Down综合征对先天性心脏病外科治疗的影响

Impact of Down syndrome on the surgical treatment of congenital heart defects

目的：评估Down综合征对先天性心脏病手术治疗的影响。方法：回顾性分析于2004年至2009年行心脏手术治疗的35例合并先天性心脏病的Down综合征患儿的临床资料，总结此类患儿的手术死亡率、并发症和随访结果。结果：所有患儿均接受一期手术修复。术后早期死亡1例（2．9％）；低心排出量综合征2例（5．7％）；肺部并发症15例（42．9％）。术后早期Ⅲ°房室传导阻滞4例（11．4％）。对30例随访10个月-6年[（3．8±1．1）年]，无远期死亡和再次手术病例，存活者心功能为NYHAI或Ⅱ级。结论：Down综合征增加了先天．陛心脏病手术的早期风险陛，但不增加死亡率，术后中期生存状态良好。

Objective： To evaluate the impact of Down syndrome （DS） on surgical management in patients with congenital heart defects （CHD）. Methods： We retrospectively analyzed the clinical data from 35 children with DS and CHD, who underwent cardiac surgery between 2004 and 2009. The data on surgical mortally, complications and follow-up results are emphasized. Results： All of the patients underwent primary repair. One child （2.9%） with DS and complete atrioventricular septal defect （CAVSD） died early postoperatively because of pulmonary hypertension. Two patients （S.7%） had low cardiac output syndrome, and 1S （42.9%） suffered pulmonary complications.atrioventricular block （AVB） occurred in 4 patients （11.5%）. Thirty children who were followed up 10 months to 6 years [（3.8±1.1） years] are in NYH class I or Ⅱ There were no reoperations or later death. Conclusion： CHD in DS children can be repaired with a low risk of mortalit） although a high incidence of severe infections and Ⅲ° AVB can result in a complicated postoperative course. The results of mid-term follow up are satisfactory.