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Published on: October 14, 2016
Obstructive intramural coronary amyloidosis and myocardial ischemia are common in primary amyloidosis
Michelle A Neben-Wittich1, Christopher M Wittich, Paul S Mueller
1Department of Internal Medicine, Mayo Clinic, Rochester, Minn 55905, USA.
Insights
Most patients with cardiac amyloidosis have obstructive intramural coronary amyloidosis, leading to myocardial ischemia. This condition can manifest as heart attacks or angina, sometimes being the first sign of primary amyloidosis.
Area of Science:
- Cardiology
- Pathology
- Amyloidosis Research
Background:
- Primary systemic amyloidosis frequently involves the heart.
- Cardiac involvement can lead to heart failure and arrhythmias.
- Coronary artery pathology in cardiac amyloidosis is not fully understood.
Purpose of the Study:
- To determine the prevalence of obstructive intramural coronary amyloidosis in patients with primary amyloidosis and cardiac involvement.
- To investigate the association between obstructive intramural coronary amyloidosis and myocardial ischemia.
- To identify clinical syndromes of myocardial ischemia in this patient population.
Main Methods:
- Retrospective review of medical records and pathologic specimens from 96 patients with primary amyloidosis and cardiac involvement.
- Analysis of autopsy or cardiac transplantation cases over a 20-year period.
- Examination for obstructive intramural coronary amyloidosis and microscopic ischemic changes.
Main Results:
- Obstructive intramural coronary amyloidosis was found in 66% of patients.
- Microscopic myocardial ischemia was significantly more common in patients with obstructive intramural coronary amyloidosis (86%) versus without (52%).
- Syndromes of myocardial ischemia occurred in 25% of patients with obstructive intramural coronary amyloidosis, sometimes as the initial presentation of primary amyloidosis.
Conclusions:
- Obstructive intramural coronary amyloidosis is highly prevalent in patients with primary systemic amyloidosis and cardiac involvement.
- Associated microscopic myocardial ischemia is common.
- Clinical syndromes of myocardial ischemia are a recognized complication.
Purpose:
The purpose of this study was to determine the prevalence of clinical syndromes and pathologic changes of myocardial ischemia due to obstructive intramural coronary amyloidosis among patients with primary amyloidosis and cardiac involvement.
Subjects And Methods:
Medical records and pathologic specimens were reviewed from 96 patients with primary amyloidosis and cardiac involvement at autopsy or after cardiac transplantation during a 20-year period. Medical records were reviewed for patient demographic and clinical characteristics, including evidence for syndromes of myocardial ischemia. Pathologic specimens were examined for obstructive intramural coronary amyloidosis and microscopic changes of myocardial ischemia.
Results:
Obstructive intramural coronary amyloidosis was present in 63 of 96 patients (66%). Microscopic changes of myocardial ischemia were more common in patients with obstructive intramural coronary amyloidosis (86%) than in those without (52%) (P <.001). In the 76 patients without coexistent severe epicardial coronary atherosclerosis, changes of myocardial ischemia were more common in those with obstructive intramural coronary amyloidosis (83%) than in those without (45%) (P <.001). In patients who had tissue available for review, none had obstruction of epicardial coronary arteries from amyloid. Syndromes of myocardial ischemia affected 16 patients (25%) with obstructive intramural coronary amyloidosis but only 2 patients (6%) without (P=.027). For 11% of the patients with obstructive intramural coronary amyloidosis, a syndrome of myocardial ischemia consisting of acute myocardial infarction or angina pectoris was the first manifestation of primary amyloidosis.
Conclusion:
Most patients with primary systemic amyloidosis and cardiac involvement have obstructive intramural coronary amyloidosis and associated microscopic changes of myocardial ischemia. Syndromes of myocardial ischemia may occur in these patients.
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