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Coronary atherosclerosis and cardiovascular mortality in hemophilia

Although mortality in individuals with hemophilia is 2- to 3-fold higher than the general population, ischemic heart disease mortality is 50–80% lower than the general population [1–5]. Moreover, hemophilia carriers whose factor VIII levels are low, but not deficient, also appear to have low death rates of ischemic cardiac disease [6]. Elevated circulating factor VIII levels [7], as well as fibrinogen [8] and von Willebrand factor [9], are considered risk factors for cardiovascular disease. Yet, it is possible that low factor VIII levels may reduce the likelihood of clot formation, even in the presence of cardiovascular risk factors, such as smoking or elevated cholesterol, and, thereby, decrease coronary thrombosis and myocardial infarction in individuals with hemophilia.