Prevalence and Risk Factors for Heart Disease Among
Males With Hemophilia
Roshni Kulkarni,1J. Michael Soucie,2*Bruce L. Evatt,2and the Hemophilia
Surveillance System Project Investigators
1Department of Pediatrics and Human Development, Michigan State University, East Lansing, Michigan
2Division of Hereditary Blood Disorders, National Center on Birth Defects and Developmental Disabilities,
Centers for Disease Control and Prevention, Atlanta, Georgia
There have been conflicting reports in the literature about the protective effect of hemo-
philia on the occurrence of ischemic heart disease (IHD). Circulatory disease has been
reported as the second most common cause of death in persons with hemophilia in the
United States. In addition to diabetes and hypertension, high levels of FVIII, as may occur
during factor concentrate infusions, may increase IHD risk in this population. To estimate
the prevalence of heart disease and examine factors associated with IHD and other heart
diseases among persons with hemophilia, we analyzed data collected from the medical
records of 3,422 males with hemophilia living in six U.S. states from 1993 to 1998. Heart
disease cases were ascertained from among 2,075 persons who were hospitalized at
least once during the 6-year period. Of these, 48 were diagnosed with IHD and 106, with
other types of heart disease. The age-specific prevalence of IHD ranged from 0.05% in
those under 30 years to 15.2% in those 60 years or older. Hospital discharge rates in
males with hemophilia with IHD and other types of heart disease were lower compared to
rates in age-matched U.S. males. In our cohort, as in the general population, IHD was
independently associated with age, hypertension, diabetes, and hyperlipidemia. Other
heart diseases were associated with HIV infection, hypertension, hemophilia B, and
diabetes. In summary, persons with hemophilia have unique risk factors such as infusion
of factor concentrates and infection with HIV that may predispose them to heart disease
as their life expectancy increases. Am. J. Hematol. 79:36–42, 2005.
ª 2005 Wiley-Liss, Inc.
words: hemophilia;coronary heartdisease; acutemyocardialinfarction;
Several European studies [1–3] and one U.S. study
 of cause-specific mortality have found lower rates
of death from ischemic heart disease in persons with
hemophilia compared to the general population.
These findings have been interpreted as providing
evidence that the hypocoagulable state offers protec-
tion from the thrombotic event that precipitates
infarction. An alternative explanation involving
lower prevalence of cardiovascular risk factors
among persons with hemophilia was examined in
a study by Rosendaal et al. who found that the dif-
ference in risk factors could account for only a frac-
tion of the decreased risk observed among Dutch
ª 2005 Wiley-Liss, Inc.
On the other hand, IHD and thrombosis in persons
with hemophilia are thought to be related to factor
VIII levels by some investigators [6,7]. Alsolaiman 
reported coronary artery occlusion during recombi-
nant FVIII infusion in a patient with hemophilia.
Coronary artery disease and myocardial infarction
are well-described complications in patients with
*Correspondence to: J. Michael Soucie, Ph.D., Centers for
Disease Control and Prevention, 1600 Clifton Road, MS E64,
Atlanta, GA 30333. E-mail: email@example.com
Received for publication 27 July 2004; Accepted 25 November
American Journal of Hematology 79:36–42 (2005)
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