Hypertrophic Cardiomyopathy Center of Excellence, Johns Hopkins University, Baltimore, MD, USA; Division of General Medicine, Department of Medicine, Taipei Veterans General Hospital, Taipei, Taiwan; Institute of Public Health, National Yang-Ming University, Taipei, Taiwan.
Hypertrophic Cardiomyopathy Center of Excellence, Johns Hopkins University, Baltimore, MD, USA.
Am Heart J. 2020 Jan;219:58-69. doi: 10.1016/j.ahj.2019.10.004. Epub 2019 Oct 20.
It is unknown whether sex-specific differences in mortality observed in HCM are due to older age of women at presentation, or whether women have greater degree of LV myopathy than men.
We retrospectively compared clinical/imaging characteristics and outcomes between women and men in our overall cohort composed of 728 HCM patients, and in an age-matched subgroup comprised of 400 age-matched patients. We examined sex-specific differences in LV myopathy, and dissected the influence of age and sex on outcomes. LV myopathy was assessed by measuring LV mass, LVEF, global peak longitudinal systolic strain (LV-GLS), diastolic function (E/A, E/e'), late gadolinium enhancement (LV-LGE) and myocardial blood flow (MBF) at rest/stress. The primary endpoint was a composite outcome, comprising heart failure (HF), atrial fibrillation (AFib), ventricular tachycardia/fibrillation (VT/VF) and death; individual outcomes were defined as the secondary endpoint.
Women in the overall cohort were older by 6 years. Women were more symptomatic and more likely to have obstructive HCM. Women had smaller LV cavity size, stroke volume and LV mass, higher indexed maximum wall thickness (IMWT), more hyperdynamic LVEF and higher/similar LV-GLS. Women had similar LV-LGE and E/A, but higher E/e' and rest/stress MBF. Female sex was independently associated with the composite outcome in the overall cohort, and with HF in the overall cohort and age-matched subgroup after adjusting for obstructive HCM, LA diameter, LV-GLS.
Our results suggest that sex-specific differences in LV geometry, hyper-contractility and diastolic function, not greater degree of LV myopathy, contribute to a higher, age-independent risk of diastolic HF in women with HCM.
肥厚型心肌病（HCM）患者死亡率的性别差异是由于女性就诊时年龄较大，还是女性的左心室（LV）心肌病变程度比男性更大，目前尚不清楚。
我们回顾性比较了我们的整体队列中 728 名 HCM 患者以及 400 名年龄匹配患者亚组中女性和男性的临床/影像学特征和结局。我们检查了 LV 心肌病变的性别差异，并剖析了年龄和性别对结局的影响。通过测量 LV 质量、LVEF、整体峰值纵向收缩应变（LV-GLS）、舒张功能（E/A、E/e'）、延迟钆增强（LV-LGE）和静息/应激时的心肌血流（MBF）来评估 LV 心肌病变。主要终点是复合终点，包括心力衰竭（HF）、心房颤动（AFib）、室性心动过速/颤动（VT/VF）和死亡；个别结局定义为次要终点。
整体队列中的女性年龄大 6 岁。女性症状更明显，更可能患有梗阻性 HCM。女性的 LV 腔更小，心搏量和 LV 质量更小，最大室壁厚度指数（IMWT）更高，LVEF 更高且更有活力，LV-GLS 更高/相似。女性的 LV-LGE 和 E/A 相似，但 E/e'和静息/应激 MBF 更高。在调整梗阻性 HCM、左心房直径和 LV-GLS 后，女性性别与整体队列中的复合结局以及整体队列和年龄匹配亚组中的 HF 独立相关。
我们的研究结果表明，LV 几何形状、高收缩性和舒张功能的性别差异，而不是 LV 心肌病变程度更大，导致 HCM 女性发生年龄独立的舒张性 HF 的风险更高。
