Systemic amyloidosis with cardiac involvement as the cause of death in an elderly female

Abstract

<h3>Background</h3> Amyloidosis is a well-recognised systemic disease entity. Patients with cardiac amyloidosis have a poor prognosis. We describe a case of death due to previously undiagnosed systemic amyloidosis with cardiac involvement in an elderly woman. <h3>Case report</h3> A 73-year-old female suffering from chronic renal failure and an undiagnosed heart condition was found dead at home. A coronal autopsy confirmed signs of right sided heart failure, revealing cardiomegaly with thickening of both ventricular walls and the septum. Microscopic examination showed severe amyloid deposit in the myocardium with associated myofibre atrophy. Amyloidosis involved the coronary arteries, liver, spleen, kidney, ovaries, adrenal and thyroid. Congo red stain showed apple-green birefringence under polarising light, which persisted after potassium permanganate treatment. The amyloidosis was undiagnosed during life and initially detected at autopsy. <h3>Conclusions</h3> This case illustrates the importance of microscopic examinations in the forensic setting. Amyloidosis may be undetected clinically during the course of unspecific cardiac presentations. Cardiac amyloidosis is a recognised cause of death. Amyloidosis should be considered within the differential diagnoses and actively investigated in elderly patients presenting with renal impairment in order to initiate appropriate management and to prevent unexpected cardiac death.