Clinical study
Symptomatic ischemic heart disease resulting from obstructive intramural coronary amyloidosis

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Abstract

PURPOSE: Obstructive intramural coronary amyloidosis is an unusual complication 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, 1999, with intramural cardiac amyloidosis diagnosed at autopsy or after examination of an explanted heart.

RESULTS: Symptomatic ischemic heart disease resulting from obstructive intramural coronary amyloidosis was found in 11 patients (8 men, 3 women). The mean (±SD) age at the diagnosis of primary amyloidosis was 62 ± 12 years. All patients had angina pectoris; angina was the presenting symptom of primary amyloidosis in 6 patients. Unstable coronary syndromes occurred in 7 patients and congestive heart failure in 8. New electrocardiographic abnormalities after the development of angina were common and included ischemic changes, bundle branch block, and dysrhythmias. Low voltage was seen in only 2 patients. All 7 patients who underwent coronary angiography had normal or clinically insignificant findings. Endomyocardial biopsy was performed on 4 patients; amyloid was found in 3 patients, none of whom had obstructive intramural coronary amyloidosis. The diagnosis of obstructive intramural coronary amyloidosis with associated myocardial injury was established only at autopsy or after examination of the explanted heart after cardiac transplantation. 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 obstructive intramural coronary amyloidosis is difficult to establish before death or cardiac transplantation. Although the condition has a poor prognosis, its accurate recognition may have therapeutic implications, because some patients may benefit from treatment, including systemic chemotherapy or cardiac transplantation.

Section snippets

Material and methods

A computerized search of the medical records of all patients seen at the Mayo Clinic in Rochester, Minnesota, with the diagnoses of primary amyloidosis and angina pectoris, myocardial infarction, or other diagnosis suggestive of ischemic heart disease was done for the period from January 1, 1960, to June 1, 1999. Permission to perform a retrospective review of the medical records was granted by the Institutional Review Board of the Mayo Foundation in compliance with federal regulations. Of the

Results

The demographic, clinical, and pathologic features of the 11 patients (8 men, 3 women) are found in Table 1, Table 2, Table 3.

ECG Changes after Onset of Symptoms of IschemiaFunctional StudyCoronary AngiogramEchocardiogramTime from Onset of Ischemic Symptoms to Death or Transplant (Months)Clinical Cause of Death
Left bundle branch block, left ventricular hypertrophy, increased voltage, ventricular tachycardiaNormalLeft ventricular ejection fraction 47%, thick septum, new infarction8Amyloid

Discussion

A MEDLINE search (1966 to the present), using the key words “amyloidosis,” “angina pectoris,” “cardiomyopathy,” “ischemic heart disease,” and “nonatherosclerotic coronary artery disease,” was conducted to identify published cases of symptomatic heart disease resulting from obstructive intramural coronary amyloidosis. The references of acquired articles were searched. We identified 11 additional cases 8, 9, 10, 11, 12, 13, 14, 15, 16 using the same criteria as in our series. Other reports 2, 17,

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