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Objective: To determine whether one and one half ventricular repair (1.5VR) is definitely superior to the Fontan procedure in patients having hypoplastic right ventricle (RV) in the setting of pulmonary atresia with intact ventricular septum (PA/IVS) or its relatives, in terms of results in the longer term. Method: Since1987, 1.5VR has been chosen in seven patients with PA/IVS and in six having PS with hypoplastic RV. On preoperative catheterization, right ventricular end-diastolic volume (RVEDV) was 47±23% of the anticipated normal value, and annular diameter of the tricuspid valve (TVD) 72±22% of normal (Z value being −2.4±2.1). Follow-up term was 3–15 (10±4) years. Results: All patients survived 1.5VR, but one patient died of arrhythmia 9 years later. Freedom from arrhythmia was 80 and 20% at 10 and 12 years, respectively. Two patients have undergone conversion to the Fontan circulation, but none …