肥厚型心肌病扩张相的临床特点及预后分析  

Clinical characteristics and outcome of patients with dilated-hypertrophic cardiomyopathy

作　　者：李品睿 李晓连[1] 许菲[1] 苟成 邓明洁[1] 刘金秋[1] 王珂[1] 

机构地区：[1]大连医科大学附属第一医院心内科,116021

出　　处：《中华心血管病杂志》2016年第4期327-330,共4页Chinese Journal of Cardiology

摘　　要：目的 探讨肥厚型心肌病扩张相（D-HCM）的临床特点及预后.方法 回顾性分析2002年1月至2015年12月于大连医科大学附属第一医院住院的肥厚型心肌病患者的临床资料,根据超声心动图测得的左心室舒张末期内径（LVEDD）分为D-HCM组和左心室内径正常组,比较两组患者的临床特点.对D-HCM患者通过门诊或电话随访了解预后情况.结果 13年间入院诊断为肥厚型心肌病的患者共616例,临床资料完整的患者579例,其中21例演变为D-HCM,发生率为3.4％.诊断D-HCM的平均年龄为（58.8±10.4）岁（42- 78岁）,从诊断肥厚型心肌病至诊断D-HCM的平均时间为（14.1±7.1）年.21例患者中男性13例（61.9％）.与左心室内径正常组（n=558）患者比较,D-HCM组患者诊断HCM的年龄较轻[（39.7±10.4）岁比（48.5±9.5）岁,P＜0.001],家族猝死史比率较高[19.0％（4/21）比2.5％（14/558）,P=0.003],室性心动过速发生率较高[38.1％（8/21）比5.7％（32/558）,P＜0.001],肌钙蛋白Ⅰ水平升高的比率较高[66.7％（14/21）比9.3％（52/558）,P＜0.001],诊断HCM时最大左心室壁厚度较大[（21.8±4.2） mm比（17.2±3.5） mm,P＜0.001],左心房内径亦较大[（39.8±5.9） mm比（35.2±3.3） mm,P＜0.001].D-HCM患者的平均随访时间为（3.8±1.9）年,随访期间12例（57.1％）死亡,其中5例（23.8％）死于心力衰竭,7例猝死（33.3％）.1例（4.8％）因严重心力衰竭行心脏移植.结论 肥厚型心肌病患者中D-HCM发生率较低.肥厚型心肌病患者中,诊断年龄小、伴家族猝死史、肌钙蛋白Ⅰ水平升高以及伴室性心动过速者演变为D-HCM的可能性大.D-HCM的患者预后差,心力衰竭和猝死为其主要死亡原因.Objective To define the clinical characteristics and outcome of patients with dilatedhypertrophic cardiomyopathy （D-HCM）.Methods Clinical data of HCM patients hospitalized from January 2002 to December 2015 in our hospital were retrospectively analyzed.Patients were divided into D-HCM and classic HCM patients.The D-HCM patients were followed up by phone.Results A total of 616 consecutive HCM patients were evaluated.Twenty one patients （3.4%） were diagnosed with D-HCM （average age （58.8 ± 10.4） years,13 males）.It took （14.2 ± 7.1） years for classic HCM patients to develop D-HCM.Compared to classic HCM patients,D-HCM patients were younger at the time of first HCM diagnosis （（39.7 ± 10.4） years old vs.（48.5 ±9.5） years old,P 〈0.001） and had higher ratio of sudden cardiac death family history （19.0% （4/21） vs.2.5% （14/558）,P =0.003）,more patients of future D-HCM patients had ventricular tachycardia （38.1% （8/21） vs.5.7% （32/558）,P 〈0.001） and higher TroponinⅠ（66.7% （14/21） vs.9.3% （52/558）,P 〈 0.001） before the left ventricular cavity enlargement.Moreover,MLVWH （（24.8 ± 4.2） mm vs.（17.2 ± 3.5） mm,P 〈 0.001） was significantly thicker and LAD （（39.8 ±5.9） mm vs.（35.2 ± 3.3） mm,P 〈 0.001） was significantly larger in D-HCM patients than in classical HCM patients.During the（3.8 ± 1.9） years follow up period,12 out of 21 D-HCM patients died （57.1%）,5 cases（23.8%）died of severe heart failure and 7 cases（33.3%） died of sudden cardiac death.One patient received heart transplantation.Conclusions Few classical HCM patients progressed into D-HCM in this cohort.Patients diagnosed as HCM at young age,HCM patients with abnormal Troponin Ⅰ and ventricular tachycardia are at higher risk of developing D-HCM.The prognosis of D-HCM is very poor,and heart failure and sudden cardiac death are the main causes of death.

关 键 词：心肌病 肥厚性 预后 

分 类 号：R542.2[医药卫生—心血管疾病]