State of the Art of Cardiac Amyloidosis

Abstract

Cardiac amyloidosis is defined by extracellular deposition of misfolded proteins in the heart. The most frequent cases of cardiac amyloidosis are caused by transthyretin and light chain amyloidosis. This condition is underdiagnosed, and its incidence has been continuously rising in recent studies because of the aging of the population and the development of noninvasive multimodal diagnostic tools. Amyloid infiltration affects all cardiac tunics and causes heart failure with preserved ejection fraction, aortic stenosis, arrythmia, and conductive disorder. Innovative, specific therapeutic approaches have demonstrated an improvement in affected organs and the global survival of patients. This condition is no longer considered rare and incurable. Thus, better knowledge of the disease is mandatory. This review will provide a digest of the clinical signs and symptoms of cardiac amyloidosis, the diagnostic tools used to confirm the diagnosis, and current symptomatic and etiopathogenic management considerations according to guidelines and recommendations.

Time to Revisit—Cardiac Amyloidosis

“…Heart failure with preserved EF (HFpEF), aortic stenosis, conduction disorders, arrhythmias, and thrombotic phenomena are the major cardiac manifestations. 7 Table 1 shows the major cardiac and extracardiac manifestations of amyloidosis. Clinically heart failure, especially in the elderly that is poorly responding to conventional therapies, associated musculoskeletal/ neurological manifestations, proteinuria, nephrotic syndrome, and autonomic disturbances are highly suggestive to work up for CA.…”