Abstract 19170: Coronary Artery Calcium Score in Patients With Heterozygous Familial Hypercholesterolemia is Proportional to Lifetime LDL Burden After up to 28 Years of Treatment
Heterozygous familial hypercholesterolemia (FH) is associated with severe hypercholesterolemia and early onset of CVD. Prior studies have demonstrated elevated coronary artery calcium deposition in FH. Initiation of treatment as early as age 8-12 years is presumed to help prevent cardiovascular complications.
Hypothesis: Long-term LDL-lowering treatment will reduce accumulation of coronary artery calcium (CAC) in FH.
Methods: Cross-sectional analysis of data from 28 patients with heterozygous FH and no prior diagnosis of CVD who had a CAC score measurement.
Results: Twenty-eight subjects treated in our University Lipid Disorders Clinic for 0-28 years were studied (current age 58 +/- 11 years, 18 women, pre-treatment LDL cholesterol (C) 245 +/- 93, lipoprotein(a) 72 +/- 104, current CAC score 210 +/- 464, CAC percentile 55 +/- 38). The cumulative lifetime LDL burden was proportional to the age treatment was initiated (r=0.47, P=0.013). The CAC score was proportional to age (r=0.52, P=0.026) and cumulative LDL burden (r=0.71, r=0.0016), and was higher in men vs women (442 +/- 721 vs 81 +/- 129, P=0.046). HDL-C, triglycerides, and lp(a) were not correlated with CAC scores. Nine patients treated > 15 years had an achieved LDL-C 105 +/- 40 compared to 202 +/- in 11 patients treated < 1 year. The long term group initiated aggressive treatment at an average age 21 years younger than the short term group (mean age 38 +/- 15 [range 20-67] vs 59 +/- 11 [range 43-71]; P=0.024). Despite these differences, many long-term patients had elevated CAC scores (33% > 400) after up to 28 years of treatment.
Summary: CAC scores, which are a marker for atherosclerotic plaque accumulation (but calcium may be absent in about 50% of plaques in patients with FH) are proportional to age and lifetime LDL burden in patients with FH, but elevated CAC scores are prevalent even after long-term aggressive LDL-lowering therapy beginning at a mean age of 38 years.
Conclusions: These findings indicate that patients with heterozygous familial hypercholesterolemia warrant aggressive LDL lowering initiated at a young age.
- © 2013 by American Heart Association, Inc.