Monitoring Disease Progression in Patients With Transthyretin Amyloid Cardiomyopathy
Pablo García-Pavía, Ronald Witteles, Thibaud Damy, Marianna Fontana, Giovanni Palladini, Kenichi Tsujita, Justin L. Grodin, Michel G. Khouri, Saurabh Malhotra, Vasvi Singh, Omar F. AbouEzzeddine, Joban Vaishnav, Mazen Hanna, Matthew J. Maurer
Abstract
Recognizing the lack of disease monitoring recommendations in transthyretin amyloid cardiomyopathy (ATTR-CM), international experts convened in 2021 to propose criteria for monitoring disease progression. Data have since been published demonstrating the prognostic value of certain parameters in ATTR-CM. Additionally, increased awareness and advances in diagnostic methods have led to a shift toward diagnosis at earlier stages of disease. In light of these developments, international experts with experience in treating ATTR-CM reviewed the available data, considered the feasibility of implementing evaluations in clinical practice, and proposed an update to the 2021 criteria. The criteria, with meaningful thresholds and monitoring frequency recommendations, are specifically designed to measure disease progression in patients with ATTR-CM, rather than to define progression of amyloid deposition. It remains unknown whether disease progression is an indicator for modifications to ATTR-CM treatment. Future studies should investigate whether changes in ATTR-CM disease-modifying treatment improve outcomes in patients demonstrating disease progression.