The Healthcare Amyloidosis European Registry (HEAR): Study design and methods

Abstract

Introduction

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. In order to gather more information on the diagnosis and management of CA, we created the Healthcare Amyloidosis European Registry (HEAR).

Objective

The registry's primary objective is to describe the demographic, clinical, biological and imaging characteristics of patients with CA. The secondary objectives are to (i) describe the different types of CA and their progression, (ii) describe the prevailing disease management procedures and any changes in these procedures, (iii) evaluate tools and quality of life questionnaires, (iv) describe the prognosis for patients with CA; (v) describe the management of CA by cardiologists, and (vi) assess hospital admissions and treatments and any changes in these factors. The HEAR will give us an opportunity to share good practice and to evaluate and optimize the quality of care for patients with CA.

Method

The HEAR is non-intervention, longitudinal, multicentre registry initiated in France, but which has been designed with a view to extension to other European countries. It includes prospective, retroprospective and retrospective cohorts of patients referred for suspected CA or with a confirmed diagnosis of CA.

Results

Since July 2021, 34 hospitals across France have joined the HEAR project. We expect to include 6500 patients in the HEAR between January 2021 and December 2027. At baseline, we use an electronic case report form to collect data on demographics, clinical, biological and imaging variables, the management of CA by cardiologists, specific treatments, quality of life, and diagnostic data. Lastly, we intend to collect in-hospital data on outcomes (deaths, cause of death, and hospital readmissions) annually.

Conclusion

The HEAR is the first nationally representative, internationally extendable registry dedicated to suspected and confirmed cases of CA. It will provide crucial information on the prevailing aetiologies, prevalences, and CA management practices.