Systemic amyloidosis presenting with angina pectoris

Background: Manifestations of cardiac amyloidosis may include congestive he art failure and sudden cardiac death. Although vascular involvement in pati ents with amyloidosis is common, systemic amyloidosis presenting with angin a is rare. Objectives: To report on patients with systemic amyloidosis presenting with angina pectoris. Design: Case series. Setting: Academic medical center. Patients: Five patients who presented with angina pectoris and normal coron ary angiogram as the initial manifestation of systemic amyloidosis. Measurements: Endothelial-dependent and endothelialin-dependent coronary fl ow reserve. Results: All patients had coronary flow reserve abnormalities and subsequen tly developed congestive heart failure and systemic manifestations of amylo idosis. Histologic evaluation revealed amyloid deposition in the intramyoca rdial coronary vessels. Conclusions: Cardiac amyloidosis can present as angina pectoris associated with coronary flow reserve abnormalities despite normal coronary angiograms . This finding may have major therapeutic and prognostic implications in th is patient population.