改良Fontan术治疗内脏异位综合征  

Modified Fontan Procedure for Patients with Heterotaxy Syndrome

作　　者：陈伟丹[1,2] 陈欣欣[2] 王武军[1] 

机构地区：[1]南方医科大学附属南方医院胸心血管外科,广州510515 [2]广州医科大学附属广州市妇女儿童医疗中心心脏中心,广州510623

出　　处：《中国胸心血管外科临床杂志》2016年第4期366-368,共3页Chinese Journal of Clinical Thoracic and Cardiovascular Surgery

摘　　要：目的总结改良Fontan术治疗内脏异位综合征的效果和经验。方法回顾性分析2008年9月至2014年11月在我院完成改良Fontan术的17例内脏异位综合征患者的临床资料。其中男11例、女6例,年龄3-10（4.7±2.5）岁,体重10.6-27.0（16.6±4.9）kg。17例患者中2例患者接受一期改良Fontan术,15例行分期手术,在行改良Fontan术前接受了双向Glenn等单心室矫治系列手术。结果无住院死亡,末梢血氧饱和度由73%±12%升高到91%±5%,无血管栓塞或严重心律失常等并发症出现。随访4个月至6.5年,死亡1例,近中期死亡率5.9%（1/17）。结论单心室矫治是治疗内脏异位合并心内复杂畸形的主要手术方式,术后早、中期效果良好。Objective To summarize the result and experience of modified Fontan procedure for patients with heterotaxy syndrome.Methods We retrospectively analyzed the clinical data of 17 patients with heterotaxy syndrome underwent cardiac operations in our hospital from September 2008 to November 2014.There were 11 males and 6 females at mean age of 4.7 ± 2.5 years ranging from 3 to 10 years and at mean weight of 16.6 ± 4.9 kilogram ranging from 10.6 to27.0 kilogram.Two patients accepted modified Fontan surgery of stage one.The rest 15 patients accepted staging operation.They accepted modified Fontan surgery after they accepted a series of surgery like bidirectional Glenn procedure.Results There was no mortality in hospital.The peripheral blood oxygen saturation raised from 73 %± 12 % to 91 %± 5 %.There was no complications existing like thrombosis or severe arrhythmia.The follow-up duration was from 4 months to 6.5 years.There was 1 death during the time.The early and middle term mortality was 5.9 %（1/17）.Conclusions Single ventricle treatment remains the preferred procedure for patients with heterotaxy syndrome and its early and middle term results are satisfied.

关 键 词：内脏异位 右房异构 左房异构 

分 类 号：R726.5[医药卫生—儿科]