Pathway for the Diagnosis and Management of Cardiac Amyloidosis

Abstract

The systemic amyloidoses are a broad spectrum of diseases that result from misfolding of proteins that aggregate into amyloid fibrils. In cardiac amyloidosis, amyloid fibrils accumulate in the interstitial space between cardiac myocytes causing cellular injury and impairing compliance. Current data suggest that cardiac amyloidosis is more common than previously thought. Advances in cardiac imaging, diagnostic strategies, and therapies have improved the recognition and treatment of cardiac amyloidosis. A position statement for the diagnosis and treatment of cardiac amyloidosis has been published in 2021 by the European Society of Cardiology and an expert consensus decision pathway was published in 2023 by the American College of Cardiology. These are excellent documents but quite lengthy and complex. For this reason, our team developed a novel and simple pathway to help health care providers diagnose and treat patients with cardiac amyloidosis. Our pathway starts with a section titled "suspicion" in which we provide simple clues or "red flags" that are associated with the cardiac amyloidosis phenotype. It is followed by a section titled "diagnosis," where we present in a simplified 2 × 2 format the laboratory and imaging tests that must be performed for an accurate diagnosis. In the section titled "treatment," we describe the 4 pillars in the management of patients with cardiac amyloidosis, which includes the following: heart failure treatments, management of arrhythmias, treatment of significant aortic stenosis, and appropriate selection of disease modifying therapies. Our algorithm ends with our simplified recommendation for follow-up.