论文部分内容阅读
目的 探讨分析马凡氏综合征的临床特征。方法 对４２例马凡氏综合征患者的临床资料进行回顾性分析。结果４２例患者中主要表现为气促（５４．８％）、胸痛（３１．０％）、主动脉瓣听诊区舒张期杂音（９１．０％）、主动脉扩张（９７．５％）、主动脉关闭不全（８５．０％）、主动脉夹层（２８．６％）、瘦长体型（５０．０％）、蜘蛛样指（趾）（８３．３％）、眼征（１９．０％）以及有家族史（１６．７％），也是本病诊断和鉴别诊断的主要依据。１３例施行了Ｂｅｎｔａｌｌ手术，术后１１例心衰症状明显改善。术后死亡二例。结论 马凡氏综合征的诊断主要依靠临床综合分析，心血管病变严重者外科治疗效果良好。
Objective To investigate the clinical features of Marfan syndrome. Methods The clinical data of 42 patients with Marfan syndrome were retrospectively analyzed. Results The main manifestations of 42 patients were shortness of breath (54.8%), chest pain (31.0%), aortic aortic diastolic murmur (91.0%), aortic dilatation (97.5%), Aortic insufficiency (85.0%), aortic dissection (28.6%), slender body (50.0%), spidery finger (83.3%), ocular sign (19.0% ) And family history (16.7%), but also the main basis for the diagnosis and differential diagnosis of the disease. Thirteen patients underwent Bentall surgery, 11 cases of heart failure symptoms improved significantly. Two cases died after operation. Conclusions The diagnosis of Marfan syndrome mainly depends on clinical comprehensive analysis. The surgical treatment of patients with severe cardiovascular disease is good.