An unusual case of systemic amyloid causing constrictive heart failure

Excerpt

A 74-year-old male was analysed for exertional dyspnoea in our outpatient clinic. Cardiac MRI showed diffuse enhancing pericardial soft-tissue (Fig. 1a), but no late gadolinium myocardial enhancement. PET-CT showed the pericardial and peri-renal soft-tissue mass to be calcified, however without FDG uptake. Surgical biopsy of the omentum revealed amyloid depositions fitting with a diagnosis of systemic amyloid. Systemic therapy was started, but the heart failure was progressive and not responding to medical therapy. On repeat cardiac MRI flattening of the ventricular septum during expiration was seen during free breathing (online movie 1). Postmortem macroscopic examinations of the heart and kidney showed extensive peri-renal and pericardial amyloid deposition (Fig. 1b).

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