Symptomatic ischemic heart disease resulting from obstructive intramural coronary amyloidosis

PURPOSE: Obstructive intramural coronary amyloidosis is an unusual complica tion of systemic amyloidosis. SUBJECTS AND METHODS: We review the characteristics of 11 patients seen at the Mayo Clinic (Rochester, Minnesota) from January 1, 1960, to June 1, 199 9, with intramural cardiac amyloidosis diagnosed at autopsy or after examin ation of an explanted heart. RESULTS: Symptomatic ischemic heart disease resulting from obstructive intr amural coronary amyloidosis was found in 11 patients (8 men, 3 women). The mean (+/-SD) age at the diagnosis of primary amyloidosis was 62 +/- 12 year s. All patients had angina pectoris; angina was the presenting symptom of p rimary amyloidosis in 6 patients. Unstable coronary syndromes occurred in 7 patients and congestive heart failure in 8. New electrocardiographic abnor malities after the development of angina were common and included ischemic changes, bundle branch block, and dysrhythmias. Low voltage was seen in onl y 2 patients. All 7 patients who underwent coronary angiography had normal or clinically insignificant findings. Endomyocardial biopsy was performed o n 4 patients; amyloid was found in 3 patients, none of whom had obstructive intramural coronary amyloidosis. The diagnosis of obstructive intramural c oronary amyloidosis with associated myocardial injury was established only at autopsy or after examination of the explanted heart after cardiac transp lantation. The mean time to death or cardiac transplantation after symptoms of cardiac ischemia developed was 18 +/- 20 months. CONCLUSIONS: The diagnosis of ischemic heart disease resulting from obstruc tive intramural coronary amyloidosis is difficult to establish before death or cardiac transplantation. Although the condition has a poor prognosis, i ts accurate recognition may have therapeutic implications, because some pat ients may benefit from treatment, including systemic chemotherapy or cardia c transplantation. (C) 2000 by Excerpta Medica, Inc.