Increased prevalence of coronary ectasia in heterozygous familial hypercholesterolemia

Krishnankutty Sudhir, Thomas A. Ports, Thomas M. Amidon, Jeffrey J. Goldberger, Vikas Bhushan, John P. Kane, Paul Yock, Mary J. Malloy

Research output: Contribution to journalArticlepeer-review

87 Scopus citations


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.

Original languageEnglish (US)
Pages (from-to)1375-1380
Number of pages6
Issue number5
StatePublished - Mar 1 1995
Externally publishedYes


  • aneurysm
  • angiography
  • hypercholesterolemia
  • lipoproteins
  • risk factors

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine
  • Physiology (medical)


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