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Proposed diagnostic criteria for arrhythmogenic cardiomyopathy. European Task Force consensus report

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Item Type:Article
Title:Proposed diagnostic criteria for arrhythmogenic cardiomyopathy. European Task Force consensus report
Creators Name:Corrado, D. and Anastasakis, A. and Basso, C. and Bauce, B. and Blomström-Lundqvist, C. and Bucciarelli-Ducci, C. and Cipriani, A. and De Asmundis, C. and Gandjbakhch, E. and J Iménez-Jaimez, J. and Kharlap, M. and McKenna, W.J. and Monserrat, L. and Moon, J. and Pantazis, A. and Pelliccia, A. and Perazzolo Marra, M. and Pillichou, K. and Schulz-Menger, J. and Jurcut, R. and Seferovic, P. and Sharma, S. and Tfelt-Hansen, J. and Thiene, G. and Wichter, T. and Wilde, A. and Zorzi, A.
Abstract:Arrhythmogenic cardiomyopathy (ACM) is a heart muscle disease characterized by prominent "non-ischemic" myocardial scarring predisposing to ventricular electrical instability. Diagnostic criteria for the original phenotype, arrhythmogenic right ventricular cardiomyopathy (ARVC), were first proposed in 1994 and revised in 2010 by an international Task Force (TF). A 2019 International Expert report appraised these previous criteria, finding good accuracy for diagnosis of ARVC but a lack of sensitivity for identification of the expanding phenotypic disease spectrum, which includes left-sided variants, i.e., biventricular (ABVC) and arrhythmogenic left ventricular cardiomyopathy (ALVC). The ARVC phenotype together with these left-sided variants are now more appropriately named ACM. The lack of diagnostic criteria for the left ventricular (LV) phenotype has resulted in clinical under-recognition of ACM patients over the 4 decades since the disease discovery. In 2020, the "Padua criteria" were proposed for both right- and left-sided ACM phenotypes. The presently proposed criteria represent a refinement of the 2020 Padua criteria and have been developed by an expert European TF to improve the diagnosis of ACM with upgraded and internationally recognized criteria. The growing recognition of the diagnostic role of CMR has led to the incorporation of myocardial tissue characterization findings for detection of myocardial scar using the late‑gadolinium enhancement (LGE) technique to more fully characterize right, biventricular and left disease variants, whether genetic or acquired (phenocopies), and to exclude other "non-scarring" myocardial disease. The "ring-like' pattern of myocardial LGE/scar is now a recognized diagnostic hallmark of ALVC. Additional diagnostic criteria regarding LV depolarization and repolarization ECG abnormalities and ventricular arrhythmias of LV origin are also provided. These proposed upgrading of diagnostic criteria represents a working framework to improve management of ACM patients.
Keywords:Cardiac Magnetic Resonance, Cardiomyopathy, Diagnosis, Ventricular Arrhythmia, Sudden Death
Source:International Journal of Cardiology
Page Range:131447
Date:15 January 2024
Official Publication:https://doi.org/10.1016/j.ijcard.2023.131447
PubMed:View item in PubMed

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