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目的:探讨肥厚型心肌病(hypertrophic cardiomyopathy,HCM)患者扩张相的特征与生存状况。方法:选取2008-04-2011-08我院收治的HCM患者496例,依据诊断结果分为扩张相组(18例)和对照组(478例),比较2组患者的临床特征和1年、3年、5年生存率,并进行COX回归分析。结果:扩张相组患者的心房颤动(房颤)、猝死、NYHA分级Ⅲ~Ⅳ级、束支传导阻滞比例明显高于对照组,左心室舒张末期内径(LVEDD)、左房最大容积(LAVmax)、左房前后径(LAD)、左室壁厚度(LVWH)水平显著高于对照组,左室射血分数(LVEF)明显低于对照组,以上差异均有统计学意义(P<0.05)。扩张相组1年、3年、5年生存率分别为94.44%、77.78%、61.11%;对照组1年、3年、5年生存率分别为97.07%、93.93%、89.96%。随访结束时,扩张相组2例患者进展为扩张期,7例死亡[2例死于心力衰竭(心衰),5例猝死];对照组中21例进展为扩张期,48例患者死亡(10例死于心衰,38例猝死)。房颤、猝死、NYHA分级Ⅲ~Ⅳ级、LVEF<50%是影响HCM患者扩张相生存状况的独立危险因素(P<0.05)。结论:HCM患者扩张相的主要临床特征为房颤,猝死,NYHA分级Ⅲ~Ⅳ级,束支传导阻滞,LVEDD、LAVmax、LAD、LVWH升高和LVEF降低,患者长期生存状况不佳,其中房颤、猝死、NYHA分级Ⅲ~Ⅳ级、LVEF<50%是影响患者生存状况的独立危险因素。
Objective: To investigate the characteristics and survival of the expansion phase in patients with hypertrophic cardiomyopathy (HCM). Methods: A total of 496 HCM patients admitted to our hospital from 2008-04-2011-08 were divided into two groups according to the diagnosis: 18 cases in the expansion group and 478 cases in the control group. The clinical features, 3 years, 5-year survival rate, and COX regression analysis. Results: Atrial fibrillation (AF), sudden death, NYHA class Ⅲ ~ Ⅳ, and bundle branch block in the patients with dilated phase group were significantly higher than those in control group. LVEDD, LAVmax ), LAD and LVWH were significantly higher than those in the control group, and LVEF was significantly lower than that in the control group (P <0.05) . The 1-year, 3-year and 5-year survival rates of the extended phase group were 94.44%, 77.78% and 61.11%, respectively. The 1-year, 3-year and 5-year survival rates of the control group were 97.07%, 93.93% and 89.96%, respectively. At the end of follow-up, two patients in the extended phase group progressed to expansion, seven died [two died of heart failure (HF) and five died of sudden death]; in the control group, 21 patients progressed to the dilatation phase and 48 patients died 10 died of heart failure, 38 cases of sudden death). Atrial fibrillation, sudden death, NYHA classification Ⅲ ~ Ⅳ, LVEF <50% were independent risk factors affecting the survival of HCM patients (P <0.05). CONCLUSIONS: The main clinical features of dilatation phase in HCM patients are long-term survival of patients with atrial fibrillation, sudden death, NYHA class Ⅲ ~ Ⅳ, bundle branch block, LVEDD, LAVmax, LAD, LVWH and LVEF Atrial fibrillation, sudden death, NYHA classification grade Ⅲ ~ Ⅳ, LVEF <50% are independent risk factors affecting the survival of patients.