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 endothelial-independent 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.