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Increased prevalence of coronary ectasia in heterozygous familial hypercholesterolemia
K Sudhir1, T A Ports, T M Amidon
1Cardiovascular Research Institute, University of California, San Francisco 94143.
Insights
Familial hypercholesterolemia (FH) is linked to a higher prevalence of coronary artery ectasia, a condition where arteries widen. Lower HDL cholesterol levels are strongly associated with this increased risk in FH patients.
Area of Science:
- Cardiology
- Genetics
- Lipidology
Background:
- Coronary atherosclerosis commonly causes stenosis, but aneurysmal disease also occurs.
- Familial hypercholesterolemia (FH) shows a higher prevalence of ectasia and aneurysms, suggesting a link with lipoproteins.
Purpose of the Study:
- To examine the prevalence of coronary ectasia in asymptomatic FH subjects.
- To investigate the association between ectasia and coronary risk factors in FH.
Main Methods:
- Quantitative coronary angiography was used to assess ectasia in 197 FH subjects and 198 controls.
- Ectasia was defined as a luminal diameter >1.5 times the adjacent segment.
- Associations with risk factors like HDL, LDL, and triglycerides were analyzed.
Main Results:
- Ectasia occurred in 15% of FH subjects versus 2.5% of controls (P < .001).
- Ectasia was more common in men and strongly associated with lower HDL and higher LDL/HDL ratio.
- FH patients with ectasia showed more severe atherosclerotic disease.
Conclusions:
- Coronary ectasia is more prevalent in FH patients compared to those with typical coronary atherosclerosis.
- Disordered lipoprotein metabolism in FH may predispose individuals to aneurysmal coronary artery disease.
Background:
Although coronary atherosclerosis most commonly produces clinical effects as a result of stenosis, aneurysmal disease also occurs. We have found an increased prevalence of ectasia and aneurysmal disease in familial hypercholesterolemia (FH) suggesting a link between plasma lipoproteins and coronary aneurysms.
Methods And Results:
In 197 asymptomatic subjects with FH, we examined the prevalence of ectasia and its association with coronary risk factors. An ectatic segment was defined as one with a luminal diameter > 1.5 times that of the adjacent normal segment, excluding poststenotic dilation. Among subjects with FH, 15% had ectasia compared with 2.5% of an age- and sex-matched control group of 198 subjects without FH presenting for coronary angiography (P < .001). These control patients had significantly more severe coronary atherosclerosis than patients with FH. Ectasia was 3 times more common in men than women (P < .025). Neither age nor hypertension was predictive. Although in part reflecting the striking sex differential, ectasia was strongly associated with a lower HDL cholesterol level (P = .003), a higher LDL/HDL ratio (P = .003), and to a lesser extent, a higher LDL cholesterol level (P = .07). No association was found with plasma triglycerides or very low-density lipoprotein cholesterol levels. Among FH patients, ectasia was strongly associated with an overall index of occlusive atherosclerotic disease, based on quantitative angiography (P = .004). Intracoronary ultrasound interrogation of aneurysmal segments revealed circumferential intimal thickening.
Conclusions:
Coronary ectasia is more prevalent in patients with FH than in other patients with coronary atherosclerosis and shows a strong inverse association with HDL cholesterol levels. This suggests that disordered lipoprotein metabolism in FH may predispose patients to aneurysmal coronary artery disease.