Cardiac amyloidosis: a review of the literature
Nils de Marneffe, R. Dulgheru, Arnaud Ancion, M. Moonen, Patrizio Lancellotti
Abstract
Cardiac amyloidosis is a rare disease associated with severe morbidity and mortality. There are three main types of amyloidosis associated with cardiac involvement: light chain (AL), familial or senile (ATTR) and secondary amyloidosis (AA). Cardiac amyloidosis often results in heart failure with preserved left ventricular ejection fraction, may display echocardiographic features of restrictive cardiomyopathy associated with left ventricular hypertrophy or mimic hypertrophic obstructive cardiomyopathy. However, left ventricular systolic dysfunction and normal wall thickness can sometimes be encountered. Imaging studies (echocardiography, bone scintigraphy, cardiac magnetic resonance) and blood and urine analysis are usually the main tools for the diagnosis. Sometimes, a tissue biopsy may be necessary. Treatment, which is constantly improving, will be carried out on two fronts: treatment of the symptoms and complications that the disease already caused and prevention of additional amyloid deposits while managing the concomitant complications. The purpose of this article is to review the management of cardiac amyloidosis.