Suman L. Sood 1 , Dunlei Cheng 2 , Margaret Ragni 3 , Craig M. Kessler 4 , Doris Quon 5 , Amy D. Shapiro 6 , Nigel S. Key 7 , Marilyn J. Manco-Johnson 8 , Adam Cuker 9 , Christine Kempton 10 , Tzu-Fei Wang 11 , M. Elaine Eyster 12 , Philip Kuriakose 13 , Annette von Drygalski 14 , Joan Cox Gill 15 , Allison Wheeler 16 , Peter Kouides 17 , Miguel A. Escobar 18 , Cindy Leissinger 19 , Sarah Galdzicka 20 , Marshall Corson 21 , Crystal Watson 2 , Barbara A. Konkle , 20 , 21
12 June 2018
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.