ABSTRACT
Little information is available on clinical and morphologic aspects of coronary artery disease in patients with genetic syndromes characterized by HDL deficiency, and no reports are available describing cardiovascular findings at necropsy in patients with Tangier disease. For quantitative assessment of the degree of narrowing and evaluation of plaque composition in the coronary arteries and in saphenous venous grafts, the vessels were dissected from the heart, decalcified, and sectioned transversely at 5-mm intervals. On the basis of electron microscopic observations, it was not possible to distinguish extracellular lipid deposits in the atherosclerotic plaques specifically related to Tangier disease, that is, those containing masses of cholesterol, from those occurring as nonspecific components of ordinary atherosclerotic plaques. Analysis of the plaque composition revealed that fibrous tissue was the major component, a finding similar to those reported in patients without Tangier disease having fatal atherosclerotic coronary artery disease.
