Primary cardiac amyloidosis in a young man presenting with angina pectoris

A 32 year old man presenting with typical angina pectoris was found to have primary cardiac amyloidosis. Myocardial infiltration was strongly suggested by echocardiography, and a histological diagnosis was confirmed by renal biopsy. Nevertheless, technetium pyrophosphate myocardial scanning, recently proposed as a sensitive non-invasive test, showed negative results despite widespread cardiac involvement confirmed at necropsy after unexpected sudden death. Postmortem findings also showed subintimal amyloid material in a severely narrowed right coronary artery. This case highlights several unusual clinical features in a patient with advanced primary cardiac amyloidosis.