右位心合并单心室的外科治疗

目的探讨右位心合并单心室的外科治疗.方法回顾性分析我院收治的2例右位心合并单心室患儿的临床资料.结果例1,5岁,为右旋右位心,大动脉转位,单心房,单心室,二尖瓣闭锁,肺动脉瓣狭窄,行双侧双向Glenn术;例2,6岁,为镜面右位心,大动脉转位,单心房,单心室,肺动脉狭窄,疑合并Kartagener综合征,行全腔静脉肺动脉连接术.例1手术成功,于术后12天出院,术后4个月随访患儿无特殊不适.例2术后并发左侧上肺不张、乳糜胸,于术后49天出院,3个月后,出现大量乳糜胸水、紫绀、气喘、腹胀、双下肢水肿,再次入院,于入院后16天死亡.结论右位心合并单心室是一种少见的紫绀型复杂先天性心脏病,Fontan类手术是可供选择的术式,但合并Kartagener综合征则应慎重选择术式.

The surgical treatment of dextrocardia with univentricular heart

Objective To explore the surgical treatment of dextrocardia with univentr icularheart. Methods Two cases were retrospectively analyzed. Results Case 1,5-year-old,was preoperative diagnosed as dextroversion with univentricular heart combined with great artery transposition, single atrium, mitral atresia, pulmonary valve stenosis.Case 2,6-year-old,was diagnosed as mirror image dextrocardia with univentricular heart combined withpulmonary artery stenosis, kartagener,s syndrome suspected. Case 1 was performed withbilateral bi-direction glenn opration while case 2 with total cavopulmonary connection.Theoperation for case 1 was successful. The child was discharged 12 days postoperative, with a4 months following up uneventful. Unfortunately, case 2 was combined with atelectasis inleft upper lung and chylopleura postoperation.and discharged at 49th day after operation.Symptoms were aggregated 3 months later, with severe chest chyle, cyanosis, gasp, flatulentand legs edema. Case two inpatient again and die after 16 days. Conclusion Dextrocardiawith univentricular heart is a rare congenital cardiac disease with cyanosis, with which Fontanor modified Fontan operation is alternative. However, dextrocardia with Kartagener,s syndromeshould be carefully accessed preoperation.