255 - Rapidly Progressive Heart Failure From Light Chain Cardiac Amyloidosis

Abstract

Introduction: Cardiac amyloidosis typically presents as a restrictive cardiomyopathy (CM) with slowly progressive heart failure (HF). Symptoms result from pulmonary edema, pleural effusions, and often right HF with peripheral edema. Echocardiographic findings include enlarged atria, diastolic dysfunction, and wall thickening due to amyloid deposition that typically results in low voltage on the ECG. Transthyretin cardiac amyloidosis (ATTR) and Light Chain (AL) cardiac amyloidosis are the most common forms; the latter results from a plasma cell dyscrasia. We present a case of rapidly progressive systolic HF from AL amyloid, treated with mechanical circulatory support (MCS) and chemotherapy. Case: A 74-year old male presented with recurrent hospitalizations for dyspnea. He had a history of diabetes, chronic kidney disease and rheumatoid arthritis. He had undergone stenting of an obtuse marginal branch 2 months ago when he presented with dyspnea and elevated troponin. A week later he had similar complaints and positive troponin with diffuse ST segment depressions on ECG. Repeat angiogram revealed non-obstructive coronaries. He then had 3 more admissions for HF within a month. His EF declined from 45% to 20% and he was in low output HF requiring an intra-aortic balloon pump (IABP) and inotropes. Right heart catheterization revealed elevated filling pressures. Endomyocardial biopsy showed extensive AL amyloid deposition (lambda) with replacement fibrosis. Serum kappa and lambda light chains were 2.5 and 38.4 mg/dl respectively, with a ratio of 0.0643 (normal 0.26–1.65). After a bone marrow biopsy showed a 3% clonal lambda population, he was started on cyclophosphamide, bortezomib and dexamethasone. A left ventricular assist device (LVAD) was placed to allow further chemotherapy and the possibility of cardiac recovery. The patient remains alive 6 months after LVAD implantation on bortezomib and dexamethasone. Discussion: Cardiac amyloidosis, especially TTR, usually presents gradually with restrictive HF. AL amyloid may have a more rapid symptom onset and progression. This is a rare case of AL amyloid presenting as rapidly progressive systolic HF requiring MCS, permitting chemotherapy and possibly remission. This is the first reported case of LVAD use in a patient with a diagnosis of AL amyloid prior to implantation. Conclusion: AL amyloid rarely manifests as a rapidly progressive dilated CM but should be considered especially in the presence of persistently elevated troponin and an abnormal free light chain ratio. While the overall prognosis is poor in patients with HF from AL amyloid, MCS may provide a bridge to remission and possible recovery.