title: Non-invasive detection of aortic and coronary atherosclerosis in homozygous familial hypercholesterolemia by 64 slice multi-detector row computed tomography angiography.
abstract: Homozygous familial hypercholesterolemia (HoFH) is a rare disorder characterized by the early onset of atherosclerosis, often at the ostia of coronary arteries. In this study we document for the first time that aortic and coronary atherosclerosis can be detected using 64 slice multiple detector row computed tomographic coronary angiography (CTCA). We studied five HoFH patients (three females, two males, mean age 19.8+/-2.9 years, age range 15-23 years, with a mean low density lipoprotein (LDL) cholesterol 618+/-211 mg/dL) using 64 slice CTCA. None of the patients showed evidence of ischemia with standard exercise testing. Calcified and mixed atherosclerotic plaques adjacent to or compromising the coronary artery ostia were found in all study subjects. Coronary plaques causing significant obstruction were found in one patient, who had previously undergone coronary artery bypass surgery and aortic valve replacement. Two other patients were noted to have non-obstructive calcified, mixed and non-calcified coronary artery plaques. Our data suggest that CTCA could be a useful non-invasive method for detection of early aortic and coronary atherosclerosis specifically affecting the coronary ostia in HoFH subjects.
authors: Lipid Clinic, Heart Institute (InCor), University of Sao Paulo Medical School Hospital, Sao Paulo, Brazil. rdsf@uol.com.br
authors: Raul D
authors: RD
authors: Santos
authors: Marcio H
authors: MH
authors: Miname
authors: Lilton R C
authors: LR
authors: Martinez
authors: Carlos E
authors: CE
authors: Rochitte
authors: Ana P M
authors: AP
authors: Chacra
authors: Edna R
authors: ER
authors: Nakandakare
authors: David
authors: D
authors: Chen
authors: Ernst J
authors: EJ
authors: Schaefer
mesh_terms: D000293:Adolescent; D000328:Adult; D001011:Aorta / Q000473:pathology; D001027:Aortography / Q000295:instrumentation / Q000379:methods*; D002114:Calcinosis / Q000000981:diagnostic imaging*; D017023:Coronary Angiography / Q000379:methods*; D003324:Coronary Artery Disease / Q000000981:diagnostic imaging*; D003331:Coronary Vessels / Q000473:pathology; D005260:Female; D006801:Humans; D006938:Hyperlipoproteinemia Type II / Q000150:complications / Q000000981:diagnostic imaging*; D007091:Image Processing, Computer-Assisted; D008297:Male; D036542:Tomography, Spiral Computed / Q000295:instrumentation / Q000379:methods*