The anatomy of a persistent left superior vena cava (SVC) to the left atrium (LA) without the innominate vein can make it challenging to complete intracardiac repair. We reviewed our five cases of the direct end-to-side anastomosis of SVCs to facilitate anatomical repair of SVC-right atrial connection for biventricular repair. Diagnoses were two partial atrioventricular septal defect with left isomerism, one complete atrioventricular septal defect (CAVSD) with left isomerism, one CAVSD without isomerism and one atrioventricular discordance and double outlet right ventricle with right isomerism. Mean age at the operation was 20+/-23 months (4-58 months) and body weight was 7.8+/-3.4 kg (4.8-12.7 kg). After completion of intracardiac repair, the SVC to LA was divided and end-to-side anastomosed to the SVC to the right atrium during cardiopulmonary bypass. No early or late death occurred during follow-up of 14.4+/-6.9 months (7-23 months). None of the patients developed an obstruction at the anastomosis site of the SVCs. The direct end-to-side anastomosis of SVCs achieved an excellent anatomical SVC-right atrium connection in complex congenital heart diseases.