修復を断念されていた肺動脈閉鎖，心室中隔欠損，主要体肺動脈側副血行路に対する心内修復

Pulmonary atresia, ventricular septal defect, and major aorto-pulmonary collateral arteries are deemed unrepairable in some patients. Reportedly, 39％ to 63％ of patients treated by the staged unifocalization pathway, and 25％ to 51％ of patients treated by the pulmonary artery rehabilitation strategy were deemed unsuitable to undergo total repair. Single-stage complete unifocalization with intracardiac repair can be performed in some older patients with pulmonary atresia, ventricular septal defect, and major aorto-pulmonary collateral arteries that were previously deemed unrepairable. We report four such patients with ages between 12 years and 28 years at surgery. Bosentan hydrate was administered pre-operatively in one female patient for 14 months to ameliorate regional pulmonary hypertension. Seventeen months after repair, she delivered a baby by cesarean section. One patient underwent aortic valve replacement concomitant with repair due to severe aortic regurgitation caused by healed infective endocarditis. All patients were discharged home 9 to 18 days after surgery. After 72 to 100 months of follow-up, three patients are in New York Heart Association （NYHA） class Ⅰ, and the remaining patient are in NYHA class Ⅱ. Single-stage complete unifocalization can allow total repair in adolescents and adults with pulmonary atresia, ventricular septal defect, and major aorto-pulmonary collateral arteries that were previously deemed unrepairable.