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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DPH-CM,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)mmvs(19±5)mm,P<0.05]及左室后壁厚度均较未出现DPHCM者为厚[(15±3)mmvs(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。
Objective To investigate the incidence, predisposing factors, clinical and pathological manifestations, treatment and prognosis of dilated-phase hypertrophic cardiomyopathy (DPHCM) with hypertrophic cardiomyopathy (HCM) evolving into dilated low-power state Prognosis. Methods A prospective follow-up study of 186 patients diagnosed with HCM (10 ± 4) years was performed. Results A total of 10 cases of HCM evolved into DPH-CM, the incidence was 4.7%. The time from typical HCM to DPHCM was (9 ± 2) years, of which 80% (8 cases) were men. 60% (6 cases) had a family history of HCM (60% vs 32%, P <0.05); age at diagnosis was (31 ± 15) vs (41 ± 18) years old The mean thickness of the wall [(22 ± 4) mm vs (19 ± 5) mm, P <0.05] and the thickness of the left ventricular posterior wall were thicker than those without DPH (15 ± 3) mm vs 0.05). The incidence of conduction block was high (40% vs 7%, P <0.05). Five patients died of heart failure within one to two years after DPHCM (50% vs 1%, P <0.05) Accept a heart transplant Conclusions The incidence of DPHCM evolvement from a typical HCM is very low, but its prognosis is very poor once it has evolved. Risk factors were diagnosed with HCM when young, with a family history of HCM, left ventricular wall was significantly thicker, with conduction block.