扩张性低收缩性状态——肥厚型心肌病的特殊演变  

Dilated-hypokinetic condition—special type of evolution of hyertrophic cardiomyopathy

作　　者：颜彦[1] 王翔飞[1] 王蚰南[2] 王健生[1] 施月芳[3] 潘文明[3] 黄国倩[3] 聂振宁[1] 葛均波[1] 

机构地区：[1]复旦大学附属中山医院心内科,上海200032 [2]复旦大学附属中山医院病理科,上海200032 [3]复旦大学附属中山医院心超室,上海200032

出　　处：《复旦学报（医学版）》2007年第2期198-201,共4页Fudan University Journal of Medical Sciences

摘　　要：目的 研究肥厚型心肌病（hypertrophic cardiomyopathy,HCM）演变为呈扩张型低动力状态的扩张期肥厚型心肌病（dilated-phase hypertrophic cardiomyopathy,DPHCM）的发生率、预测因素、临床及病理表现、治疗与预后.方法 对186例确诊的HCM前瞻性随访研究（10±4）年.结果 共有10例HCM演变为DPHCM,发生率为4.7%.从典型HCM演变为DPHCM的时间为（9±2）年,其中80%（8例）为男性.60%（6例）有HCM家族史（60%vs32%,P＜0.05）;诊断有HCM时年龄较轻[（31±15）岁vs（41±18）岁,P＜0.05];左室壁最大厚度[（22±4）mm vs（19±5）mm,P＜0.05]及左室后壁厚度均较未出现DPHCM者为厚[（15±3）mm vs（13±5）mm,P＜0.05],室内传导阻滞发生率高（40%vs7%,P＜0.05）,演变为DPHCM后1～2年内,5例因心力衰竭死亡（50%vs1%,P＜0.05）,1例接受心脏移植.结论 从典型HCM演变为DPHCM的发生率很低,但一旦演变则预后极差.危险因素有年轻时被诊断有HCM、有HCM家族史、左室壁明显增厚、伴有室内传导阻滞.Purpose To explore the prevalence, predictive factors, clinical and pathologic feature, as well as prognosis of dilated-phase hypertrophic cardiomyopathy （DPHCM）, evolved from classic hypertrophic cardiomyopathy （HCM）. Methods A total of 186 consecutive HCM patients were prospectively evaluated for a mean of （10 ± 4） years. Results Ten patients were diagnosed with DPH- CM with prevalence 4.7%. It took （9 ± 2） years for classic HCM evolving into DPHCM. Of the 10 patients with DPHCM, 8 （80%） were male. Patients with DPHCM were younger at first diagnosis with HCM E（31 ± 15） years vs （41 ± 18） years, P〈0. 05-] and often had a family history of HCM （60% vs 32%, P〈0. 05） with respect to patients who maintained classic HCM. They had greater maximum left ventricular wall thickness [（22 ± 4） mm vs （19 ± 5） mm, P〈0.05] and posterior wall thickenss [（15 ± 3） mm vs （13 ± 5） mm, P〈0.05]. Moreover, they showed a higher occurrence of intraventricular conduction delay （40% vs 7%, P〈0. 05）. During 1 to 2 years after being diagnosed with DPHCM, 5 patients died from heart failure （50% vs 1%, P〈0. 05）. One received heart trans- plantation. Conclusions The occurrence of DPHCM from classic HCM is very low. Patients with young age at diagnosis, family history of HCM, and great wall thickness are at high risk of developing DPHCM, the prognosis of DPHCM is ominous.

关 键 词：肥厚型心肌病 扩张性 低收缩性 扩张期肥厚型心肌病 

分 类 号：R542.2[医药卫生—心血管疾病]