马方综合征并发A型主动脉夹层的手术疗效

Surgical Outcomes of Patients with Marfan Syndrome Complicated by Type A Aortic Dissection

目的观察并评价马方综合征(Marfan syndrome,MFS)并发A型主动脉夹层(type A aortic dissection,AAD)的手术疗效。方法回顾性分析2006年1月至2012年1月武汉亚洲心脏病医院收治44例MFS并发AAD患者的临床资料,其中男31例,女13例;年龄12～54(33.0±9.8)岁。23例在AAD发病后不同疾病阶段施行外科手术治疗,均行Bentall手术;21例因经济或其它原因未采取手术治疗。采用含时变协变量的COX回归分析,分别以主要终点事件、主要+次要终点事件作为结局变量对相关因素进行分析,观察手术患者术后手术疗效和生活质量,并与未手术患者的随访资料进行对比。结果术后有1例手术患者死于多器官功能衰竭,其余22例手术均成功。随访43例,随访时间1～75个月。22例手术患者术后3年生存率为95.7%,随访期间生活质量良好。未行手术治疗的21例患者生存状况及生活质量较差,13例(61.9%)死亡,主要死亡原因:急性心脏压塞、主动脉夹层破裂、急性心肌梗死并发心源性休克等;发病后3年生存率仅为31.7%。采用含时变协变量COX回归模型分析校正手术时间不一产生影响后的结果显示,手术治疗患者与未手术治疗患者相比较,其临床疗效的差异有统计学意义(T_COV_的OR值=0.088,P=0.028),手术治疗死亡风险仅为非手术治疗的8.8%(P<0.05)。结论 MFS并发AAD预后凶险,应尽早外科手术,不论是急性期还是慢性期均可获益,疗效可靠,随访结果令人满意。

Objective To evaluate surgical outcomes of patients with Marfan syndrome （MFS） complicated by type A aortic dissection （AAD） during follow-up. Methods We retrospectively reviewed clinical data of 44 patients with MFS complicated by AAD who were admitted to Wuhan Asia Heart Hospital from January 2006 to January 2012. There were 31 male patients and 13 female patients with their age of 12-54 （33.0±9.8） years. Twenty-three patients underwent Bentall procedure at different time after the onset of AAD, while the other 21 patients received conservative treatment in stead of surgery because of economical or other reasons. COX regression with time-varying covariates was performed to analyze related factors, using primary end point, primary end point ＋ secondary end point as the outcome variables respectively, to compare postoperative outcomes and quality of life between the surgical treatment patients and conservative treatment patients. Results Postoperatively 1 patient died of multiple organ failure, and the other 22 patients survived the surgery. All the 43 patients were followed up from 1 to 75 months. The 3-year survival rate of the 22 surgical treatment patients was 95.7%, and they all had a good quality of life during follow-up. The survival condition and quality of life of the 21 conservative treatment patients was poor, and 13 patients （61.9%） died with the 3-year survival rate of only 31.7%. The main causes of their death included acute cardiac tamponade, aortic dissection rupture, acute myocardial infarction and cardiogenic shock. COX regression with time-varying covariates showed that the treatment outcomes of the surgical treat- ment patients were statistically different from those of the conservative treatment patients after modifying the influence caused by different operating time （ OR ofT_ COV_ ：0.088, P=0.028 ）. The risk of death of surgical treatment was only 8.8% of that of conservative treatment. Conclusion The prognosis of patients with MFS complicated by AAD is very poor Therefore, all these patient, both in acute stage and chronic stage, should undergo surgical treatment as early as possible The short-term and follow-up outcomes of surgical treatment are satisfactory.