The Dutch cardiomyopathy registry (DCR); rationale and registry design

Cardiomyopathies are important causes of arrhythmias, sudden cardiac death, and heart failure. With accumulating knowledge in clinical, genetic, and molecular phenotyping, the number of distinct disease entities is growing. There is a need for broad, inclusive, high-quality prospective multi-center registries to better analyze these phenotypes, provide accurate risk stratification, and tailor therapies. The Dutch Cardiomyopathy Registry (DCR) will serve as a longitudinal registry for all cardiomyopathy patients, exploiting previously built local databases, allowing adequately powered research studies.

The DCR is a multi-center observational registry that unites all Dutch University Medical centers. The registry will have local and central shared databases, which will be hosted and coordinated by the Netherlands Heart Institute. Patients with an established cardiomyopathy diagnosis and carriers of pathogenic or likely-pathogenic cardiomyopathy gene variants will be included. Furthermore, selected family members and individuals who are referred for screening for potential cardiomyopathy are eligible for inclusion. The subjects will receive, per best clinical practice, guideline-recommended diagnostics and treatment. Clinical data will be collected from clinical care at baseline and follow-up, including clinical visits, imaging studies, laboratory assessments, ECG, and additional functional tests. Outcome data consists of clinical performance status, cardiovascular complications and major adverse events, recorded through regular electronic health record files.

The DCR will function as a national database, providing relevant information on epidemiology, demographics, natural history, diagnosis, and treatment. This will aid risk prediction and monitoring of new therapies. The DCR can identify patients who are eligible for future studies, including registry-based research.