TY - JOUR

T1 - Proposed diagnostic criteria for arrhythmogenic cardiomyopathy

T2 - European Task Force consensus report

AU - Corrado, Domenico

AU - Anastasakis, Aris

AU - Basso, Cristina

AU - Bauce, Barbara

AU - Blomström-Lundqvist, Carina

AU - Bucciarelli-Ducci, Chiara

AU - Cipriani, Alberto

AU - De Asmundis, Carlo

AU - Gandjbakhch, Estelle

AU - Jiménez-Jáimez, Juan

AU - Kharlap, Maria

AU - McKenna, William J.

AU - Monserrat, Lorenzo

AU - Moon, James

AU - Pantazis, Antonis

AU - Pelliccia, Antonio

AU - Perazzolo Marra, Martina

AU - Pillichou, Kalliopi

AU - Schulz-Menger, Jeanette

AU - Jurcut, Ruxandra

AU - Seferovic, Petar

AU - Sharma, Sanjay

AU - Tfelt-Hansen, Jacob

AU - Thiene, Gaetano

AU - Wichter, Thomas

AU - Wilde, Arthur

AU - Zorzi, Alessandro

N1 - Publisher Copyright: © 2023 The Author(s)

PY - 2024

Y1 - 2024

N2 - 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.

AB - 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.

KW - Cardiac magnetic resonance

KW - Cardiomyopathy

KW - Diagnosis

KW - Sudden death

KW - Ventricular arrhythmia

U2 - 10.1016/j.ijcard.2023.131447

DO - 10.1016/j.ijcard.2023.131447

M3 - Journal article

C2 - 37844667

AN - SCOPUS:85175465239

VL - 395

JO - International Journal of Cardiology

JF - International Journal of Cardiology

SN - 0167-5273

M1 - 131447

ER -