A Cross-Sectional Analysis of Cardiovascular Disease in the Hemophilia Population

描述（由申请人提供）：本申请重点关注RFA的优先领域5，“慢性合并症，如肥胖、心血管疾病和糖尿病”。由于治疗成功，血友病患者的寿命更长，生活更有成效。然而，与结果和合并症及其管理有关的许多问题仍然存在。虽然最初认为男性血友病患者不会患心血管疾病，但现在很清楚，动脉粥样硬化血栓事件发生在这一人群中。然而，这类事件的流行程度和造成危险的因素并没有明确的定义。治疗心血管疾病（CVD）的主要方法是抗血栓治疗，这增加了血友病患者出血的风险。我们假设：1)心血管疾病在血友病人群中存在，但不像在普通美国人群中那么普遍。我们进一步假设：2)心血管疾病的危险因素与美国普通男性人群相似，但是3)血友病患者不太可能接受适合年龄的心血管疾病危险因素筛查，4)抗血栓治疗相关出血并发症的风险增加。为了验证这些假设，我们计划利用血友病治疗中心（HTCs）的附属美国血栓和止血网络（ATHN），并使用ATHN安全数据库进行数据输入，以达到以下具体目的：1)确定200名54-73岁的中度或重度血友病A或B男性受试者的心血管疾病患病率；2)比较ARIC数据库中血友病患者与同龄男性患者的患病率数据和心血管疾病危险因素；3)收集200例男性患者抗栓治疗的使用情况及不良事件；4)确定35-54岁的血友病患者中接受适合年龄的心血管疾病危险因素筛查的比例。这些研究结果将为血友病的心血管疾病及其预防和管理提供重要的新数据。这将改善这一患者群体的临床护理，并为今后在这一人群中开展介入性研究和其他研究提供数据基础。

DESCRIPTION (provided by applicant): This application focuses on Priority Area 5 of the RFA,"Chronic co-morbid conditions, such as obesity, cardiovascular disease, and diabetes." Due to successful treatment, men with hemophilia are living longer, more productive lives. However, many questions related to outcomes and co-morbidities and their management remain. While men with hemophilia were initially thought to be protected from cardiovascular disease, it is now clear that atherothrombotic events occur in this population. However, the prevalence of such events and the contributing risk factors are not clearly defined. The mainstay of treatment of cardiovascular disease (CVD) is antithrombotic therapy, which carries increased risks of bleeding in patients with hemophilia. We hypothesize that 1) CVD is present in the hemophilia population but less prevalent than found in the general U.S. population. We further hypothesize that 2) CVD risk factors are similar to those found in the general U.S. male population, but 3) patients with hemophilia are less likely to receive age- appropriate screening for CVD risk factors and 4) are at increased risk of bleeding complications related to antithrombotic treatment. To test these hypotheses we plan to utilize the American Thrombosis and Hemostasis Network (ATHN) of affiliate hemophilia treatment centers (HTCs) and data entry using the ATHN secure database in the following Specific Aims: 1) To determine the prevalence of cardiovascular disease in 200 male subjects with moderate or severe hemophilia A or B aged 54-73 years followed at HTCs; 2) To compare prevalence data and CVD risk factors in subjects with hemophilia with male subjects in the ARIC database of the same age; 3) To collect data on use and adverse events related to antithrombotic therapy in the 200 men; and 4) To determine the proportion of patients with hemophilia ages 35-54 years of age who have had recommended age appropriate screening for CVD risk factors. The results of these studies will provide important new data on CVD in hemophilia and its prevention and management. This should improve clinical care for this patient population as well as provide data on which to base future interventional and other studies in this population.