SHM Converge Scientific Abstract ePoster Gallery-Rapidly Fatal Cardiac Amyloidosis: A Case of Rare Lambda Light Chain Al Amyloidosis

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This document describes a medical poster template and a case report of <strong>cardiac AL amyloidosis</strong>. The template instructions explain poster sizing, image quality requirements, and how to change PowerPoint color themes or print through Genigraphics.

The clinical case involves a <strong>70-year-old man</strong> who presented with fatigue and worsening exertional shortness of breath, initially diagnosed as heart failure exacerbation. Echocardiogram and cardiac MRI suggested amyloidosis. A <strong>PYP scan was negative</strong>, making transthyretin amyloidosis (ATTR) unlikely. Serum free light chain testing showed a <strong>lambda monoclonal gammopathy</strong> with markedly abnormal light chain values, and serum protein electrophoresis detected a monoclonal spike.

An <strong>endomyocardial biopsy</strong> confirmed cardiac amyloidosis, showing amyloid deposition with <strong>lambda light chain staining</strong> and no kappa or transthyretin expression. Bone marrow testing found a small population of plasma cells and lambda-restricted monoclonal plasma cells, supporting a diagnosis of <strong>lambda light chain AL amyloidosis</strong>.

The patient was started on treatment using the <strong>ANDROMEDA protocol</strong>: daratumumab, bortezomib, cyclophosphamide, and dexamethasone. Despite prompt therapy, he died of <strong>cardiac arrest 21 days after diagnosis</strong>, highlighting the severe prognosis of advanced cardiac AL amyloidosis.

The discussion emphasizes key imaging findings suggestive of cardiac amyloidosis, including <strong>biatrial enlargement, biventricular wall thickening, low-voltage ECG despite increased LV wall thickness, diastolic dysfunction, and preserved ejection fraction with low stroke volume</strong>.

Overall, the case underscores that cardiac amyloidosis should be considered in patients with heart failure and preserved ejection fraction, and that <strong>early diagnosis using imaging, serum markers, and biopsy is critical</strong> because delayed recognition often leads to irreversible damage and poor outcomes.

Asset Subtitle

Aliaa Hassanin

Meta Tag

Author List Aliaa Hassanin, Manisha Piryani, Mohammad Rahman

Category Clinical Vignettes

Concept Amyloidosis

Concept Cardiac Amyloidosis

Concept AL Amyloidosis

Concept Heart Failure with Preserved Ejection Fraction

Concept Biopsy

Distinguished Non-Finalist

Presenter Organization Trinity Health System

Presenting Author Aliaa Hassanin

Track Adult

Keywords

cardiac AL amyloidosis

lambda light chain

endomyocardial biopsy

PYP scan

monoclonal gammopathy

heart failure with preserved ejection fraction

cardiac MRI

daratumumab

bortezomib

biatrial enlargement

Amyloidosis

Cardiac Amyloidosis

AL Amyloidosis

Heart Failure with Preserved Ejection Fraction

Biopsy