致心律失常性右心室心肌病522例临床分析:单中心21年诊断与治疗变迁

Analysis on Characteristics of 522 Patients With Arrhythmogenic Right Ventricular Cardiomyopathy:Diagnosis and Treatment Progress During the Past 21 Years in a Single Center

目的:总结过去21年间中国医学科学院阜外医院致心律失常性右心室心肌病的诊疗情况。方法:选取1996年7月至2017年12月间就诊的全部致心律失常性右心室心肌病(ARVC)患者共522例。根据患者首次就诊时间分为2010年前就诊患者(简称2010年前,n=141)和2010年及以后就诊患者(简称2010年及以后,n=381)。回顾患者的病历资料,分析不同时间段就诊的ARVC患者接受的诊断和治疗方式。结果:在522例患者中,男性患者373例(71.5%),确诊时平均年龄(39.0±15.1)岁。1996~2017年新就诊患者随时间的变化而增多(P<0.01)。(1)患者首次就诊至确诊间隔时间中位数为277(30,1096)d,2010年及以后较2010年前时间缩短[244(30,984)d vs.357(31,1400)d,P<0.01]。(2)所有患者均接受了心电图和经胸超声心动图检查,137例(26.2%)的患者接受了基因检测,360例(69.0%)患者接受了心脏磁共振成像(CMR)检查,11例(2.1%)患者接受了心内膜活检。患者行CMR和心内膜活检的比例2010年及以后高于2010年前(74.5%vs.53.9%,P<0.01;2.9%vs.0,P<0.05),行基因检测的比例低于2010年前(22.8%vs.35.5%,P<0.01)。(3)患者接受β受体阻滞剂、索他洛尔和胺碘酮治疗的比例分别为28.5%、35.6%和10.9%,2010年及以后较2010年前高(分别为36.5%vs.7.1%、39.9%vs.24.1%、14.4%vs.1.4%,P均<0.01)。(4)接受导管消融术、埋藏式心律转复除颤器(ICD)植入以及心脏移植的患者比例分别为62.3%、19.5%、10.0%,但2010年及以后较2010年前无明显变化(分别为62.7%vs.61.0%、20.5%vs.17.0%、9.4%vs.11.3%,P均>0.05)。结论:ARVC的诊断和治疗水平在过去的20余年中得到了长足的提高,导管消融术成为重要的治疗手段,但在基因检测及心肌活检、规范药物治疗等方面仍存在提升空间。

Objectives:To summarize the improvement of medical management quality of arrhythmogenic right ventricular cardiomyopathy(ARVC)in Fuwai Hospital over the past 21 years.Methods:Five hundred and twenty-two patients with ARVC,who admitted in our hospital between July 1996 and December 2017,were consecutively included in this study.141 and 381 patients admitted to our hospital before and after the year of 2010,respectively.The characteristic of clinical data of patients were analyzed to define the pattern of diagnosis and treatment on this disease during the two phases.Results:There were 373(71.5%)male patients.The average age of all patients was(39.0±15.1)years.The number of first-visit patients significantly increased year by year(P<0.01).(1)The interval between first hospital contact and diagnosis was 277 days(30,1096).The interval between first hospital contact and diagnosis was shorter in patients before 2010 than in patients after 2010[244(30,984)days vs.357(31,1400)days,P<0.01].(2)All patients received electrocardiography(ECG)and echocardiography examinations.Gene test was performed in 137(26.2%)patients,enhanced cardiac magnetic resonance imaging(CMR)was performed in 360(69.0%)patients,endocardial biopsy was performed in 11(2.1%)patients.CMR and endocardial biopsy were performed more often in patients after 2010 compared to patient before 2010(74.5%vs.53.9%,P<0.01;2.9%vs.0,P<0.05),but rate of gene test was lower in patients after 2010 compared to patient before 2010(22.8%vs.35.5%,P<0.01).(3)The rates of patients administratedβblocker,sotalol and amiodarone were 28.5%,35.6%and 10.9%,respectively.βblocker,sotalol and amiodarone were more often used after 2010 than before 2010(36.5%vs.7.1%,39.9%vs.24.1%and 14.4%vs.1.4%,all P<0.01).(4)62.3%,19.5%and 10.0%patients received catheter ablation,implantable cardioverter defibrillator(ICD)and cardiac transplantation.There were no differences of rates of above treatments between before and after 2010(62.7%vs.61.0%,20.5%vs.17.0%and 9.4%vs.11.3%,all P>0.05).Conclusions:The quality of diagnosis and treatment of ARVC is significantly improved in the past two decades in our center,catheter ablation has become an influential therapy strategy.There is still a long way for improvement in gene test,endocardial biopsy and standard medication for patients with ARVC.