Thelansis

  Systemic amyloidosis encompasses a group of diseases characterized by the deposition of misfolded fibrillar proteins in the extracellular matrix. Over 30 different precursor proteins can undergo substantial molecular transformations to form amyloid fibrils in vivo. Two primary types of amyloid commonly infiltrate the cardiac tissue: immunoglobulin light chain (AL) amyloidosis and transthyretin (ATTR) amyloidosis. In systemic amyloidosis, cardiac involvement is the primary cause of morbidity and mortality, irrespective of the specific mechanism underlying amyloid production. Both light chain amyloidosis (AL) and transthyretin amyloidosis (ATTR) can affect the heart, leading to symptoms such as heart failure, arrhythmias, and other extracardiac manifestations. AL amyloidosis is relatively rare, while ATTR has varying clinical penetrance and is associated with different variants. The pathophysiology involves the extracellular deposition of misfolded protein amyloid fibrils, compro...