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目的探讨肥厚型心肌病(HCM)患者的长期预后及其死亡的相关危险因素。方法回顾性分析1999年6月至2006年3月期间收入院的234例 HCM 患者的病史资料,并对其中获得随访的199例患者的随访结果采用 SPSS 13.0软件包进行统计学处理。结果 234例患者中,199例(85%)获得随访,35例(15%)患者失访。平均随访时间为(31.7±22.6)个月,由确诊到随访结束时间中位数为35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,包括心脏骤停11例(57.9%)、心力衰竭死亡7例(36.8%)、脑卒中死亡1例,另有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Ⅲ及以上、心房颤动、持续或短阵室速、左心房增大、左心室流出道梗阻、HCM 家族史7个变量与 HCM 预后相关。而多因素分析显示,仅持续或短阵室速(RR=2.234,P<0.001)、心功能Ⅲ及以上(RR=1.964,P=0.003)是 HCM 的独立预后危险因素。心脑事件死亡患者中,超声心动图表现以 MaronⅢ型为多见(73.7%),仅1例患者表现为心尖肥厚型心肌病(5.2%)。对心脏骤停者和心力衰竭死亡者进行分析显示,7例(63.6%)心脏骤停者发生在60岁以下的患者中(P=0.12,但仅1例患者小于35岁),5例(71.4%)心力衰竭死亡者发生在60岁以上的患者中(P=0.22)。仅2例心脏骤停者和1例心力衰竭死亡者存在静息状态下左心室流出道压差。7例(63.6%)心脏骤停者和2例(28.6%)心力衰竭死亡者合并阵发性室速。心脏骤停者与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 HCM 患者的中长期预后相对较好,以心肌肥厚仅累及心尖部者预后最佳。持续或短阵室速、心功能Ⅲ级以上是发生 HCM 相关心脑血管不良事件的独立危险因素。心脏骤停可发生在各年龄段,对其的预防在年轻和中老年患者中同样重要。
Objective To investigate the long-term prognosis and related risk factors of death in patients with hypertrophic cardiomyopathy (HCM). Methods A retrospective analysis of medical records of 234 HCM patients from June 1999 to March 2006 was conducted. The follow-up results of 199 patients who were followed up were analyzed by SPSS 13.0 software package. Results Of the 234 patients, 199 (85%) were followed up and 35 (15%) were lost. The average follow-up time was (31.7 ± 22.6) months and the median time from diagnosis to follow-up was 35 months. Of the 21 patients who died at follow-up, 19 were identified as HCM-related deaths, including cardiac arrest in 11 patients (57.9%), heart failure in 7 patients (36.8%), stroke in 1 patient and another 2 patients Patients were killed due to accident 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 seven variables of male, cardiac function Ⅲ and above, atrial fibrillation, persistent or bradyarrhythmia, left atrial enlargement, left ventricular outflow tract obstruction, and family history of HCM were associated with HCM prognosis. Multivariate analysis showed that only sustained or short-term ventricular tachycardia (RR = 2.234, P <0.001), cardiac function Ⅲ and above (RR = 1.964, P = 0.003) were independent prognostic risk factors for HCM. Echocardiographic findings were more common with Maron type III (73.7%) in patients with cardiocerebral events, and only 1 patient showed apical hypertrophic cardiomyopathy (5.2%). Analysis of cardiac arrest and heart failure deaths revealed that 7 (63.6%) cardiac arrest occurred in patients under 60 years of age (P = .12, but only 1 patient was less than 35 years of age) and 5 ( 71.4%) of those who died of heart failure occurred in patients over the age of 60 (P = 0.22). Only 2 cases of cardiac arrest and 1 case of heart failure have left ventricular outflow tract pressure at rest. Seven patients (63.6%) with cardiac arrest and two patients (28.6%) with heart failure died of paroxysmal supraventricular tachycardia. The wall thickness of those who died of cardiac arrest and heart failure were (20.4 ± 4.7) mm and (22.7 ± 6.3) mm, respectively. Conclusion The long-term prognosis of HCM patients is relatively good, and the best prognosis of patients with cardiac hypertrophy involving apex only. Continuous or short-term ventricular tachycardia, cardiac function above grade III is an independent risk factor for HCM-related cardiovascular and cerebrovascular adverse events. Cardiac arrest can occur at all ages and its prevention is equally important in young and middle-aged patients.