右位心合并单心室的外科治疗  

The surgical treatment of dextrocardia with univentricular heart

作　　者：张小宁[1] 陈道中[1] 孙旭东[1] 陈良万[1] 邱罕凡[1] 黄忠耀[1] 吴锡阶[1] 

机构地区：[1]福建医科大学附属协和医院心外科,福建福州350001

出　　处：《罕少疾病杂志》2005年第5期10-12,F0002,共4页Journal of Rare and Uncommon Diseases

摘　　要：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。Objective To explore the surgical treatment of dextrocardia with univentricular heart. Methods Two cases were retrospectively analyzed. Results Case 1,5-year-old, was preoperative diagnosed as clextroversion 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 clextrocardia with univentricular heart combined with pulmonary artery stenosis, kartageners syndrome suspected. Case 1 was performed with bilateral bi-direction glenn opration while case 2 with total cavopulmonary connection.The operation for case 1 was successful. The child was discharged 12 days postoperative, with a 4 months following up uneventful. Unfortunately, case 2 was combined with atelectasis in left upper lung and chylopleura postoperation.ancl discharged at 49th clay after operation. Symptoms were aggregated 3 months later, with severe chest chyle, cyanosis, gasp, flatulent and legs edema. Case two inpatient again and die after 16 days. Conclusion Dextrocardia with univentricular heart is a rare congenital cardiac disease with cyanosis, with which Fontan or modified Fontan operation is alternative. However, clextrocardia with Kartagener＇s syndrome should be carefully accessed preoperation.

关 键 词：右位心 单心室 外科治疗 

分 类 号：R654.2[医药卫生—外科学]