Accelerated subclinical coronary atherosclerosis in patients with familial hypercholesterolemia.
OBJECTIVE
We determined the extent, severity, distribution and type of coronary plaques in cardiac asymptomatic patients with familial hypercholesterolemia (FH) using computed tomography (CT).


BACKGROUND
FH patients have accelerated progression of coronary artery disease (CAD) with earlier major adverse cardiac events. Non-invasive CT coronary angiography (CTCA) allows assessing the coronary plaque burden in asymptomatic patients with FH.


METHODS
A total of 140 asymptomatic statin treated FH patients (90 men; mean age 52 ± 8 years) underwent CT calcium scoring (Agatston) and CTCA using a Dual Source CT scanner with a clinical follow-up of 29 ± 8 months. The extent, severity (obstructive or non-obstructive plaque based on >50% or <50% lumen diameter reduction), distribution and type (calcified, non-calcified, or mixed) of coronary plaque were evaluated.


RESULTS
The calcium score was 0 in 28 (21%) of the patients. In 16% of the patients there was no CT-evidence of any CAD while 24% had obstructive disease. In total 775 plaques were detected with CT coronary angiography, of which 11% were obstructive. Fifty four percent of all plaques were calcified, 25% non-calcified and 21% mixed. The CAD extent was related to gender, treated HDL-cholesterol and treated LDL-cholesterol levels. There was a low incidence of cardiac events and no cardiac death occurred during follow-up.


CONCLUSIONS
Development of CAD is accelerated in intensively treated male and female FH patients. The extent of CAD is related to gender and cholesterol levels and ranges from absence of plaque in one out of 6 patients to extensive CAD with plaque causing >50% lumen obstruction in almost a quarter of patients with FH.
Department of Radiology, Erasmus Medical Center, Rotterdam, The Netherlands. l.neefjes@erasmusmc.nl
Lisan A
LA
Neefjes
Gert-Jan R
GJ
Ten Kate
Rossi
R
Alexia
Koen
K
Nieman
Annette J
AJ
Galema-Boers
Janneke G
JG
Langendonk
Annick C
AC
Weustink
Nico R
NR
Mollet
Eric J
EJ
Sijbrands
Gabriel P
GP
Krestin
Pim J
PJ
de Feyter
D058070:Asymptomatic Diseases; D017023:Coronary Angiography / Q000379:methods; D003324:Coronary Artery Disease / Q000000981:diagnostic imaging / Q000235:genetics*; D018450:Disease Progression; D005260:Female; D020022:Genetic Predisposition to Disease; D006801:Humans; D019161:Hydroxymethylglutaryl-CoA Reductase Inhibitors / Q000627:therapeutic use; D006938:Hyperlipoproteinemia Type II / Q000150:complications / Q000188:drug therapy / Q000235:genetics*; D016014:Linear Models; D008297:Male; D008875:Middle Aged; D015999:Multivariate Analysis; D010641:Phenotype; D058226:Plaque, Atherosclerotic / Q000000981:diagnostic imaging / Q000235:genetics*; D011446:Prospective Studies; D018570:Risk Assessment; D012307:Risk Factors; D012720:Severity of Illness Index; D014057:Tomography, X-Ray Computed; D061205:Vascular Calcification / Q000000981:diagnostic imaging / Q000235:genetics*
D019161:Hydroxymethylglutaryl-CoA Reductase Inhibitors