The management of pulmonary atresia with a ventricular septal defect (PA/VSD) depends on the anatomy of the pulmonary artery or on the surgical strategy used at individual institutions. In our institution, we adopted a right ventricle-to-pulmonary artery (RV-PA) shunt in 2011 as a palliative procedure for PA/VSD to overcome the disadvantages of a Blalock-Taussig shunt. We evaluated the results of the RV-PA shunt as initial palliative surgery for PA/VSD. Thirteen patients with ductus-dependent PA/VSD from August 2011 to August 2015 were enrolled. The mean age at surgery was 17.9 ± 15.3 (range 5–60) days, and the mean body weight was 2.9 ± 0.6 (range 2.2–4.0) kg. A Gore-Tex tube graft was used in all patients. We retrospectively observed intra- and postoperative complications, early and late mortality, and palliation duration to definitive repair. Left pulmonary artery angioplasty was performed as a concomitant procedure in three patients. There were no early hospital mortalities, although two inter-stage deaths occurred 34 and 47 days postoperatively: one patient died of aspiration and the other of right ventricular outflow tract (RVOT) pseudoaneurysm rupture. Two patients (15.4%) required the extracorporeal membrane oxygenation support postoperatively: one because of failure to wean from a bypass caused by persistent hypoxemia and the other because of sudden massive bleeding from the RVOT suture line in the intensive care unit 2 days postoperatively. These two patients underwent second-stage definitive repair successfully. Five patients (41.7%) required catheter intervention, for juxtaductal left pulmonary artery stenosis in three patients, right pulmonary artery stenosis in one, and shunt inflow stenosis in one. Two patients (15.4%) required re-operation because of shunt inflow stenosis and RVOT pseudoaneurysm, respectively. All patients who survived the RV-PA shunt underwent total correction at a mean interval of 13.1 months. A RV-PA shunt is an option for the initial palliation of ductus-dependent PA/VSD. Major complications can occur, including RVOT pseudoaneurysm, shunt inflow stenosis, persistent hypoxemia during the immediate postoperative period, and dehiscence of the shunt anastomosis site. Caution should be taken when performing the RV-PA shunt for palliation of PA/VSD.