FOCUS on Cardiac Amyloidosis

Cardiac amyloidosis is a progressive disease caused by the extracellular deposition of amyloid fibrils in the heart. More frequent than previously believed, it contributes to many common clinical scenarios such as severe aortic stenosis, increased left ventricular wall thickness and heart failure with preserved ejection fraction. Today, we know that the most frequent subtype of cardiac amyloidosis in clinical practice is transthyretin amyloidosis (ATTR-CA) which is caused by transthyretin deposition. This subtype has an acquired or wild type (ATTRwt-CA) and a hereditary (ATTRv-CA) form. Most patients with ATTR-CA can be diagnosed without biopsies, simply by performing a scintigraphy and evaluating the presence of a monoclonal protein in the blood and urine. The prognosis of patients with ATTR-CA has improved in recent years, due to earlier diagnosis, closer follow-up and the development of specific therapies that target the amyloidogenic cascade. This month’s FOCUS resources are all about cardiac amyloidosis. Build your knowledge about diagnosis, therapies and what we’ve learned from recent clinical trials.