A review of recent advances in the diagnosis of cardiac amyloidosis, treatment of its cardiac complications, and disease-modifying therapies

Abstract

Cardiac amyloidosis (CA), a significant condition resulting in infiltrative cardiomyopathy and heart failure with preserved ejection fraction (HFpEF), is caused by extracellular deposition of amyloid fibrils in the heart. Even though this has been known for an extended period, its prevalence in elderly patients with heart failure is increasingly being recognized. Recent advances in diagnosis with non-invasive methods like technetium pyrophosphate-labeled cardiac scintigraphy (i.e., Tc-PYP scan) and treatment options with tafamidis have played a pivotal role in awareness of the burden of this disease. Management of cardiac complications like heart failure, atrial arrhythmias, conduction block, ventricular arrhythmias, coronary artery disease, and aortic stenosis is now more critical than ever. We aim to review and outline the recent advances in diagnoses of CA. We also review management strategies for cardiac complications of CA with a brief summary of disease-modifying therapies.

Keywords: AL amyloidosis; ATTR amyloidosis; bone scintigraphy; cardiac amyloidosis; heart failure; tafamidis.