摘要
目的：回顾性总结采用双室性矫治法洛四联症合并完全性房室间隔缺损（TOF/CAVSD）的外科手术经验及其效果分析.方法：自2004年1月至2011年12月期间,上海儿童医学中心连续对16例TOF/CAVSD,其中伴有唐氏综合征2例,手术年龄中位数9个月（4个月～12岁）,手术时体质量中位数8 kg（4.7～35 kg）,术前有56％患者伴有中度以上左侧房室瓣反流,31％患者伴有中度以上右侧房室瓣反流;术前平均肺动脉指数为（168±23） mm2/m2.合并的CAVSD矫治中均采用了双片法,其中有8例（50％）采用右心室流出道（RVOT）切口径路修补室间隔缺损,另外50％患者采用右心房切口径路修补室间隔缺损,仅有1例室间隔缺损补片上留孔,右心室流出道梗阻（RVOTO）处理中,13例患者（81％）施用心包补片跨瓣扩大成形右心室流出道和肺动脉,其中1例带有单瓣.结果：术后医院生存率为93.75％.术后平均呼吸机辅助时间为（166 ±22.5）h,平均监护室留驻时间中位数为7d.4例患者出现术后严重并发症,其中发生低心排出量综合征（低心排）者3例,多脏器功能损害者1例.出院时12.5％（2例）患者术后二尖瓣反流（轻-中度）,12.5％患者术后轻度三尖瓣反流（TR）,无1例存在左心室流出道梗阻（LVOTO）.随访期6个月～5年,随访期无死亡,无1例出现LVOTO,无再手术干预;3例肺动脉远端流速增快者已登记介入治疗;随访发现生存60％患者二尖瓣反流为轻微-轻度;同时发现有1/3生存者三尖瓣反流为轻微-轻度;随访期存在中度肺动脉血反流患者为86％.1/3生存患者的右心室舒张末容积＞相应正常年龄正常值.结论：双心室矫治法TOF/CAVSD的短中期效果好,推荐采用双片法矫治CAVSD;与单纯TOF或CAVSD手术一样,必须定期随访复查,需关注房室瓣功能、肺动脉发育、肺动脉逆向血流和心室功能.
Objective：To review our experience with Bi-ventricular repair of tetralogy of Fallot associated with complete atrioventricular septal defect （TOF/CAVSD）,using a two-patch technique in closure of septal defect.Methods：Between 2004 and 2011,18 children with TOF/CAVSD were admitted; 2 had Down＇s syndrome （11％）.Bi-ventricular repair was performed in all patients at a median age of 9 months （range,4 month to 12 years）.The two-patch technique for atrioventricular septal defect was used.The ventricular septal defect was closed through a right atriotomy in 50％ cases; the other by a right ventriculotomy.The commissure between the superior and inferior bridging leaflets of the left portion of the common atrioventricular valve was closed in each patient.RVOTO was relieved by a transannular autologous pericardium patch in fifteen patients （83％）,including with monocusp valve in one case,and by infundibular patch with preservation of the pulmonary valve in 3 （17％）.Results：Overall in-hospital survival was 95％.Among patients with CAVSD-TOF,one died in hospital （5％） ; causes of death were heart block,progressive heart failure and multiple organ failure.Median follow-up was 3.7 years （range 6 months-5 years）.There was no late mortality and 4 patients had mild-moderate left atrioventricular valve regurgitation,2 patients with moderate TR.Marked PI with higher RVEDV was in 25％ survival.RVOT was 83％ of freedom from reinterventions.Conclusion：Bi-ventricular repair with two-patch technique closuring septal defect in patients with TOF/CAVSD seem to offer favorable intermediate survival.The RVOTO should be relieved in the same fashion as for isolated TOF.Long-term follow-up is necessary for AV valve dysfunction and pulmonary insufficiency.
出处
《心肺血管病杂志》
CAS
2014年第2期159-162,共4页
Journal of Cardiovascular and Pulmonary Diseases
关键词
法洛四联症
完全性房室间隔缺损
双片法
双心室修补术
Tetralogy of Fallot
Complete atrioventricular septal defect
Two-patch technique
Bi-ventricular repair
