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A 10-year-old girl was referred to us with a diagnosis of pulmonary atresia and intact ventricular septum with a severely hypoplastic right ventricle and atrial septal defect. Her tricuspid valve diameter (TVD) was 48.5% of normal, with a TVD Z-score of -6.7, while right ventricular end-diastolic volume was 53.4% of normal. As a definitive procedure, one and one half ventricular repair using a bidirectional cavopulmonary shunt (BCPS) was performed instead of a Fontan procedure. The postoperative course was uneventful without episodes of low cardiac output or elevated right atrial pressure. She has been doing well during a follow-up period of 3 years.

Mid-term Result of One and One Half Ventricular Repair in a Patient with Pulmonary Atresia and Intact Ventricular Septum.