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目的探讨中国肥厚型心肌病(hypertrophic cardiomyopathy,HCM)患者的长期预后及与HCM死亡相关的危险因素。方法回顾性分析1999年6月至2006年3月我院收治的234例HCM患者的病史资料,并对其中199例患者的随访结果采用SPSS 13．0统计学软件进行分析。结果234例患者中,85％获得随访,平均随访时间为(31．7±22．6)个月。随访中,21例患者死亡,其中19例患者被确认为HCM相关性死亡,包括猝死11例、心力衰竭死亡7例、脑卒中死亡1例;另2例患者分别因意外事故和急性胰腺炎死亡。患者确诊后1、2、3、4、5年生存率分别为96．7％、94．7％、94．7％、93．6％、89．0％。单因素分析显示男性、心功能分级、合并心房颤动、阵发性室性心动过速、左房增大、左室流出道梗阻、HCM家族史7个变量与HCM预后相关;多因素分析显示,阵发性室性心动过速(RR=2．234,P<0．001)、心功能III及以上(RR=1．964,P=0．003)、男性(RR=0．712,P=0．304)心房颤动(RR=0．654,P=0．259)、左房增大(RR=0．579, P=0．348)、左室流出道梗阻(RR=0．160,P=0．821)及HCM家族史(RR=0．663,P=0．275)均为HCM死亡相关危险因素．单因素分析亦显示,死亡患者的超声心动图表现以MaronIII型多见,仅1例患者表现为心尖肥厚型心肌病。对猝死者和心力衰竭死亡者进行分析显示,7例猝死者为60岁以下的患者,其中仅1例患者<35岁,5例心力衰竭死亡者为60岁以上的患者;仅2例猝死者和1例心力衰竭死亡者存在静息状态下左室流出道压差;7例猝死者和2例心力衰竭死亡者合并阵发性室性心动过速;猝死者和心力衰竭死亡者的室壁厚度分别为(20．4±4．7)mm和(22．7±6．3)mm。结论中国肥厚型心肌病的长期预后较好,且超声心动图示肥厚仅累及心尖部者预后更佳。发生HCM相关心脑血管不良事件的独立危险因素包括持续或短阵室性心动过速、心功能III级以上。心源性猝死可发生在各年龄,预防中老年患者发生心源性猝死与年轻人同样重要。
Objective To investigate the long-term prognosis and the risk factors associated with HCM death in patients with hypertrophic cardiomyopathy (HCM) in China. Methods The clinical data of 234 HCM patients admitted to our hospital from June 1999 to March 2006 were retrospectively analyzed. The follow-up results of 199 patients were analyzed by SPSS 13.0 statistical software. Results Of the 234 patients, 85% were followed up for a mean follow-up of (31.7 ± 22.6) months. Twenty-one of the patients died during follow-up, of which 19 were identified as HCM-related deaths, including 11 cases of sudden death, 7 cases of heart failure and 1 case of stroke; the other 2 died of accidents and acute pancreatitis . The 1, 2, 3, 4, 5-year survival rates of patients after diagnosis were 96.7%, 94.7%, 94.7%, 93.6%, 89.0% respectively. Univariate analysis showed that there were 7 variables associated with HCM prognosis in men, cardiac function classification, atrial fibrillation, paroxysmal ventricular tachycardia, left atrial enlargement, left ventricular outflow tract obstruction, HCM prognosis; multivariate analysis showed that, Paroxysmal ventricular tachycardia (RR = 2.234, P <0.001), cardiac function III and above (RR = 1.964, P = 0.003) = 0.304), atrial fibrillation (RR = 0.654, P = 0.259), left atrial enlargement (RR = 0.579, P = 0.348), left ventricular outflow tract obstruction , P = 0.821) and family history of HCM (RR = 0.663, P = 0.275) were all risk factors for HCM death. Univariate analysis also showed that echocardiographic findings of death patients were more common in Maron III type, and only one patient showed apical hypertrophic cardiomyopathy. Analysis of sudden death and heart failure death revealed that among the seven sudden death cases, only one patient <35 years old, five patients died of heart failure were over 60 years old, and only two cases of sudden death And 1 patient died of heart failure left ventricular outflow tract pressure at rest; 7 cases of sudden death and 2 cases of heart failure patients with paroxysmal ventricular tachycardia; sudden death and heart failure patients with ventricular wall The thicknesses were (20.4 ± 4.7) mm and (22.7 ± 6.3) mm, respectively. Conclusion The long-term prognosis of hypertrophic cardiomyopathy in China is good, and the echocardiographic hypertrophy involving the apical part of the prognosis is better. Independent risk factors for HCM-related cardiovascular and cerebrovascular events included persistent or bradycardic ventricular tachyarrhythmias, with class III or higher cardiac function. Sudden cardiac death can occur at all ages, prevention of middle-aged and elderly patients with sudden cardiac death and young people are equally important.