Framingham Score in Congenital Hemophilia Patients (HP) with Cardiovascular Disease (CVD).

Abstract

CVD risk factors of age, hypertension, high cholesterol (HC), smoking, diabetes are expressed in the Framingham coronary risk score. Previous data suggest that hemophilia may protect against CVD. However, CVD risk factors may place HP at risk as they age. HIV infected patients are a rapidly developing group with CVD. No data address the effect of established risk factors on CVD prevalence in HP. We report an extensive analysis of a single hemophilia treatment center (HTC) database for CVD risk factors and prevalence in HP. Framingham coronary risk scores were calculated for HP patients with documented CVD events, using age at time of event to calculate risk. HTC population ≥40 yrs, used as denominator, comprised 156 with FVIII deficiency (63 severe, 16 moderate, 77 mild);109 with FIX deficiency (24 severe, 53 moderate, 31 mild). Forty-eight HP had HIV infection, 113 had hepatitis C, and 25 had HIV/hepatitis C coinfection. Twenty-one HP, 10 with viral infection, ranging 49 to75 years, experienced 23 CVD events. They included 11 FVIII (0 severe, 1 moderate, 10 mild),10 FIX (2 severe,6 moderate, 2 mild). CVD events included 13 myocardial infarction (MI); 5 cerebrovascular accidents (CVA); 1 patient with carotid stenosis and 4 HP with coronary artery disease on catheterization. Two patients each had both MI and CVA. Four patients underwent coronary artery bypass graft surgery. Of 8 patients on antiplatelet therapy, 2 had major bleeds (CNS and GI bleed); one had a minor bleed (epistaxis). Of these 21 HP with CVD, one patient had HIV disease; 8 had hepatitis C, 1 had co-infection with HIV and hepatitis C. One patient was on Vioxx® at the time of his CVD event.Six are deceased, all due to CVD. Overall, CVD affected 7.9% of HP aged >40 years in this population. Of all HP with viral infection in this HTC population, 40% experienced CVD. Ten-year CVD risk per Framingham model at time of event ranged from 10% to 37% (mean 20.1%; median 16%). All HP patients with CVD met the definition of high coronary risk per AHA guidelines with 10-year risk of CVD>10%. Management of established CVD in HP is difficult due to need for invasive procedures and bleeding risk with antiplatelet therapy. Primary prevention of CVD is thus crucial. Aggressive risk factor modification is indicated in all HP with 10-year risk score >10% for primary prevention of CVD. To our knowledge, this is the first application of the well validated Framingham score in the HP population.We conclude that the easily calculable Framingham score should be assessed in all HP over age 40, especially those with viral infections, and incorporated into the comprehensive care model.