Comparison of electrocardiographic findings in patients with AL (primary) amyloidosis and in familial amyloid polyneuropathy and anginal pain and their relation to histopathologic findings

To assess the prevalence of chest pain and ischemic electrocardiographic (E CG) changes and relate them to histopathologic findings of coronary arterie s in cardiac amyloidosis, 33 patients with AL (primary) amyloidosis and 60 patients with familial amyloid polyneuropathy (FAP) were examined. Five pat ients (15%) with AL amyloidosis had recurrent anginal pain with exertion an d 2 of them also experienced anginal pain after orthostatic hypotension. Th e chest pain was associated with transient downsloping or horizontal ST-seg ment depression with or without T-wave inversion in right precordial leads, whereas the remaining patients with AL amyloidosis and all patients with F AP did not show anginal pain or ischemic ST-T changes. Histologic sections of coronary arteries were obtained in 12 patients with AL amyloidosis, incl uding 4 of the 5 patients who had angina pectoris and in 25 patients with F AP. Three patients with anginal pain had variable degrees of stenoses of th e intramural coronary arteries by amyloid deposition predominantly in the m edia with normal or nearly normal epicardial arteries. One patient with AL amyloidosis who had effort angina showed marked stenosis and complete occlu sion of the small coronary vessels by transmural amyloid deposition. The re maining 8 patients with AL amyloidosis and 25 with FAP without chest pain d id not exhibit any stenosis or occlusion of both the epicardial and intramu ral vessels. These findings suggest that ischemic ST-T changes with chest p ain are not so rare in patients with AL amyloidosis, and that markedly decr eased myocardial oxygen supply due to diffuse stenotic or occlusive disease of the small coronary vessels by amyloid deposition contributes to the dev elopment of clinically significant ischemic heart disease in these patients . (C)2000 by Excerpta Medica, Inc.