Extent and severity of atherosclerotic involvement of the aortic valve and root in familial hypercholesterolaemia

Abstract

Conclusions-In patients with familial hypercholesterolaemia, aortic stenosis is common in homozygotes, and aortic root involvement is always present despite the lower CYS than in heterozygotes. It appears to be determined by short term exposure to high cholesterol concentrations in early life. Conversely, aortic root and valve involvement are rare in heterozygotes and occur only with severe, prolonged hypercholesterolaemia, possibly accelerating age related degenerative effects. (Heart 1998;80:583-590)

The Agenda for Familial Hypercholesterolemia

“…106 Transthoracic and transesophageal echocardiography can be used to quantify the severity of aortic valve and supra-aortic disease. 107 Magnetic resonance imaging can also be used as an alternative to CTA to study the aorta and to plan possible surgical interventions.…”

Effect of Lipid Lowering With Rosuvastatin on Progression of Aortic Stenosis

“…Indeed, valvular AS and supravalvular AS are known complications in patients with familial hypercholesterolemia, and sclerotic changes in the aortic valve are ubiquitous in homozygotes and common in heterozygotes. 27,28 It is thus a reasonable hypothesis that statins, which are effective in treating hypercholesterolemia and reducing coronary events, may also be effective in reducing the rate of progression of AS. Several retrospective studies have supported this notion.…”

Characteristics and Vascular Complications of Familial Hypercholesterolemia in Korea

“…Conversely, the rate of aortic valve surgery was 24% in homozygous FH patients 39) . Groups of researchers have demonstrated that cholesterol levels and duration after diagnosis of FH are associated with aortic stenosis 40,41) . However, the relation between cholesterol exposure and aortic stenosis…”