Cardiac amyloidosis

Abstract

Objective: To analyze the characteristics of Cardiac Amyloidosis (CA). Bibliographic review: Cardiac amyloidosis results from circulating cytotoxic amyloid oligomers and amyloid fibrils that distort the structure of cardiac tissue, resulting in thickening and stiffening of the ventricular walls with heart failure and conductive dysfunction. There are two amyloidogenic proteins responsible for more than 95% of cardiac involvement: light chain amyloidosis (AL) and transthyretin amyloidosis (ATTR), which is divided into hereditary (ATTRm) or acquired (ATTRwt) forms. While AL results from the excessive production of antibody light chains that misfold and deposit in tissues, ATTR is caused by the extracellular deposition of misfolded transthyretin monomers. Final considerations: Cardiac amyloidosis is considered a rare but underdiagnosed cause of restrictive cardiomyopathy that, if left untreated, has a short survival for its two trigger proteins. In this sense, CA should be highly suspected in patients with HF with preserved ejection fraction, unexplained left ventricular hypertrophy and involvement of systemic organs, which can be diagnosed non-invasively by echocardiography, cardiac magnetic resonance or nuclear scintigraphy.