INCREASED PREVALENCE OF CORONARY ECTASIA IN HETEROZYGOUS FAMILIAL HYPERCHOLESTEROLEMIA

Background Although coronary atherosclerosis most commonly produces cl inical effects as a result of stenosis, aneurysmal disease also occurs . We have found an increased prevalence of ectasia and aneurysmal dise ase in familial hypercholesterolemia (FH) suggesting a link between pl asma lipoproteins and coronary aneurysms. Methods and Results In 197 a symptomatic subjects with FH, we examined the prevalence of ectasia an d its association with coronary risk factors. An ectatic segment was d efined as one with a luminal diameter >1.5 times that of the adjacent normal segment, excluding poststenotic dilation. Among subjects with F H, 15% had ectasia compared with 2.5% of an age- and sex-matched contr ol group of 198 subjects without FH presenting for coronary angiograph y (P<.001). These control patients had significantly more severe coron ary atherosclerosis than patients with FH. Ectasia was 3 times more co mmon in men than women (P<.025). Neither age nor hypertension was pred ictive. Although in part reflecting the striking sex differential, ect asia was strongly associated with a lower HDL cholesterol level (P=.00 3), 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 tr iglycerides 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 ect asia 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 meta bolism in FH may predispose patients to aneurysmal coronary artery dis ease.