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

Total Repair of Pulmonary Atresia, Ventricular Septal Defect, and Major Aorto-pulmonary Collateral Arteries That were Deemed Unrepairable in an Adolescent and Adults

成人先天性心疾患手術には，先天的形態異常を有しても機能的障害が成人期まで出現しない症例の手術（大動脈二尖弁や左室拡張障害をきたした心房中隔欠損など）や，心内修復後の続発症に対する手術（Fallot四徴修復後の肺動脈弁逆流や先天性修正大血管転位の機能的修復後の三尖弁逆流など）がある．その他にも，姑息術後に心内修復を断念されていた例に対し，健康状態の改善を得るために判断を変更して修復手術を行うこともある．肺動脈閉鎖（pulmonary atresia：PA），心室中隔欠損（ventricular septal defect：VSD），主要体肺動脈側副血行路（major aorto-pulmonary collateral arteries：MAPCAs）の患者では，しばしば手術を受けずに，または姑息術の後に心内修復が不可能と判断され，そのまま成人になることがある．その中には，一期的肺動脈統合と同時に心内修復術を行うことにより健康状態の改善を得られる患者もいる．

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 preoperatively 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.