Criteria for diagnosis of arrhythmogenic cardiomyopathy (ACM) were first proposed in 1994 andrevised in 2010 by an International Task Force. Although the International Task Force criteriademonstrated a good accuracy for diagnosis of the original right ventricular phenotype (arrhythmogenic right ventricular cardiomyopathy), they lacked sensitivity for identification of theexpanding phenotypic spectrum of ACM, which includes left-sided variants and did not incorporate late-gadolinium enhancement findings by cardiac magnetic resonance. The lack of specificdiagnostic criteria for the left ventricular phenotype of ACM has resulted in clinical under-recognition of patients with phenotypes other than the original ARVC over the 4 decades since thedisease discovery. The development of the 2020 upgrading of diagnostic criteria (“the Paduacriteria”) by International experts was based on the evolving clinical experience with the expanding spectrum of ACM phenotypes and needed to fill the diagnostic gap of the previous 1994 and2010 TF guidelines. The key upgrade was the incorporation of tissue characterization findingsby cardiac magnetic resonance for noninvasive detection of late-gadolinium enhancement/myocardial fibrosis that are determinants for characterization of arrhythmogenic biventricularand left ventricular phenotypes. and to provide a codification for future translational and clinicalresearch. The 2020 Padua criteria are heavily dependent on cardiac magnetic resonance, whichhas become mandatory to characterize the ACM phenotype and to exclude other diagnoses. Newcriteria regarding left ventricular depolarization and repolarization ECG abnormalities and ventricular arrhythmias of left ventricular origin were also provided. The 2020 Padua criteria aimedto improve the diagnosis of ACM by providing new criteria for diagnosis of biventricular and ALVCphenotypes, particularly by means of the incorporation of CMR tissue characterization findingsas well as LV depolarization and repolarization ECG abnormalities and ventricular arrhythmias.This new diagnostic approach needs to be validated by clinical studies on large patient populations. Preliminary data confirm that the clinical use of the Padua criteria substantially impactsthe diagnostic accuracy and permits a comprehensive identification of the phenotypic variety ofACM, mostly by virtue of demonstration of RV and LV LGE/myocardial fibrosis by CMR. The application of the upgraded diagnostic criteria in real-world clinical practice will be crucial for futurerefinement and correlation with therapeutic outcomes.
The aim of this Conference is to refine the “Padua criteria” and to reach a large internationalconsensus for the proposal of the 2022 European Task Force Criteria (“refined Padua criteria”),as a result of an interactive discussion between European panellists chosen for their expertise inthe basic science or clinical manifestations of the disease.
The conference will run over two full days and will consist of a series of short presentationsfollowed by interactive round table discussion focused on key aspects of the disease diagnosis,differential diagnosis with phenocopies and characterization of etiologic variants of the disease.
President of the Meeting
Prof. Domenico Corrado
Inherited Arrhythmogenic Cardiomyopathies
and Sports Cardiology Unit,
Department of Cardiac, Thoracic and Vascular
Sciences and Public Health
University of Padua Medical School,