Cardiac Amyloidosis: Mutimodality Imaging for Diagnosis and Prognosis

Abstract

Cardiac amyloid cardiomyopathy (CM) is a rapidly progressive disease that is frequently underrecognized and frequently diagnosed late in a significant number of individuals suffering from heart failure. Cardiac amyloid infiltration resulting in myocardial hypertrophy and restrictive CM is primarily caused by the misfolding of precursor proteins such as transthyretin, light chain immunoglobulin, and apolipoprotein AI-IV. The utilization of echocardiography, cardiac magnetic resonance, and bone avid radiotracer scintigraphy are essential in establishing a reliable diagnosis and prognosis of cardiac amyloidosis in the majority of patients. However, in certain clinical scenarios, the use of cardiac or extracardiac biopsy is necessary. There are significant developments in the understanding of the pathobiology of amyloid formation, which has paved the way for the development of new-targeted therapies, specifically for transthyretin cardiac amyloidosis. Imaging techniques are developing to monitor quantitatively the progression and regression of cardiac and systemic amyloid infiltration.

Keywords

• apical sparing
• free light chain assay
• heart failure with preserved ejection fraction
• immunofixation electrophoresis
• primary amyloidosis
• pyrophosphate scan
• restrictive cardiomyopathy
• transthyretin amyloidosis