Cardiac Amyloidosis: The Forgotten Disease

Abstract

Cardiac amyloidosis leads to signs and symptoms of heart failure. A clinical condition when one of more than 30 different precursor proteins with unstable tertiary structure, misfolds and aggregates as insoluble amyloid fibrils which deposit in the extracellular space of organs and soft tissue. Among the many types of amyloidosis, nearly all cases of clinical cardiac amyloidosis (95 percent) are due to transthyretin amyloidosis (ATTR) or light chain amyloidosis. Studies suggest that wild-type transthyretin amyloidosis (ATTRw) is an underdiagnosed cause of heart failure, with a prevalence 
of 13 percent in a cohort of patients presenting with clinical manifestations of HF with preserved ejection fraction