Transthyretin-mediated amyloid cardiomyopathy (ATTR-CM) is a microangiopathy driven by the thromboinflammation of capillary beds and angiogenic revascularization dysregulation, according to a new study published in the Journal of the American Heart Association.
he authors hypothesized that in this model, elevated capillary permeability exposes vascular basement membrane components to misfolded TTR protein, which then drives aggregation and stabilizes amyloid fibrils. The resulting congestion within the basement membrane impairs proper revascularization, ultimately diminishing cardiac exertional capacity over time and culminating in heart failure.
“Testing this hypothesis could shed light on transthyretin amyloidosis cardiomyopathy disease progression as well as the nonpathologic functions of the transthyretin protein,” the authors said.