Despite low mortality and excellent long-term results after repair of Tetralogy of Fallot, certain associated anomaly such as single pulmonary artery continues to be told to have a high operative mortality and morbidity, and there is still some debate on appropriate surgical intervention. During the 4 year period from 1981 to 1984, surgical repair was performed on 5 patients with tetralogy of Fallot and congenital [4 cases] or acquired [1 case] absence of left pulmonary artery. Previous left pneumonectomy had been performed in the patient with acquired absence of a pulmonary artery. Transannular patch or RVOT patch alone with or without pulmonic valvotomy was used with some modifications to reduce pulmonic insufficiency in individual patient. In contrast with previous reports, all patients survived operation and have exhibited marked symptomatic improvement without pulmonic valve insertion nor valved conduit.