Publication:

Unravelling the cardiac conundrum of amyloidosis: an experience from a tertiary care centre

Amyloidosis encompasses a spectrum of diseases caused due to extracellular deposition of misfolded proteins in body. The most common subtypes that infiltrate cardiac tissue are AL, ATTRwt (wild type), ATTRv (mutated) and Isolated atrial amyloidosis. Here, we elaborate such an interesting case of cardiac AL amyloidosis. A 69-year-old male presented with shortness of breath and restrictive cardiomyopathy. Further endomyocardial biopsy showed acellular, light eosinophilic deposit. Direct immunofluorescence showed lambda light chain restriction, concordant with elevated serum free λ light chain level. Finally, positive Congo red stain with apple green birefringence under polarised light clinched the diagnosis of Cardiac AL Amyloidosis. Endomyocardial biopsy with congo red stain and immunofluorescence is indispensable for diagnosis and subtyping of AL amyloidosis. This sets apart other amyloid subtypes and cardiac light chain deposition disease. Early diagnosis of cardiac amyloidosis subtype is imperative to guide the therapy and evaluate the prognosis of the patient.