Aims Sex differences in hypertrophic cardiomyopathy (HCM) remain unclear. We sought to characterize sex differences in a large HCM referral centre population. Methods and results Three thousand six hundred and seventy-three adult patients with HCM underwent evaluation between January 1975 and September 2012 with 1661 (45.2%) female. Kaplan-Meier survival curves were assessed via log-rank test. Cox proportional hazard regression analyses evaluated the relation of sex with survival. At index visit, women were older (59 ± 16 vs. 52 ± 15 years, P < 0.0001) had more symptoms [New York Heart Association (NYHA) Class III-IV 45.0% vs. 35.3%, P < 0.0001], more obstructive physiology (77.4% vs. 71.8%, P = 0.0001), more mitral regurgitation (moderate or greater in 56.1% vs. 43.9%, P < 0.0001), higher E/e' ratio (n = 1649, 20.6 vs. 15.6, P < 0.0001), higher estimated pulmonary artery systolic pressure (n = 1783, 40.8 ± 15.4 vs. 34.8 ± 10.8 mmHg, P < 0.0001), worse cardiopulmonary exercise performance (n = 1267; percent VO2 predicted 62.8 ± 20% vs. 65.8 ± 19.2%, P = 0.007), and underwent more frequent alcohol septal ablation (4.9% vs. 3.0%, P = 0.004) but similar frequency of myectomy (28% vs. 30%, P = 0.24). Median follow-up was 10.9 (IQR 7.4-16.2) years. Kaplan-Meier analysis demonstrated lower survival in women compared with men (P < 0.0001). In multivariable modelling, female sex remained independently associated with mortality (HR 1.13 [1.03-1.22], P = 0.01) when adjusted for age, NYHA Class III-IV symptoms, and cardiovascular comorbidities. Conclusion Women with HCM present at more advanced age, with more symptoms, worse cardiopulmonary exercise tolerance, and different haemodynamics than men. Sex is an important determinant in HCM management as women with HCM have worse survival. Women may require more aggressive diagnostic and therapeutic approaches.

中文翻译：

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患有肥厚型心肌病的女性生存率更低

目的 肥厚型心肌病 (HCM) 的性别差异仍不清楚。我们试图描述大型 HCM 转诊中心人群的性别差异。方法和结果 1975 年 1 月至 2012 年 9 月，3673 名成年 HCM 患者接受了评估，其中 1661 名（45.2%）女性。通过对数秩检验评估 Kaplan-Meier 生存曲线。Cox 比例风险回归分析评估了性别与生存的关系。在第一次就诊时，年龄较大的女性（59 ± 16 对 52 ± 15 岁，P < 0.0001）有更多症状 [纽约心脏协会 (NYHA) III-IV 级 45.0% 对 35.3%，P < 0.0001]，更多阻塞性生理学（77.4% vs. 71.8%，P = 0.0001），更多二尖瓣反流（中度或更大，56.1% vs. 43.9%，P < 0.0001），更高的 E/e' 比值（n = 1649, 20.6 vs. 15.6 , P < 0。0001），较高的估计肺动脉收缩压（n = 1783, 40.8 ± 15.4 vs. 34.8 ± 10.8 mmHg, P < 0.0001），较差的心肺运动表现（n = 1267；VO2 预测百分比 62.8 ± 20% vs. 65.8 ± 19.2 %，P = 0.007），并且接受了更频繁的酒精间隔消融（4.9% 对 3.0%，P = 0.004），但肌切除术的频率相似（28% 对 30%，P = 0.24）。中位随访时间为 10.9 (IQR 7.4-16.2) 年。Kaplan-Meier 分析表明，女性的生存率低于男性（P < 0.0001）。在多变量模型中，在调整年龄、NYHA III-IV 级症状和心血管合并症后，女性仍然与死亡率独立相关（HR 1.13 [1.03-1.22]，P = 0.01）。结论 HCM 女性患者年龄较大，症状较多，心肺运动耐量较差，和不同于男性的血液动力学。性别是 HCM 管理的一个重要决定因素，因为患有 HCM 的女性生存率较差。女性可能需要更积极的诊断和治疗方法。