Maron Barry J, Casey Susan A, Hauser Robert G, Aeppli Dorothee M
Hypertrophic Cardiomyopathy Center, Minneapolis Heart Institute Foundation, 920 E. 28th Street, Suite 60, Minneapolis, MN 55407, USA.
J Am Coll Cardiol. 2003 Sep 3;42(5):882-8. doi: 10.1016/s0735-1097(03)00855-6.
This study was designed to clarify and resolve the clinical profile of older patients with hypertrophic cardiomyopathy (HCM).
Adverse consequences of HCM such as sudden death and incapacitating symptoms have been emphasized for the young and middle-aged.
Long-term outcome of HCM was assessed in a community-based cohort not subject to tertiary center referral bias.
Of 312 patients, 73 (23%) achieved normal life expectancy (> or =75 years; range to 96); 44 (14%) were > or =80 years old. Most patients > or =75 years (47; 64%) experienced no or only mild limiting symptoms and lived virtually their entire lives with few HCM-related clinical consequences; 26 patients (36%) experienced severe progressive symptoms. In elderly patients with HCM, diagnosis and symptom onset were considerably delayed to 74 +/- 8 and 70 +/- 11 years, respectively. For patients > or =50 years at diagnosis, the probability of survival for 5, 10, and 15 years was 85 +/- 3%, 74 +/- 4%, and 57 +/- 6%, respectively, and did not significantly differ from a matched general population (p = 0.20). Patients > or =75 years were predominantly women, and had less marked wall thickness and more frequently showed basal outflow obstruction > or =30 mm Hg (compared with those <75 years; p < 0.01 and 0.001, respectively).
Hypertrophic cardiomyopathy is frequently well tolerated and compatible with normal life expectancy, and may remain clinically dormant for long periods of time with symptoms and initial diagnosis deferred until late in life. These observations afford a measure of reassurance to many patients with HCM, a disease for which clinical course is often unfavorable and unpredictable.
本研究旨在阐明并解决老年肥厚型心肌病（HCM）患者的临床特征。
HCM的不良后果如猝死和致残症状在中青年患者中受到了更多关注。
在一个基于社区的队列中评估HCM的长期预后，该队列不存在三级中心转诊偏倚。
312例患者中，73例（23%）达到正常预期寿命（≥75岁；范围至96岁）；44例（14%）年龄≥80岁。大多数≥75岁的患者（47例，64%）没有或仅有轻微的限制症状，几乎一生都没有HCM相关的临床后果；26例患者（36%）出现严重的进行性症状。老年HCM患者的诊断和症状出现分别显著延迟至74±8岁和70±11岁。对于诊断时年龄≥50岁的患者，5年、10年和15年的生存率分别为85±3%、74±4%和57±6%，与匹配的一般人群无显著差异（p = 0.20）。≥75岁的患者以女性为主，室壁厚度不那么明显，更频繁地出现基底部流出道梗阻≥30 mmHg（与<75岁的患者相比；分别为p < 0.01和0.001）。
肥厚型心肌病通常耐受性良好，与正常预期寿命相符，可能在临床上长时间处于隐匿状态，症状和初始诊断延迟至晚年。这些观察结果让许多HCM患者放心，这种疾病的临床病程通常不佳且不可预测。
