Department of Cardiology, Hospital Universitario Virgen de la Arrixaca, and Centro Regional de Hemodonación, Universidad de Murcia, Murcia, Spain.
Thromb Res. 2010 Jul;126(1):e46-50. doi: 10.1016/j.thromres.2010.01.010. Epub 2010 Feb 13.
Hypertrophic cardiomyopathy (HCM) is characterised by inappropriate hypertrophy, small-vessel coronary artery disease, myocyte disarray and increased interstitial fibrosis. Microvascular dysfunction is a common finding in HCM and its extent has been proposed as an important prognostic marker. Plasma von Willebrand factor (vWf) is an established marker of endothelial damage or dysfunction; however it has scarcely been studied in HCM. We hypothesised that vWf could be raised in patients with HCM and be related to different variables associated with severity of HCM.
We included 124 HCM patients, 93 males, aged 48+/-15 years, 59 healthy control subjects with similar age and sex and 20 patients with ischemic heart disease but clinical stability for the last 6 months. A complete history and clinical examination was performed, including 12-lead electrocardiogram, echocardiography, 24 hours ECG-Holter monitoring, and symptom limited treadmill exercise test. Risk factors for sudden death were evaluated. A blinded cardiac MRI was performed with late enhanced study with Gadolinium. Plasma vWf levels were assayed by commercial ELISA.
Patients showed higher levels of vWf (140.0+/-65.0 UI/ml vs 105.0+/-51.0 UI/ml, p<0.001) even after adjusting for ABO blood group. vWf levels were found raised in patients with severe functional class (168.4+/-65.9 UI/mL vs 132.4+/-60.7 UI/mL, p=0.020), atrial fibrillation (175.8+/-69.4 UI/mL vs 133.0+/-59.0 UI/mL, p=0.005), hypertension (161.4+/-60.8 vs 128.9+/-60.5, p=0.010) obstruction (153.9+/-67.9 vs 128.2+/-57.4 UI/mL, p=0.046) and non sustained ventricular tachycardia (159.3+/-59.1 vs 133.0+/-63.0, p=0.049). vWf correlated with age (r:0.26; p=0.006) and obstruction (r:0.22; p=0.021).
We show, for the first time, patients with HCM present significantly raised levels of vWf. These are associated with different conditions related to the severity of the disease.
肥厚型心肌病（HCM）的特征是不适当的心肌肥厚、小血管冠状动脉疾病、心肌排列紊乱和间质纤维化增加。微血管功能障碍是 HCM 的常见表现，其程度被认为是一个重要的预后标志物。血浆血管性血友病因子（vWf）是内皮损伤或功能障碍的一个既定标志物；然而，它在 HCM 中的研究甚少。我们假设 vWf 可能在 HCM 患者中升高，并与与 HCM 严重程度相关的不同变量有关。
我们纳入了 124 名 HCM 患者，93 名男性，年龄 48+/-15 岁，59 名年龄和性别相似的健康对照者，以及 20 名患有缺血性心脏病但在过去 6 个月内临床稳定的患者。进行了完整的病史和临床检查，包括 12 导联心电图、超声心动图、24 小时心电图 Holter 监测和症状限制跑步机运动试验。评估了猝死的危险因素。使用钆增强的晚期对比增强心脏 MRI 进行了心脏 MRI。通过商业 ELISA 测定血浆 vWf 水平。
患者的 vWf 水平较高（140.0+/-65.0 UI/ml 比 105.0+/-51.0 UI/ml，p<0.001），即使在调整 ABO 血型后也是如此。在严重功能分级（168.4+/-65.9 UI/mL 比 132.4+/-60.7 UI/mL，p=0.020）、心房颤动（175.8+/-69.4 UI/mL 比 133.0+/-59.0 UI/mL，p=0.005）、高血压（161.4+/-60.8 比 128.9+/-60.5，p=0.010）、梗阻（153.9+/-67.9 比 128.2+/-57.4 UI/mL，p=0.046）和非持续性室性心动过速（159.3+/-59.1 比 133.0+/-63.0，p=0.049）患者中发现 vWf 升高。vWf 与年龄（r：0.26；p=0.006）和梗阻（r：0.22；p=0.021）相关。
我们首次表明，HCM 患者的 vWf 水平显著升高。这些与与疾病严重程度相关的不同情况有关。
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