The Healthcare Amyloidosis European Registry (HEAR): design of a national registry with a European extension strategy, and foundation of the F-CRIN GRACE network.

BACKGROUND

Cardiac amyloidosis (CA) is a rare disease that can lead to poor quality of life, conduction disorders, arrhythmia, heart failure, and even death. Fortunately, specific treatments that can modify the natural history of the disease and the disease outcomes are now available. However, data on the prevailing patient management procedures and long-term outcomes of CA are scarce.

OBJECTIVE

The Healthcare Amyloidosis European Registry (HEAR) is 34-centre registry initiated in France and structured for European expansion through the French Clinical Research Infrastructure Network's Group for Research on Amyloidosis and Care Excellence and the European Clinical Research Infrastructure Network. We expect to include 8500 patients between January 2021 and December 2027.

METHODS

The HEAR has been designed to capture detailed demographic, clinical, laboratory, imaging, and therapeutic data from both suspected and confirmed cases of all cardiac amyloidosis subtypes, including wildtype transthyretin amyloidosis, variant transthyretin amyloidosis, light-chain amyloidosis, and rarer forms. This comprehensive approach has been designed to (i) improve our understanding of real-world diagnostic pathways, treatment practices, and patient outcomes and (ii) incorporate patient-centred innovations. To enhance the patient-centred nature of the registry, patient-reported outcome measures (PROMs) will be systematically collected.

CONCLUSIONS

By addressing diagnostic pathways, real-world management and PROMs and by applying technological innovations and European scalability, the next-generation HEAR is establishing itself as a valuable resource for clinical research, public health interventions, and better patient care in the field of CA.