Division of Allergy & Clinical Immunology, Johns Hopkins University School of Medicine, Baltimore, MD, 21224, USA.
Department of Respiratory and Critical Care Medicine, West China Hospital, Sichuan University, Chengdu, Sichuan, 610041, China.
Sci Rep. 2017 Sep 20;7(1):11957. doi: 10.1038/s41598-017-12289-4.
Hypertrophic cardiomyopathy (HCM) is a clinically and genetically heterogeneous disorder but data on survival rates are still conflicting and have not so far been quantitatively reviewed. The aim of this study is to conduct a meta-analysis of cohort studies to assess pooled survival rates and prognostic factors for survival in patients with HCM. Nineteen studies were included representing 12,146 HCM patients. The pooled 1-, 3-, 5- and 10-year survival rates were 98.0%, 94.3%, 82.2% and 75.0%, respectively. Among patients with HCM, age, NYHA functional class, family history of sudden death (FHSD), syncope, atrial fibrillation, non-sustained ventricular tachycardia (nsVT), maximum left ventricular wall thickness and obstruction were significant prognostic factors for cardiovascular death. For sudden cardiac death, FHSD, nsVT, and obstruction showed significant predictive values. Moreover, estimation of population attributable risk (PAR) suggested that nsVT was the strongest predictor for cardiovascular death (13.02%, 95% CI 3.60-25.91%), while left ventricular outflow tract obstruction/mid-ventricular obstruction (LVO/MVO) was the strongest predictor for all-cause death and sudden cardiac death (10.09%, 95% CI 4.72-20.42% and 16.44%, 95% CI 7.45-31.55%, respectively). These risk factors may thus be useful for identifying HCM patients who might benefit from early diagnosis and therapeutic interventions.
肥厚型心肌病（HCM）是一种临床表现和遗传异质性的疾病，但目前关于生存率的数据仍存在争议，尚未进行定量综述。本研究旨在对队列研究进行荟萃分析，以评估 HCM 患者的总体生存率和生存率的预测因素。纳入了 19 项研究，共代表了 12146 名 HCM 患者。总体的 1 年、3 年、5 年和 10 年生存率分别为 98.0%、94.3%、82.2%和 75.0%。在 HCM 患者中，年龄、NYHA 功能分级、家族性猝死史（FHSD）、晕厥、心房颤动、非持续性室性心动过速（nsVT）、左心室壁最大厚度和梗阻是心血管死亡的重要预后因素。对于心脏性猝死，FHSD、nsVT 和梗阻具有显著的预测价值。此外，人群归因风险（PAR）估计表明 nsVT 是心血管死亡的最强预测因素（13.02%，95%CI 3.60-25.91%），而左心室流出道梗阻/中室梗阻（LVO/MVO）是全因死亡和心脏性猝死的最强预测因素（10.09%，95%CI 4.72-20.42%和 16.44%，95%CI 7.45-31.55%）。这些危险因素可能有助于识别可能受益于早期诊断和治疗干预的 HCM 患者。
