CORONARY ANGIOGRAPHY AND INTRAVASCULAR UL TRASOUND EXAMINATION OF A 60-YEAR-OLD PATIENT WITH FAMILIAL HIGH-DENSITY-LIPOPROTEIN DEFICIENCY

A 60-year-old homozygous patient with familial high density lipoprotei n deficiency (Tangier disease) was examined by coronary angiography an d intravascular ultrasound because of typical angina pectoris. We foun d a normal left ventricular function, moderately diffuse coronary scle rosis without stenosis, and no critical stenosis of peripheral arterie s. Intravascular ultrasound revealed normal thickness and the three-la yer appearance of the arterial intima, media, and adventitia within th e peripheral arteries, and showed a single, discrete arteriosclerotic lesion in one iliac artery segment. The lack of severe atherosclerosis was remarkable insofar as massive foam cell formation in reticuloendo thelial tissues and the virtually complete absence of circulating HDL is characteristic of Tangier disease and had been previously demonstra ted in this patient.