Unmasking Cardiac Amyloidosis: From Pathophysiology to Emerging Therapies

The spectrum of disease which is characterized by misfolded proteins that get deposited in extracellular tissues, (as an aggregate of fibrils called Amyloid fibrils) is called as Amyloidosis. Cardiac Amyloidosis, an aggregate of fibrils called Amyloid fibrils which gets deposited in heart consequently resulting into Cardiomyopathy which is “Infiltrative” Type and which is progressive in course, resulting, into wide spectrum of cardiovascular manifestations that range from Heart Failure, Arrhythmias, Ventricular arrhythmias to coronary vascular involvement. This Review Article analyzes and summarizes advancements in the field of pathology, physiology, genetics, Diagnostic modalities, Management strategies, and Novel Therapeutics. The increasing trend in diagnosis of transthyretin amyloidosis (ATTR) is due to improved diagnostic imaging and heightened clinical awareness. Development of noninvasive diagnostic tools, is playing a major role in identifying the cases in early course of disease. Endomyocardial biopsy, an invasive diagnostic approach in diagnosing the cases is a “A GOLDSTANDARD TEST”, however non-invasive tests often obviate the need for biopsy. Emerging Novel therapeutic agents like tafamidis, patisiran, vultisiran, and many more are transforming management and improving outcomes (both morbidity and mortality-related outcomes).