Systemic Amyloidosis Presenting with Angina Pectoris

7 December 1999 | Volume 131 Issue 11 | Pages 838-841

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 endothelialindependent 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.

Author and Article Information

From Mayo Clinic and Foundation, Rochester, Minnesota, and the National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, Maryland.

Acknowledgment: The authors thank W.D. Edwards, MD, Department of Anatomic Pathology, Mayo Clinic, for assistance in the interpretation of histologic slides.

Grant Support: By the Miami Heart Research Institute, the Bruce and Ruth Rappaport Vascular Biology Program, and Mayo Foundation.

Requests for Reprints: Amir Lerman, MD, Mayo Clinic, 200 First Street SW, Rochester, MN 55905; e-mail, lerman.amir{at}mayo.edu. For reprint orders in quantities exceeding 100, please contact the Reprints Coordinator; phone, 215-351-2657; e-mail, reprints{at}mail.acponline.org.

Current Author Addresses: Drs. Al Suwaidi, Velianou, Higano, Holmes, and Lerman: Division of Cardiovascular Diseases, Mayo Clinic and Foundation, 200 First Street SW, Rochester, MN 55905.

Dr. Gertz: Division of Hematology, Mayo Clinic and Foundation, 200 First Street SW, Rochester, MN 55905.

Dr. Cannon: Cardiology Branch, National Institutes of Health, National Heart, Lung, and Blood Institute, 10 Center Drive, Building 10, Room 7B15, Mail Stop MSC 1650, Bethesda, MD 20892.