Systemic amyloidosis presenting with angina pectoris

Background: Manifestations of cardiac amyloidosis may include congestive heart failure and sudden cardiac death. Although vascular involvement in patients with amyloidosis is common, systemic amyloidosis presenting with angina 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 coronary angiogram as the initial manifestation of systemic amyloidosis. Measurements: Endothelial-dependent and endothelialin-dependent coronary flow reserve. Results: All patients had coronary flow reserve abnormalities and subsequently developed congestive heart failure and systemic manifestations of amyloidosis. Histologic evaluation revealed amyloid deposition in the intramyocardial 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 this patient population.