title: Results of serial coronary angiography in patients with homozygous familial hypercholesterolaemia.
abstract: We studied the progression of coronary angiographic findings in a series of 10 patients with homozygous familial hypercholesterolaemia. All patients had medical treatment; six had additional surgical treatment. They were followed over a period of 2-10 years (mean follow-up five years). Three types of evolution were observed: (a) normal coronary angiography with no development of any lesion over a period of eight years in two young patients; (b) regression of proximal coronary stenoses in three patients, and (c) progression of coronary angiographic status in five patients. A different evolution of proximal and distal segment lesions was observed; while more than two-thirds of proximal segments stenoses improved and no proximal lesion increased with a 45% decrease in plasma cholesterol level, no improvement was obtained at the distal level. Distal lesions tended to increase and new lesions appeared in spite of a major plasma cholesterol level reduction. When early treatment results in a cholesterol plasma level decrease of more than 45% and a level of about 4 g l-1 over a very long period of time, the coronary prognosis may be good.
authors.affiliation: Department of Cardiology, CHU Pitié-Saltpétrière, Paris, France.
authors.forename: J M
authors.initials: JM
authors.lastname: Klein
authors.forename: G
authors.initials: G
authors.lastname: Drobinski
authors.forename: E
authors.initials: E
authors.lastname: Bruckert
authors.forename: F
authors.initials: F
authors.lastname: Dairou
authors.forename: D
authors.initials: D
authors.lastname: Thomas
authors.forename: J L
authors.initials: JL
authors.lastname: De Gennes
authors.forename: Y
authors.initials: Y
authors.lastname: Grosgogeat
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D002784:Cholesterol / Q000097:blood; D017023:Coronary Angiography; D003327:Coronary Disease / Q000000981:diagnostic imaging / Q000235:genetics*; D005260:Female; D005500:Follow-Up Studies; D006720:Homozygote; D006801:Humans; D006938:Hyperlipoproteinemia Type II / Q000000981:diagnostic imaging / Q000235:genetics* / Q000628:therapy; D008297:Male
chemical_list: D002784:Cholesterol