Pacifico A D, Ricchi A, Bargeron L M, Colvin E C, Kirklin J W, Kirklin J K
Division of Cardiothoracic Surgery, University of Alabama, Birmingham 35294.
Ann Thorac Surg. 1988 Dec;46(6):645-51. doi: 10.1016/s0003-4975(10)64726-1.
Twenty-nine patients with balanced forms of complete atrioventricular (AV) canal defects and tetralogy of Fallot in 12, double-outlet right ventricle (DORV) in 10, and additional anomalies associated with atrial isomerism in 7 underwent corrective repair. Age ranged from 39 days to 24 years (median, 3.7 years). Repair included reconstruction of the common AV valve in 25 patients and prosthetic valve replacement in 4. Construction of an intraventricular tunnel connecting the left ventricle with the aorta was accomplished in 23 of 25 patients with ventriculoarterial concordant or double-outlet connections. In 2 patients with situs inversus and associated DORV and in 4 patients in the isomerism group who had ventriculoarterial discordant connections, repair included intraatrial venous diversion by a modified Senning procedure in 2, a modified Mustard venous switch in 1, and a tailored atrial baffle in 3. A valved extracardiac conduit was required for repair in 4 patients. Partial anomalous systemic venous connection was repaired in 7 patients and total anomalous pulmonary venous connection, in 5. There was 1 hospital death (3%) and 3 late deaths (11%). There was 1 (3.6%) reoperation to replace an incompetent right (systemic) AV valve 17 months postoperatively, and each of the 25 surviving patients was in New York Heart Association Functional Class I (80%) or II (20%) at follow-up.
29例患有完全性房室通道缺损平衡型的患者接受了矫正修复，其中12例合并法洛四联症，10例合并右心室双出口，7例合并与心房异构相关的其他异常。年龄范围为39天至24岁（中位数为3.7岁）。修复包括25例患者重建共同房室瓣，4例患者进行人工瓣膜置换。25例心室动脉协调或双出口连接的患者中，23例完成了连接左心室与主动脉的室内隧道构建。2例镜像右位心合并右心室双出口的患者以及异构组中4例心室动脉不协调连接的患者，修复包括2例采用改良森宁手术进行心房内静脉转流，1例采用改良马斯塔德静脉转换术，3例采用定制心房挡板。4例患者修复时需要使用带瓣心外管道。7例患者修复了部分体静脉异常连接，5例患者修复了完全性肺静脉异位连接。有1例医院死亡（3%）和3例晚期死亡（11%）。术后17个月有1例（3.6%）再次手术更换功能不全的右（体）房室瓣，25例存活患者在随访时纽约心脏协会心功能分级均为Ⅰ级（80%）或Ⅱ级（20%）。
