Cardiac Amyloidosis

Abstract

Cardiac amyloidosis is a protein-folding disorder mostly caused by abnormal deposition of either transthyretin proteins or light chain (AL) proteins, into one or more organs, including the heart. The main cardiac manifestations are right ventricular heart failure and arrhythmias. Extracardiac symptoms usually precede cardiac symptoms and are evident several years before the development of symptomatic cardiac problems. The prognosis is poor without appropriate management. Non-invasive evaluation with multi-imaging modalities has allowed earlier diagnosis, particularly when used in combination with monoclonal gammopathy evaluation. Management will vary depending on the subtype of amyloidosis. It consists of supportive treatment of cardiac-related symptoms, pharmacological treatment that targets amyloid fibrils formation and deposition, thus attacking the underlying disease, and addressing the management of extracardiac symptoms to improve the patients’ quality of life.