Table 3.

Primary and secondary endpoints

Primary endpointa

LV end-diastolic volume, increase > 10% (assessed with CMR)

LV ejection fraction, absolute decrease > 5% (assessed with CMR)

RV end-diastolic volume, increase > 10% (assessed with CMR)

RV ejection fraction, absolute decrease > 5% (assessed with CMR)

Late enhancement, absolute increase > 5% (assessed with CMR)

QRS voltage, decrease > 25% (ECG, measured in I, II and III in mV)

Occurrence of non-sustained ventricular tachycardia (Holter monitoring, exercise testing)

Heart failure signs and/or symptoms or arrhythmias necessitating medical treatment according to the guidelines and likely due to arrhythmogenic cardiomyopathy

Secondary endpoints

All individual components of the primary endpoint

Diagnosis of DCM (according to Mestroni criteria [19])

Diagnosis of ARVC (according to revised task force criteria [18])

Development of global or regional dysfunction and structural alterations (according to revised task force criterion 1 [18])

QRS axis (12-lead ECG)

Conduction intervals (PR interval, QRS duration [12-lead ECG; SA-ECG])

STT segment changes (12-lead ECG)

Change in biomarkers

Occurrence of sustained ventricular tachycardia or ventricular fibrillation

Hospitalisation for a cardiovascular reason

LV left ventricular, CMR cardiac magnetic resonance, RV right ventricular, ECG electrocardiogram, DCM dilated cardiomyopathy, ARVC arrhythmogenic right ventricular cardiomyopathy, SA-ECG signal-averaged electrocardiography

^aThe primary endpoint is a multiparametric assessment of disease progression defined as a responder-based composite primary endpoint. If one of the predefined parameters of the composite primary endpoint is reached, CMR imaging is performed and study end date recorded