Arrhythmic Risk Prediction in ARVC | 165 INTRODUCTION Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited heart disease which predisposes patients to life-threatening ventricular arrhythmias (VA) and progressive cardiac failure.1 The disease is characterized by fibrofatty replacement and subsequent structural and functional alterations of primarily right ventricular (RV) myocardium. VA can occur already in an early stage of the disease, making it a leading cause of sudden cardiac death in especially the young and seemingly healthy.1,2 Consideration of an implantable cardioverter defibrillator (ICD) is a central component of clinical management of ARVC patients, but selecting the optimal candidates, especially for primary prevention of sudden cardiac death, has proven difficult.3 To support clinicians and patients in this process, a multimodality risk calculation tool was published in 20194 and its utility has been replicated in multiple independent cohorts.5–8 In addition, a recent study validated the risk calculator as an accurate tool to repeatedly calculate arrhythmic risk during follow-up.9 Visualisation of the structural disease substrate is an important part of arrhythmic risk stratification in ARVC. RV ejection fraction (RVEF), measured by cardiac magnetic resonance imaging (CMR), is currently the only imaging parameter included in the ARVC risk calculator. While global systolic function of the RV is well reflected in the EF, we know that life-threatening VA can already occur early in the disease. In this stage, fibrofatty replacement of myocardial tissue leads to regional wall motion abnormalities while the global systolic function is preserved. Echocardiographic deformation imaging has developed as a more sensitive method for detection of regional abnormalities in myocardial function. Regional deformation abnormalities have been associated to arrhythmia in ARVC patients, whereby especially negative predictive value of normal deformation was high.10–13 Although results of previous studies were promising, the added predictive value of echocardiographic deformation imaging should be tested in a multimodality approach and on top of current clinical practice. The purpose of this study was to test the predictive value of echocardiographic deformation imaging in a primary prevention cohort of patients with ARVC, and to further improve arrhythmic risk stratification by investigating whether it has prognostic value independent of the ARVC VA risk calculator. METHODS Study design and population We included consecutive patients with definite ARVC who presented to the University Medical Centre Utrecht in the Netherlands and the Oslo University Hospital in Norway up until January 1st 2023. These patients were partially included in previous studies.10,11,14,15 ARVC diagnosis was defined according to the 2010 Task Force Criteria (TFC).16 Patients with previous myocardial infarction or congenital heart disease were excluded. Date of inclusion was defined as the date of echocardiographic examination closest to date of diagnosis and available for performing deformation imaging. Patients with VA prior to baseline evaluation were excluded. Dutch patients were included in the UCC-UNRAVEL biobank for inherited heart diseases. The study was approved by both local institutional ethics review boards and complies with the Declaration of Helsinki. 8
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