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

Blood ◽  
2015 ◽  
Vol 126 (23) ◽  
pp. 4687-4687
Author(s):  
Kamila Izabela Cisak ◽  
Jianmin Pan ◽  
Shesh Nath Rai ◽  
Patricia Ashby ◽  
Vivek R. Sharma

Abstract 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. Disclosures No relevant conflicts of interest to declare.

Atherosclerosis: pathophysiology 212Development of atherosclerotic plaques 214Epidemiology 216Assessment of atherosclerotic risk 218Risk factors for coronary artery disease 220Hypertension 226Treatment of high blood pressure 228Combining antihypertensive drugs 230Lipid management in atherosclerosis 232Lipid-lowering therapy 236When to treat lipids ...


2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Mustafa Kinaan ◽  
Arelys Ramos Rivera ◽  
Hanford Yau

Abstract More than 70% of individuals with atherosclerotic cardiovascular disease are believed to have underlying gene-linked mechanisms leading to hyperlipidemia. It is estimated that 1 in 200 individuals in the United States has heterozygous Familial Hypercholesterolemia (FH). We present a case that highlights the importance of comprehensive care for a patient with heterozygous FH, from screening and risk stratification, to therapy. Our patient is a 43-year-old gentleman with history of hyperlipidemia. At age 25, he was diagnosed with hyperlipidemia and was started on statin therapy. He has strong family history of cardiovascular disease. His mother had her first myocardial infarction (MI) at age 40 and required coronary artery bypass. She also suffered from three strokes. His maternal aunt and uncle suffered from MIs at age 38 and 40, respectively. Additionally, his maternal grandfather passed away from MI at age 38. The patient’s daughter was found to have total cholesterol level > 300 mg/dL at age 8. He does not have history of obesity, diabetes, previous cardiovascular events, or hypothyroidism. He is athletic and follows a healthy diet. He did not have any xanthomas, xanthelasmas, nor arcus cornealis. At time of initial evaluation, the patient had low-density lipid (LDL) level of 180 mg/dL despite therapy with rosuvastatin, ezetimibe, and niacin. Based on these findings, we proceeded with genetic testing. Results of testing showed a heterozygous c.6delG (p.Trp4Glyfs*202) pathogenic mutation of the LDL receptor. We also obtained cardiovascular risk stratification studies. On cardiac CT angiogram, he was found to have extensive, four-vessel disease with 80-90% stenosis of the left ascending artery (LAD) with coronary calcium score of 136 and total score of 219 (99th percentile). Exercise, stress myocardial perfusion scan showed small reversible anteroseptal perfusion abnormality suggestive of mild to moderate ischemia. LAD stenosis was confirmed on a left heart catheter, but no intervention was required. We proceeded with aggressive lipid-lowering therapy with rosuvastatin 40mg daily and alirocumab 300mg monthly. He was also started on aspirin and beta-blocker given coronary artery disease. Following initiation of therapy, the patient’s LDL level dropped to 51 mg/dL with total cholesterol level of 153 mg/dL, HDL of 47mg/dL, and triglycerides of 109 mg/dL. The patient was encouraged to seek genetic counseling for his children and first degree relatives. His daughter was started on rosuvastatin 7.5mg daily by her pediatrician. The patient has not suffered any cardiovascular events and continues to follow up for therapy. Without aggressive lipid-lowering therapy, the lifespan of FH patients can be significantly shortened. Therefore, identifying FH patients is imperative to prevent cardiovascular disease in these patients and their afflicted family members.


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