Department of Medicine and Medical Specialities, IRCCS Maggiore Hospital, Milan, Italy.
Haemophilia. 2010 May;16 Suppl 3:58-66. doi: 10.1111/j.1365-2516.2010.02262.x.
Improvements in treatment options and healthcare provision mean that haemophilia patients now have a life expectancy approaching that of the normal male population. An increased life expectancy, however, also brings an increased risk of developing age-related disorders, the foremost of which is cardiovascular disease. The epitome of age-related morbidity, cardiovascular disease is also a leading cause of mortality in elderly individuals, and presents a particular challenge when it occurs in persons with haemophilia. While the exact incidence of cardiovascular disease in haemophilia is unknown, incidence rates from conditions such as ischaemic heart disease (IHD) have steadily risen over the last 20-30 years, suggesting that cardiac problems are increasingly relevant for these patients. Management of cardiovascular disease in haemophilia warrants close cooperation between cardiologists and haematologists, and evidence-based guidelines are not available. In the absence of such guidelines, antithrombotic treatment is currently based on local clinical experience and adaptation of the general guidelines used in the non-haemophilic population. In this article, we outline the local guidelines used by our two centres in the antithrombotic treatment of IHD, coronary bypass and valve surgery, and atrial fibrillation in patients with haemophilia. Strategies for the management of haemostasis and thrombosis during cardiovascular surgery in haemophilia patients are also briefly reviewed. Finally, we present the cases of three elderly haemophilia patients with cardiovascular and other age-related health problems in whom such treatment strategies were applied.
治疗方案和医疗保健的改进意味着血友病患者的预期寿命现在接近正常男性人口。然而，预期寿命的延长也带来了与年龄相关的疾病风险的增加，其中首要的是心血管疾病。心血管疾病是与年龄相关的发病率的缩影，也是老年人死亡的主要原因，在血友病患者中更为特殊。尽管确切的心血管疾病发病率在血友病中尚不清楚，但过去 20-30 年来，缺血性心脏病（IHD）等疾病的发病率稳步上升，表明心脏问题对这些患者越来越重要。血友病患者的心血管疾病管理需要心脏病专家和血液学家之间的密切合作，并且没有基于证据的指南。在没有此类指南的情况下，抗血栓治疗目前基于当地的临床经验和非血友病人群中使用的一般指南的适应。在本文中，我们概述了我们两个中心在 IHD、冠状动脉旁路和瓣膜手术以及血友病患者心房颤动的抗血栓治疗中使用的当地指南。还简要回顾了血友病患者心血管手术期间止血和血栓形成管理的策略。最后，我们介绍了三名患有心血管疾病和其他与年龄相关健康问题的老年血友病患者的病例，在这些病例中应用了这种治疗策略。
