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.