Down综合征对先天性心脏病外科治疗的影响

摘要

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 mortality, 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 (5.7%) had low cardiac output syndrome, and 15 (42.9%) suffered pulmonary complications. III° 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 NYHA class I or II. There were no reoperations or later death.Conclusion: CHD in DS children can be repaired with a low risk of mortality, 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.%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NYHA Ⅰ或Ⅱ级.结论:Down综合征增加了先天性心脏病手术的早期风险性,但不增加死亡率,术后中期生存状态良好.