VASOSPASTIC ANGINA IN A PATIENT WITH FABRYS-DISEASE WHO SHOWED NORMALCORONARY ANGIOGRAPHIC FINDINGS

It has been reported that coronary diseases in patients with Fabry's d isease are induced by deposits in endothelial cells and coronary smoot h muscle cells. Most of those are ischemia due to stenosis. This repor t describes a case of a patient with Fabry's disease who showed severe vasospasms without coronary artery stenosis during acetylcholine load ed coronary angiography. In this case, a myocardial biopsy revealed th at the deposits in the endothelial cells of the myocardial capillaries were lamellated appearance. Recently, it is reported that endothelial cell damage could be an important cause of coronary vasospasm. This c ase suggests that the some sort of functional disorder was induced by glycosphingolipid deposits in the coronary endothelial cells, and that this might have led to coronary artery spasms without the organic ste nosis of coronary arteries.