Ten Cate F J
Thoraxcentre, University Hospital, The Netherlands.
J Insur Med. 1996;28(1):42-5.
The actual prognosis of hypertrophic cardiomyopathy, a disorder previously thought of to be notorious for an increased risk of untimely death is poorly defined.
The present study describes the findings of patients with Hypertrophic Cardiomyopathy followed for more than nineteen years in a large clinic population.
A clinic population of 113 patients with Hypertrophic Cardiomyopathy was prospectively studied to assess cardiac mortality in the overall groups and in selected subgroups commonly thought to be at high risk for sudden death. Diagnosis of hypertrophic cardiomyopathy was based on the presence of left ventricular hypertrophy without a known cause. Left ventricular hypertrophy was determined by echocardiography.
During follow-up there were 11 cardiac and 2 non cardiac deaths. The annual cardiac mortality was 1% (95% confidence interval 0.2-1.8%). Relative risk for cardiac death was not significantly different in the presence of young age (< 30 years), family history for Hypertrophic Cardiomyopathy (HCM) and sudden death, history for syncope or previous cardiac arrest or both, ventricular tachyeardia on 24-hour, holter monitoring or operation for refractory symptoms and out-flowtract obstruction.
HCM has a relatively benign prognosis (1% cardiac annual mortality) that is 2-4 times less than previously thought. These findings might have important consequences for risk assessment in individual patients. Echocardiography is obligatory to determine the presence, and extent of myocardial hypertrophy. In addition, the technique allows differentiation between Hypertrophic Cardiomyopathy and Athlete's Heart.
肥厚型心肌病曾被认为是因过早死亡风险增加而声名狼藉的一种疾病，但其实际预后情况却界定不清。
本研究描述了在一大群临床患者中对肥厚型心肌病患者进行超过19年随访的结果。
对113例肥厚型心肌病临床患者进行前瞻性研究，以评估总体人群以及通常被认为猝死风险高的特定亚组中的心脏死亡率。肥厚型心肌病的诊断基于存在原因不明的左心室肥厚。左心室肥厚通过超声心动图确定。
在随访期间，有11例心脏死亡和2例非心脏死亡。年心脏死亡率为1%（95%置信区间0.2 - 1.8%）。在年龄小于30岁、有肥厚型心肌病（HCM）家族史和猝死家族史、有晕厥或既往心脏骤停史或两者皆有、24小时动态心电图监测发现室性心动过速或因难治性症状及流出道梗阻接受手术的情况下，心脏死亡的相对风险无显著差异。
肥厚型心肌病预后相对良好（年心脏死亡率为1%），比之前认为的低2至4倍。这些发现可能对个体患者的风险评估有重要影响。超声心动图对于确定心肌肥厚的存在及程度是必不可少的。此外，该技术可区分肥厚型心肌病和运动员心脏。
