conduit replacement with extensive pulmonary artery reconstruction at 25 months post-repair. Patient 2 (birth weight: 2.5 kg) underwent RMBT at post-natal day #30 (body weight: 3.4 kg) using 4 mm PTFE vascular graft and staged Rastelli-type repair at post-natal 11 months using a hand-made Gore-Tex valved conduit (14 mm). No post-repair re-intervention has been performed. Cardiac CT scan at 90 months post-repair showed no branch pulmonary artery stenosis.CT computed tomography, ICU intensive care unit, LPA left pulmonary artery, PA pulmonary atresia, PTFE polytetrafluoroethylene, RMBT right modified Blalock-Taussig shunt, RV-PA right ventricle to pulmonary artery, VSD ventricular septal defect.