儿童马方综合征的心血管并发症及其处理的临床分析

Clinical analysis of cardiovascular complications and management on children Marfan syndrome

目的:分析儿童马方综合征(MFS)患者心血管并发症及治疗的经验和教训。方法:对我院1998年至2008年收治的28例儿童MFS的临床资料,采用超声心动图,心电图和胸片检查,逐一进行回顾性分析。结果:28例儿童MFS患者中家族史阳性率为35.71%,主动脉窦部扩张的发生率为78.57%,主动脉瓣关闭不全的发生率为39.29%,二尖瓣关闭不全的发生率为71.42%。结论:儿童MFS患者以主动脉窦部扩张为主。存在二尖瓣病变时是否同期处理主动脉依据具体情况确定治疗原则,降低手术风险。

Objective： To analysis cardiovascular complications and management on children Marian syndrome. Method： The clinical data of 28 cases of children Marian syndrome at Anzhen hospital from 1998 to 2008 were analyzed retrospectively by means of echocardiography, ECG and chest X-ray. Result： The family history positive rate was 35.71%, incidence of aortic sinus dilatation was 78.57%, incidence of aortic valve insufficiency was 39.29% and incidence of mitral valve insufficiency was 71.42% in the 28 cases of patients. Conclusion： Children Marian syndrome based mainly aortic sinus dilatation. When they involved mitral valve disease, whether or not management of the aorta disease simultaneously according for actual situation determined principle of treatment, decreased surgical risk.