Oshima Y, Yamaguchi M, Yoshimura N, Oka S, Ootaki Y
Department of Cardiothoracic Surgery, Kobe Children's Hospital, Japan.
Ann Thorac Surg. 2001 Aug;72(2):424-9. doi: 10.1016/s0003-4975(01)02674-1.
Although satisfactory results of corrective surgery for atrioventricular septal defects (AVSDs) with complex lesions such as double-outlet right ventricle (DORV) or atrial isomerism have been reported in recent years, the optimal surgical options for isomerism hearts is still a controversial issue.
We performed anatomically corrective repair on 13 children with balanced forms of complete AVSDs and associated major cardiac anomalies. Eight of the 13 patients had atrial isomerism (right in 1, left in 7), 5 of whom had DORV. Four others had DORV with trisomy 21, and 1 had tetralogy of Fallot. Atrial septation for isomerism with the placement of an additional prosthesis was performed on 4 patients.
Two hospital deaths and one late death occurred only in the isomerism group. Three reoperations were required solely in the left isomerism group: one replacement of the valved external conduit concomitantly with reconstruction of the left ventricular outflow obstruction, one mitral valve replacement for severe regurgitation of left atrioventricular valve, and one relief of progressing left ventricular outflow obstruction.
The results of anatomically corrective surgery for AVSDs with major associated cardiac anomalies in the nonisomerism group were excellent. The optimal surgical options for isomerism heart, however, remain a controversial issue.
尽管近年来已有报道称，对于合并诸如右心室双出口（DORV）或心房异构等复杂病变的房室间隔缺损（AVSD）进行矫正手术可取得满意效果，但异构心脏的最佳手术选择仍是一个存在争议的问题。
我们对13例患有平衡型完全性AVSD及相关主要心脏异常的儿童进行了解剖矫正修复。13例患者中有8例存在心房异构（右异构1例，左异构7例），其中5例合并DORV。另外4例合并21三体综合征的DORV患者，1例患有法洛四联症。4例患者进行了伴有额外假体植入的异构心房分隔术。
仅在异构组发生了两例院内死亡和一例晚期死亡。仅在左异构组需要进行三次再次手术：一次在重建左心室流出道梗阻的同时更换带瓣外管道，一次因左房室瓣严重反流进行二尖瓣置换，一次缓解进行性左心室流出道梗阻。
非异构组中伴有主要相关心脏异常的AVSD进行解剖矫正手术的效果极佳。然而，异构心脏的最佳手术选择仍是一个存在争议的问题。
