Pathophysiology of Cardiac Amyloidosis

KEY POINTS • Cardiac amyloidosis (CA) is a degenerative condition caused by progressive infiltration of misfolded proteins within myocardial extracellular space, which induces both mechanical and cytotoxic damage. • Several pathogenetic mechanisms have been identified, including increased oxidative stress, mitochondrial damage, apoptosis, impaired metabolism, and modifications of intracellular calcium balance. Cardiac fibrosis and inflammation have been proposed as potential contributors to cardiac remodeling. • The amyloidogenic cascade results in a restrictive cardiomyopathy characterized by biventricular thickening, diastolic and then systolic dysfunction, arrhythmias, and valvular disease.