成人期Taussig-Bing奇形に対するDamus-Kaye-Stansel（DKS）手術の1例

Revisiting Damus-Kaye-Stansel Procedure for Taussig-Bing Anomaly in Adults；Report of a Case

はじめに　Taussig-Bing奇形（TBA）は両大血管右室起始症（DORV）の一形態であり，1949年にはじめて提唱されたまれな先天性心疾患の一つである1）．近年では，小児期TBAの心内修復術ではJatene手術がもっとも施行される術式であるが，成人期のTBAにおける手術戦略に関してはいまだ報告例が少ない．われわれは成人期TBAに対してDamus-Kaye-Stansel（DKS）手術を施行し心内修復した例を経験し，良好な結果を得たので報告する．

We successfully performed the DKS＋Rastelli procedure for Taussig-Bing anomaly （TBA） in an adult. A 22-year-old male was admitted to our hospital due to persistent lower leg edema and dyspnea. He had been diagnosed with TBA. On physical examination, his peripheral oxygen saturation （Spo2） was 73％ in room air. Chest X-ray showed congestive heart failure. We decided to perform arterial switch operation （ASO） and VSD closure. Operation was performed via a re-median sternotomy. It seemed to be very difficult to dissect and mobilize great arteries and coronary arteries for ASO. Therefore, we decided to perform DKS, aortic valve closure, left ventricule to pulmonary artery rerouting, and right ventricular outflow reconstruction using expanded polytetrafluoroethylene （ePTFE） valved conduit. Postoperative course was uneventful, an oxygen saturation was improved. At 5 years after surgery, his oxygen saturation is about 97％ in room air and he is now doing well without any symptoms. Long-term follow-up is necessary to ensure maintenance of optimal function.