See also Makris M, van Veen JJ. Reduced cardiovascular mortality in hemophilia despite normal atherosclerotic load. This issue, pp 20–2; Biere-Rafi S, Tuinenburg A, Haak BW, Peters M, Huijgen R, de Groot E, Verhamme P, Peerlinck K, Visseren FLJ, Kruip MJHA, Laros-van Gorkom BAP, Gerdes VEA, Buller HR, Schutgens REG, Kamphuisen PW. Factor VIII deficiency does not protect against atherosclerosis. This issue, pp 30–7.
Summary. Background/objectives: The traditional view that patients with hemophilia are protected against cardiovascular disease is under debate. The aim of the present study was to evaluate the presence and extent of atherosclerosis by coronary artery calcification score (CACS) and carotid intima media thickness (IMT) in patients with hemophilia, and to evaluate their cardiovascular risk profile. Methods: Sixty-nine patients (51 with hemophilia A; 18 with hemophilia B) were studied [median age: 52 years (interquartile range [IQR] 43–64)]. Cardiovascular risk factors and prior major adverse cardiovascular events (MACEs) were recorded. CACS was derived from electron-beam or dual-source computed tomography, and carotid IMT was assessed by ultrasound measurements and compared with age-specific reference values. Results: The median CACS in all patients was 35 (IQR 0–110) and the geometric mean IMT was 0.80 mm (95% confidence interval [CI] 0.76–0.84); neither was different from the reference values. Patients with a previous MACE (n = 9) had significantly higher CACS and IMT than patients without a previous MACE: CACS median 1013 (IQR 530–1306) vs. 0 (IQR 0–67), and IMT geometric mean 1.09 mm (95% CI 0.95–1.26) vs. 0.76 mm (95% CI 0.73–0.79), both P < 0.001. A higher calculated 10-year cardiovascular risk was related to higher IMT and CACS. Conclusion: Patients with hemophilia are not protected against the development of atherosclerosis as measured by CACS and IMT. The extent of atherosclerosis is related to the traditional cardiovascular risk factors. This suggests that traditional cardiovascular risk factors should be monitored and treated in patients with hemophilia.