A Closer Evaluation for Refractory Atrial Fibrillation with Heart Failure, a Case Report

Abstract

<h3>Introduction</h3> Atrial fibrillation (AF) is highly prevalent after the age of 55 years. However, it can be associated with infiltrative cardiomyopathy. <h3>Case Report</h3> A 63 years old had new onset of palpitation and shortness of breath. He presented to ED with rapid AF at rate of 140 bpm treated with metoprolol, diltiazem and rivaroxiban. The patient started to develop lower limb swelling, dizziness, shortness of breath with walking short distance. His blood pressure was 110/60 mmHg off antihypertensive medication. He had NYHA class III symptoms and significant peripheral edema. A TTE showed a moderate concentric left ventricle hypertrophy IVS 16 mm with mild global systolic dysfunction LVEF 50% restrictive diastolic function and pericardial effusion. The patient had significant myocardial hypertrophy, HFpEF and AF with intolerance to medical therapy we proceeded with PYP bone scan which was equivocal for TTR amyloidosis. The bone to heart ration was 1.27. Cardiac MRI showed progressive decline in LVEF to 27% with severe RV systolic dysfunction EF 28% . There was a diffused subendocardial delayed enhanced lesion. Given these findings hematology workup sent which showed serum Free Lambda of 147mg/L; Free Kappa 6.71mg/L and Kappa/Lambda ratio of 0.05 (0.26 - 1.65). Bone marrow biopsy showed plasma cell percentage up to 40%. Flowcytometry showed lambda restricted plasma cell population together with positive CD38, CD138, CD56 and negative CD19. Fat Pad biopsy positive for Congo red stain consistent of amyloid deposits. He received chemotherapy CyBorDex 4 cycles followed by reduction in plasma cells to 2% in the bone marrow. He had cardiac function recovery. <h3>Summary</h3> AL cardiac amyloidosis can present with cardiac manifestation. High suspicion of underlying infiltrative disease should be investigated in refractor cases AF and HFpEF.