Department of Cardiology, Tokyo Women's Medical University, Shinjuku-ku, Tokyo, Japan.
J Card Fail. 2011 Jun;17(6):459-64. doi: 10.1016/j.cardfail.2011.01.015. Epub 2011 Mar 25.
It remains unclear whether end-stage hypertrophic cardiomyopathy (HCM) is associated with as high a rate of sudden death as occurs among HCM patients with preserved left ventricular (LV) systolic function. The purpose of this study was to evaluate the incidence of sudden death among patients with end-stage HCM and to identify high-risk end-stage patients.
A total of 490 consecutive patients with HCM, who were diagnosed and followed-up at our hospital, were analyzed retrospectively. End-stage HCM was defined by an LV ejection fraction <50% on echocardiography during follow-up. Among the 490 HCM patients, 43 patients (8.8%) were diagnosed as having end-stage HCM during a mean follow-up period of 12 ± 7 years after the initial diagnosis. During a mean follow-up period of 5 ± 3 years after progression to end-stage HCM, sudden death occurred in 21 of 43 patients (47%). Cox proportional hazards analysis identified syncope as an independent predictor of sudden death (hazard ratio = 6.15; 95% confidence interval, 2.40-15.75; P < .001).
This study demonstrated that patients with end-stage HCM have a high incidence of sudden death. Therefore, it is suggested that an aggressive therapeutic strategy to counter sudden death should be considered for patients with end-stage HCM.
目前尚不清楚终末期肥厚型心肌病（HCM）是否与左心室（LV）收缩功能正常的 HCM 患者一样，具有较高的猝死发生率。本研究旨在评估终末期 HCM 患者猝死的发生率，并确定高危终末期患者。
回顾性分析了在我院诊断和随访的 490 例连续 HCM 患者。终末期 HCM 定义为在随访过程中超声心动图显示 LV 射血分数<50%。在 490 例 HCM 患者中，43 例（8.8%）在最初诊断后 12±7 年的平均随访期间被诊断为终末期 HCM。在进展为终末期 HCM 后平均 5±3 年的随访期间，21 例（47%）患者发生猝死。Cox 比例风险分析确定晕厥是猝死的独立预测因素（风险比=6.15；95%置信区间，2.40-15.75；P<0.001）。
本研究表明，终末期 HCM 患者猝死发生率较高。因此，建议对终末期 HCM 患者采取积极的抗猝死治疗策略。
