马方综合征的外科治疗

目的总结马方综合征(MFS)的外科治疗经验。方法对28例MFS患者的临床资料进行回顾性分析。结果3例术前因主动脉破裂死亡;术后死亡2例,1例死于心室颤动,1例死于败血症。余术后24h胸腔引流量300～1500ml,呼吸机使用时间10～52h。出院前复查超声心动图显示左室舒张末期内径均明显缩小,主动脉瓣启闭良好;术后病理示主动脉壁退行性变及囊性坏死。结论心血管病变是MFS最危急的病症和主要的死亡原因。死亡原因主要为升主动脉夹层分离引起的心包填塞、主动脉瓣反流引起的左心功能不全、冠状动脉开口破裂引起的心肌缺血以及因主动脉弓受累导致的中风。升主动脉瘤直径>5.5cm者应行手术治疗,Bentall手术为首选的手术方式。

Surgical treatment of Marfan syndrome

Objective To summarize the experience of surgical treatment of Marfan syndrome.Methods Clinical data of 28 cases with Marfan syndrome were retrospectively analyzed.Results Three patients died of aortic rupture before operation,two died of ventricular fibrillation and septicemia after operation,respectively.24 h later after operation the drainage volume reached 300 1500ml and respirator was used for 10 52h.The diastolic interior diameter of left ventricular was obviously decreased before discharge from hospital and aortic valve recovered well.Postoperative pathology showed cystic necrosis and retrograde degeneration.Conclusion The most emergent symptom and the main causes of death are the cardiovascular complications in Marfan syndrome.The main causes of death are tamponade,left ventricular failure,myocardial ischemia and stroke.Prophylactic surgery is recommended to decrease the death rate when the diameter of the sinus of valsalva exceeds 5.5cm in an adult.