Arrhythmogenic Right Ventricular Cardiomyopathy: Improvement of Diagnosis and Risk Stratification by Implementation of Echocardiographic Normative Values in Children.

Abstract

A recent review on arrhythmogenic right ventricular cardiomyopathy (ARVC) in pediatric 28 patients highlights the diagnostic challenge and its´ important therapeutic consequences in this 29 rare disease [1]. ARVC leads to fibro-fatty infiltration of predominantly the right ventricular 30 (RV) myocardium and typically manifests beyond 10 years of age, causing arrhythmias and 31 sudden cardiac death. For diagnosis, the "Task Force Criteria" [2] are used, defining major 32 and minor criteria from 2D echocardiography, cardiac magnetic resonance imaging 33 (MRI), tissue characterization, electrocardiogram (ECG), RV angiography, arrhythmias, and 34 family history. Echo and MRI criteria include right ventricular outflow tract (RVOT) 35 dimension and RV function parameters. However, these "Task Force Criteria" were designed 36 for patients above 14 years of age, and do not incorporate pediatric normative values. RVOT 37 dimensions for example, are assessed as mm/m² body surface area (BSA) derived from the 38 parasternal short (PSAX) and parasternal long axis (PLAX). For pediatric patients the cut-off 39 values for RVOT dimensions included in the "Task Force Criteria" are not appropriate. 40However, normative values for the RV size and for RVOT dimensions in children exist [3,4]. 41