Atherosclerotic Cardiovascular Risk in Adult Patients with Hemophilia: What We Can Do to Reduce Risk of Cardiovascular Events? Implementation of 2013 ACC/AHA Guideline on the Treatment of Blood Cholesterol in Patients with Hemophilia

Introduction

Hemophilia A and B are genetic disorders characterized by deficiency of clotting factors resulting in delayed bleeding. Despite hypocoagulable state, patients with hemophilia are prone to developing coronary artery disease or its equivalents. It is known that proper treatment of dyslipidemia has relevant impact of atherosclerotic cardiovascular events reduction. The goal of our study was to determine implementation of newest 2013 ACC/AHA Guideline on the Treatment of Blood Cholesterol in our patients with hemophilia and assess how many more patients currently may require lipid-lowering therapy.

Methods

We performed retrospective chart review of patients followed at single hemophilia treatment center in United States. We included 30 patients with factor VIII or IX deficiency, age 30 and older, followed in clinic between 2005 and 2014 with available lipid profile results. Patients with acquired hemophilia were excluded from study. We used stepwise approach proposed by above guidelines and divided patients into four groups.

Results

4 patients among 30 were already on lipid lowering therapy. 1 (3.3%) additional patient [95% CI 0.001-0.17] required lipid lowering therapy due to presence of clinical atherosclerotic cardiovascular disease (group 1), 0 patients had LDL-C at least 190 mg/dl (group 2), 2 (6.7%) additional patients [95% CI 0.008-0.21] required therapy due to presence of diabetes mellitus and 40 to 75 year of age and LDL-C levels of 70 to 189 mg/dl (group 3); 9 (30%) additional patients [95% CI 0.17-0.51] should receive therapy due to age 40 to 75 and estimated 10-year ASCVD risk above 7.5%. We had total 12 (40%) additional patients among 30 with known lipid profile who were not on lipid lowering therapy but who require such therapy based on the latest 2013 ACC/AHA Guideline on the Treatment of Blood Cholesterol.

Conclusion

Aggressive cardiovascular risk factor modifications play a significant role in prevention of coronary artery disease, stroke and peripheral vascular disease. This may be even more relevant in patients with hemophilia who have an increased baseline risk of bleeding and may therefore be at greater risk of complications from anti-thrombotic therapies used for treating cardiovascular disease. Above results suggest that according to actual 2013 ACC/AHA Guideline on the Treatment of Blood Cholesterol, a significant number of patients with hemophilia may require lipid lowering therapy. It is important for hemophilia treatment centers to screen their patients with regard to this since many of them may either not have primary care physicians or may not be perceived as having high risk for cardiovascular disease due to their bleeding disorder.