Abstract
Arrhythmogenic Cardiomyopathy (ACM) is a heredo-familial cardiac disease characterized by fibro-fatty myocardial replacement and increased risk of sudden cardiac death. The diagnosis of ACM can be challenging due to the lack of a single gold-standard test: for this reason, it is required to satisfy a combination of multiple criteria from different categories including ventricular morpho-functional abnormalities, repolarization and depolarization ECG changes, ventricular arrhythmias, tissue characterization findings and positive family history/molecular genetics. The first diagnostic criteria were published by an International Task Force (ITF) of experts in 1994 and revised in 2010 with the aim to increase sensitivity for early diagnosis. Limitations of the 2010 ITF criteria include the absence of specific criteria for left ventricle (LV) involvement and the limited role of cardiac magnetic resonance (CMR) as the use of the late gadolinium enhancement technique for tissue characterization was not considered. In 2020, new diagnostic criteria (“the Padua criteria”) were proposed. The traditional organization in six categories of major/minor criteria was maintained. The criteria for identifying the right ventricular involvement were modified and a specific set of criteria for identifying LV involvement was created. Depending on the combination of criteria for right and LV involvement, a diagnosis of classic (right dominant) ACM, biventricular ACM or left-dominant ACM is then made. The article reviews the rationale of the Padua criteria, summarizes the main modifications compared to the previous 2010 ITF criteria and provides three examples of the application of the Padua criteria in clinical practice.
Highlights
Background published maps and institutional affilArrhythmogenic cardiomyopathy (ACM) is an inherited heart muscle disease characterized by progressive fibro-fatty replacement and malignant ventricular arrhythmias that may lead to sudden cardiac death, especially in young people and athletes [1,2]
The fatty tissue replacement can be detected with dedicated sequences by cardiac magnetic resonance (CMR), and it is often observed in the same regions of late gadolinium enhancement (LGE): it is not considered a diagnostic criterion when found in isolation because of its lack of specificity
We provided an overview of the 2020 Padua criteria for ACM diagnosis and examples of their practical application
Summary
The Padua criteria are organized in two different sets of criteria to identify, respectively, clinical signs of RV and LV involvement. In both sets of criteria, the traditional organization in six diagnostic categories is maintained, including morpho-functional changes, tissue characterization, repolarization and depolarization ECG abnormalities, ventricular arrhythmias and family history/genetic testing. Global RV systolic dysfunction (reduction of RV EF according to the imaging test specific monograms for age and sex). I. Morpho-functional ventricular abnormalities or Regional RV akinesia, aneurysm of RV free wall dyskinesia. LV dilatation (increase in LV EDV according to the imaging test specific nomograms for age, sex, and BSA). LV LGE (stria pattern) of ≥1 Bull’s Eye segment(s) (in 2 orthogonal views) of the free wall (subepicardial or midmyocardial), septum or both (excluding septal junctional LGE)
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