Department of Cardiovascular Surgery, Kyushu University Hospital, 3-1-1, Maidashi, Higashi-ku, Fukuoka, 812-8582, Japan.
Department of Pediatrics, Kyushu University Hospital, Fukuoka, Japan.
Gen Thorac Cardiovasc Surg. 2021 Dec;69(12):1585-1588. doi: 10.1007/s11748-021-01702-5. Epub 2021 Sep 14.
Tricuspid atresia with pulmonary valve absence is a rare malformation characterized by left ventricular outflow obstruction due to asymmetrical ventricular septal hypertrophy and associated with a poor prognosis. Coexisting coronary-right ventricular fistula is rarely described. We encountered a case of tricuspid atresia with pulmonary valve absence coexisting coronary-right ventricle fistula that successfully underwent Fontan completion. Right ventricle dilatation due to coronary-right ventricle fistula was observed in addition to mass like ventricular septal hypertrophy protruding into the left ventricular outflow tract. Right ventricle reduction and prevention of progressive left ventricular outflow obstruction were achieved by closure of the coronary-right ventricle fistula closure and plication of the right ventricle with the bidirectional Glenn procedure.
三尖瓣闭锁伴肺动脉瓣缺如为一种罕见的畸形，由于不对称性室间隔肥厚导致左心室流出道阻塞，伴有预后不良。同时合并冠状动脉-右心室瘘管则更为罕见。我们遇到一例三尖瓣闭锁伴肺动脉瓣缺如合并冠状动脉-右心室瘘管，该病例成功地进行了 Fontan 完成手术。除了块状室间隔肥厚突入左心室流出道外，还观察到由于冠状动脉-右心室瘘管导致的右心室扩张。通过关闭冠状动脉-右心室瘘管并使用双向 Glenn 手术对右心室进行折叠，实现了右心室缩小和防止左心室流出道进行性梗阻。
