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Published on: March 28, 2017
A cross-sectional analysis of cardiovascular disease in the hemophilia population
Suman L Sood1, Dunlei Cheng2, Margaret Ragni3
1Department of Internal Medicine, University of Michigan, Ann Arbor, MI.
Insights
Men with hemophilia experience cardiovascular disease (CVD), despite earlier beliefs. This study found lower CVD rates in hemophilia patients compared to the general population, but prevention efforts remain crucial.
Area of Science:
- Cardiovascular Medicine
- Hematology
- Epidemiology
Background:
- Historically, men with hemophilia were considered protected from cardiovascular disease (CVD).
- Emerging evidence indicates that atherothrombotic events do occur in this population.
- Understanding CVD prevalence and risk factors in hemophilia is critical for patient care.
Purpose of the Study:
- To determine the prevalence of CVD and associated risk factors in US older men with moderate to severe hemophilia.
- To compare CVD rates in hemophilia patients with age-comparable men from the Atherosclerosis Risk in Communities (ARIC) cohort.
- To test the hypothesis that lower factor levels might be protective against CVD.
Main Methods:
- The CVD in Hemophilia study enrolled 200 men aged 54-73 with hemophilia A or B (factor levels ≤ 5%) from 19 US centers.
- Data collection included patient interviews, medical records, fasting blood samples, and electrocardiograms (ECGs).
- CVD was defined by specific clinical events including angina, myocardial infarction, stroke, transient ischemic attack, and revascularization procedures.
Main Results:
- CVD risk factors were prevalent among the study participants.
- Men with hemophilia showed significantly lower CVD prevalence (15%) compared to age-matched men in the ARIC cohort (25.8%; P < .001).
- Despite lower overall rates, individual CVD events occurred, highlighting the need for preventive strategies.
Conclusions:
- While hemophilia patients exhibit lower CVD rates than the general population, cardiovascular events still occur.
- Preventive measures for cardiovascular events are warranted in men with hemophilia.
- Few patients were receiving secondary prophylaxis with low-dose aspirin, indicating a potential gap in care despite its established safety.
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.
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