Dexamethasone for the Treatment of Cardiac Al Amyloidosis: A Case Report and Literature Review

Abstract

Background: Systemic immunoglobulin light chain (AL) amyloidosis is a condition characterised by the conversion of light chains (LCs) from their soluble states into highly organised fibrillar aggregates that deposit in tissues, resulting in progressive organ damage and dysfunction. While previously considered incurable, treatment strategies are emerging for cardiac amyloidosis, underscoring the importance of early diagnosis. Case Summary: We present the case of a 69-year-old male patient who was admitted with acute onset of dyspnea and diagnosed with heart failure. The diagnosis of cardiac amyloidosis was made on the basis of a discrepancy between the results of the electrocardiogram and the echocardiogram, a conclusion that was further substantiated by the use of scintigraphy and myelogram. Given the poor left ventricular fraction, the standard chemotherapy regimen was replaced with corticosteroid therapy, which led to a significant improvement within the first 48 hours, unfortunately reversible with rapid clinical deterioration followed by sudden death within a week. Conclusion: Our aim is to highlight that early administration of dexamethasone is associated with significant clinical improvement but a high mortality rate in patients with AL cardiac amyloidosis.