LAUSR

Introduction Cardiac amyloidosis is an under recognized cause of cardiomyopathy leading to progressive heart failure from deposition of misfolded protein in the myocardial extracellular matrix. AL and ATTR amyloidosis are the main forms of cardiac amyloidosis accounting for >90% of cases in the U.S. Case Report A 57 year old woman with hypertension, bilateral carpal tunnel and recent diagnosis of heart failure was transferred for evaluation. She presented to her PCP three months prior with pedal edema. Echocardiogram at that time showed an LVEF of 55% and she was diagnosed with HFpEF. She was hospitalized twice for progressive symptoms; coronary angiography showed luminal irregularities intracardiac pressures were notable for RAP of 23 mmHg, RVP of 41/11 mmHg, PAP 42/25 mmHg, PCWP of 31 mmHg with CO of 3.2 L/min. MRI was recommended but not attained. On admission, ECG showed sinus tachycardia and low voltage. TTE showed biventricular hypertrophy with LVEF 40%, biatrial enlargement and thickened mitral valve with restrictive inflow pattern concerning for amyloidosis. UPEP demonstrated a monoclonal spike with serum lambda free light chains of 1721 mg/L and κ/λ ratio of Summary AL amyloidosis is a multiorgan disease in which one system usually predominates with cardiac involvement portending the worst prognosis. It is irreversible and can be rapidly fatal; early work-up and recognition is crucial as therapies exist.