Light Chain Cardiac Amyloidosis With Skeletal Muscle Weakness

Figures

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  Figure 1. MRI of Skeletal Muscle Magnetic resonance imaging (MRI) of skeletal muscle demonstrating symmetric short tau inversion recovery hypersensitivity involving proximal lower limb musculature, consistent with an inflammatory myopathic pattern (arrow).

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  Figure 2. 12-Lead Electrocardiogram 12-lead electrocardiogram demonstrating sinus rhythm with first-degree atrioventricular block, left anterior fascicular block, and low limb lead voltage.

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  Figure 3. Transthoracic Echocardiogram Transthoracic echocardiogram demonstrating parasternal long axis (top left) and apical 4-chamber (top-right) views. Global longitudinal strain with reduced strain (−15.9%) and relative apical sparing (so-called cherry on top pattern) is consistent with features of cardiac amyloidosis (bottom panel).

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  Figure 4. FDG-PET/CT Demonstrating Increased Myocardial Uptake Fluorodeoxyglucose positron emission tomography/computed tomography (FDG-PET/CT) demonstrates that axial cross-sectional views (left and middle panels) show uptake in the basal to mid-lateral and anterolateral segments. Sagittal view (right panel) demonstrates uptake in the basal septum.

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  Figure 5. Endomyocardial Biopsy Endomyocardial biopsy specimen demonstrating amyloidosis light chain deposition disease.

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  Figure 6. Trends in Lambda Free Light Chain Levels Trends in lambda free light chain levels from diagnosis through treatment (actual vs log10).

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  Figure 7. Serial Cardiac and Renal Biomarkers Serial cardiac (troponin I and N-terminal pro–B-type natriuretic peptide) and renal biomarkers (creatinine) over 12 months.