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European Heart Journal 1988 9(10):1067-1073;
Copyright © 1988 by the European Society of Cardiology.
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© 1988 The European Society of Cardiology

Results of serial coronary angiography in patients with homozygous familial hypercholesterolaemia

J. M. KLEIN*, G. DROBINSKI*,, E. BRUCKERT{dagger}, F. DAIROU{dagger}, D. THOMAS* and J. L. DE GENNES{dagger}

*Department of Cardiology
{dagger}Department of Endocrinology, CHU Pitié-Saltpétrière 47 bl. de l'hôpital, 75651 Paris cedex 13, France

Received 26 November 1987; revised 15 March 1988; .

Address for correspondence. Dr G. Drobinski, Service de Cardiologie, CHU Pitié-Salpétrière, 47 bl. de l'hôpital, 75651 Paris cedex 13. France.

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.

Key Words: Coronary stenosis • homozygous familial hypercholesterolaemia • coronary heart disease


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