Key result
Severe cardiac AL amyloid light chains selectively impair ventricular relaxation while preserving contractile function.
Why the study?
Cardiac death in AL amyloidosis is usually linked to myocardial infiltration, but infiltration alone does not correlate with heart failure severity or survival, suggesting circulating light chains may directly impair cardiac function.
Does infusion of light chains from patients with severe cardiac AL amyloidosis impair cardiac function in isolated mouse hearts?
Population
Isolated Langendorff-perfused, isovolumically contracting mouse hearts
Comparison
Infusion of light chains from severe cardiac AL amyloidosis vs saline or light chains from nonamyloid, noncardiac, or mild cardiac AL amyloidosis
Design
Ex vivo infusion study using isolated mouse hearts
Authors
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Supports direct light chain toxicity in diastolic dysfunction; leaves open human relevance in AL amyloidosis.
Does infusion of light chains from patients with severe cardiac AL amyloidosis impair cardiac function in isolated mouse hearts?
Light chains from patients with severe cardiac AL amyloidosis directly cause diastolic dysfunction in isolated mouse hearts, suggesting a direct pathogenic role independent of fibril deposition.
Liao et al. (2001) studied Primary (AL) amyloidosis. Light chains (LC) from patients with severe cardiac involved AL amyloidosis vs. Saline, control LC, noncardiac LC, and mild-cardiac LC was evaluated on Diastolic and systolic cardiac function. Infusion of light chains from patients with severe cardiac AL amyloidosis caused marked impairment of ventricular relaxation with preserved contractile function in isolated mouse hearts.
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