An unexpected mortality following cardiac surgery: a post-mortem diagnosis of cardiac amyloidosis

Abstract

Amyloidosis is a rare systemic disease that is notoriously difficult to diagnose early. Cardiac involvement is associated with a poor prognosis. We present the case of a 72-year-old gentleman who underwent elective coronary artery bypass grafting and mitral valve repair. While initially making a good post-operative recovery, a sudden deterioration on post-operative day 3 led to an unexpected mortality. Post-mortem examination demonstrated previously undiagnosed severe systemic amyloidosis, which particularly affected his heart, due to underlying multiple myeloma. It is important to emphasise that due to the absence of the typical findings on routine pre-operative investigations suggestive of cardiac amyloidosis in this patient, cardiac surgeons should be aware that symptoms inconsistent with angiography findings should prompt consideration of amyloidosis. Diagnostic tools including cardiac MRI and ultimately endomyocardial biopsy could then facilitate earlier recognition of this enigmatic disease process.