A cross-sectional analysis of cardiovascular disease in the hemophilia population.

• Authors: Suman L. Sood, University of Michigan; Dunlei Cheng, American Thrombosis and Hemostasis Network; Margaret Ragni, University of Pittsburgh; Craig M. Kessler, Georgetown University; Doris Quon, Orthopedic Hospital; Amy D. Shapiro, Indiana Hemophilia and Thrombosis Center; Nigel S. Key, University of North Carolina; Marilyn J. Manco-Johnson, University of Colorado; Adam Cuker, University of Pennsylvania; Christine Kempton, Emory University; Tzu-Fei Wang, The Ohio State University; M. Elaine Eyster, Penn State Milton S. Hershey Medical Center; Philip Kuriakose, Henry Ford Hospital; Annette von Drygalski, University of California; Joan Cox Gill, Blood Center of Wisconsin; Allison Wheeler, Vanderbilt University; Peter Kouides, Mary M. Gooley Hemophilia Center; Miguel A. Escobar, University of Texas Health Science Center and McGovern Medical School; Cindy Leissinger, Tulane University.; Sarah Galdzicka, Bloodworks Northwest; Marshall Corson, University of Washington; Crystal Watson, American Thrombosis and Hemostasis Network; Barbara A. Konkle, Bloodworks Northwest
• Journal Title: Blood Advances
• Volume: Volume 2, Number 11
• Publisher: American Society of Hematology: Blood Advances | 2018-06-12, Pages 1325-1333
• Type of Work: Article | Final Publisher PDF
• Publisher DOI: http://doi.org/10.1182/bloodadvances.2018018226
• Subjects: Health Sciences, Medicine and Surgery

Abstract

Men with hemophilia were initially thought to be protected from cardiovascular disease (CVD), but it is now clear that atherothrombotic events occur. The primary objective of the CVD in Hemophilia study was to determine the prevalence of CVD and CVD risk factors in US older men with moderate and severe hemophilia and to compare findings with those reported in age-comparable men in the Atherosclerosis Risk in Communities (ARIC) cohort. We hypothesized if lower factor levels are protective from CVD, we would see a difference in CVD rates between more severely affected and unaffected men. Beginning in October 2012, 200 patients with moderate or severe hemophilia A or B (factor VIII or IX level ≤ 5%), aged 54 to 73 years, were enrolled at 19 US hemophilia treatment centers. Data were collected from patient interview and medical records. A fasting blood sample and electrocardiogram (ECG) were obtained and assayed and read centrally. CVD was defined as any angina, any myocardial infarction by ECG or physician diagnosis, any self-reported nonhemorrhagic stroke or transient ischemic attack verified by physicians, or any history of coronary bypass graft surgery or coronary artery angioplasty. CVD risk factors were common in the population. Compared with men of similar age in the ARIC cohort, patients with hemophilia had significantly less CVD (15% vs 25.8%; P < .001). However, on an individual patient level, CVD events occur and efforts to prevent cardiovascular events are warranted. Few men were receiving secondary prophylaxis with low-dose aspirin, despite published opinion that it can be used safely in this patient population.