Hypertrophic Cardiomyopathy Center, Minneapolis Heart Institute Foundation, Minneapolis, Minnesota.
Hypertrophic Cardiomyopathy Center, Tufts Medical Center, Boston, Massachusetts.
J Am Coll Cardiol. 2015 May 12;65(18):1915-28. doi: 10.1016/j.jacc.2015.02.061.
Hypertrophic cardiomyopathy (HCM) has been prominently associated with adverse disease complications, including sudden death or heart failure death and a generally adverse prognosis, with annual mortality rates of up to 6%.
This study determined whether recent advances in management strategy, including implantable cardioverter-defibrillators (ICDs), heart transplantation, or other therapeutic measures have significantly improved survival and the clinical course of adult HCM patients.
We addressed long-term outcomes in 1,000 consecutive adult HCM patients presenting at 30 to 59 years of age (mean 45±8 years) over 7.2±5.2 years of follow-up.
Of 1,000 patients, 918 (92%) survived to 53±9.2 years of age (range 32 to 80 years) with 91% experiencing no or only mild symptoms at last evaluation. HCM-related death occurred in 40 patients (4% [0.53%/year]) at 50±10 years from the following events: progressive heart failure (n=17); arrhythmic sudden death (SD) (n=17); and embolic stroke (n=2). In contrast, 56 other high-risk patients (5.6%) survived life-threatening events, most commonly with ICD interventions for ventricular tachyarrhythmias (n=33) or heart transplantation for advanced heart failure (n=18 [0.79%/year]). SD occurred in patients who declined ICD recommendations, had evaluations before application of prophylactic ICDs to HCM, or were without conventional risk factors. The 5- and 10-year survival rates (confined to HCM deaths) were 98% and 94%, respectively, not different from the expected all-cause mortality in the general U.S. population (p=0.25). Multivariate independent predictors of adverse outcome were younger age at diagnosis, female sex, and increased left atrial dimension.
In a large longitudinally assessed adult HCM cohort, we have demonstrated that contemporary management strategies and treatment interventions, including ICDs for SD prevention, have significantly altered the clinical course, now resulting in a low disease-related mortality rate of 0.5%/year and an opportunity for extended longevity.
肥厚型心肌病（HCM）与不良疾病并发症显著相关，包括猝死或心力衰竭死亡和普遍不良预后，年死亡率高达 6%。
本研究旨在确定管理策略的最新进展，包括植入式心脏复律除颤器（ICD）、心脏移植或其他治疗措施是否显著改善了成年 HCM 患者的生存和临床病程。
我们研究了 1000 例连续就诊的成年 HCM 患者的长期预后，这些患者在 7.2±5.2 年的随访中于 30 至 59 岁（平均 45±8 岁）就诊。
在 1000 例患者中，918 例（92%）存活至 53±9.2 岁（范围 32 至 80 岁），91%的患者在最后一次评估时无症状或仅有轻度症状。HCM 相关死亡发生在 40 例患者（4%[0.53%/年]），死亡时间距就诊时间为 50±10 年，原因包括进行性心力衰竭（n=17）、心律失常性猝死（SD）（n=17）和栓塞性卒中（n=2）。相比之下，56 例（5.6%）其他高危患者存活过危及生命的事件，最常见的是因室性心动过速/心室颤动而接受 ICD 干预（n=33）或因晚期心力衰竭而接受心脏移植（n=18[0.79%/年]）。SD 发生在拒绝 ICD 建议的患者、在应用预防性 ICD 于 HCM 之前进行评估的患者或无常规危险因素的患者中。5 年和 10 年生存率（仅限于 HCM 死亡）分别为 98%和 94%，与美国一般人群的全因死亡率无差异（p=0.25）。不良预后的多变量独立预测因素是诊断时年龄较小、女性和左心房内径增加。
在一项大型成年 HCM 队列的纵向评估中，我们证明了当代管理策略和治疗干预措施，包括用于 SD 预防的 ICD，已显著改变了临床病程，现在导致 0.5%/年的低疾病相关死亡率和延长寿命的机会。
