Related Experiment Video
Updated: Jan 12, 2026

Dual-Dye Optical Mapping of Hearts from RyR2R2474S Knock-In Mice of Catecholaminergic Polymorphic Ventricular Tachycardia
Published on: December 22, 2023
European task force reclassification of dilated and non-dilated cardiomyopathies identifies a high arrhythmic-risk
Francesca Bonanni1, Ignazio Alessio Gueli2, Irina Bellisario3
1Interdisciplinary Center for Health Sciences, Scuola Superiore Sant'Anna, Pisa, Italy.
Insights
The European Task Force Criteria identified an overlapping cardiomyopathy phenotype with higher arrhythmic risk. This highlights the importance of multiparametric approaches for improved risk stratification in patients with cardiomyopathies.
Area of Science:
- Cardiology
- Genetics
- Medical Diagnostics
Background:
- Classifying left ventricular cardiomyopathies is complex due to evolving definitions and overlapping phenotypes.
- The European Task Force Criteria refine arrhythmogenic cardiomyopathy diagnosis using structural, functional, arrhythmic, and genetic data.
- This study aimed to characterize genotype-phenotype features in arrhythmogenic left ventricular/biventricular cardiomyopathy (ALVC/ABVC), dilated cardiomyopathy (DCM), and their overlap.
Purpose of the Study:
- To reclassify patients with DCM or non-dilated left ventricular cardiomyopathy (NDLVC) using the European Task Force Criteria.
- To identify and characterize an overlapping DCM-ALVC/ABVC phenotype.
- To assess the genotype-phenotype correlation and clinical outcomes in these patient groups.
Main Methods:
- Retrospective evaluation of 306 patients with DCM or NDLVC.
- Reclassification into isolated DCM, isolated ALVC/ABVC, and DCM-ALVC/ABVC overlapping phenotypes based on European Task Force Criteria.
- Exclusion of NDLVC not meeting criteria and isolated arrhythmogenic right ventricular cardiomyopathy; composite endpoint of cardiac death, VT, or VF.
Main Results:
- 66 patients were categorized as DCM, 79 as ALVC/ABVC, and 76 as DCM-ALVC/ABVC.
- Genetic variants were more frequent in ALVC/ABVC (50.6%) than DCM (28.8%) and DCM-ALVC/ABVC (27.6%).
- DCM-ALVC/ABVC patients showed extensive LGE, higher NT-proBNP, increased arrhythmic burden, and significantly lower event-free survival.
Conclusions:
- The European Task Force Criteria successfully identified an overlapping phenotype with increased arrhythmic risk.
- Multiparametric diagnostic approaches are clinically valuable for improved risk stratification.
- These findings support tailored therapeutic decisions for patients with cardiomyopathies.
Background:
The classification of left ventricular cardiomyopathies is challenging due to evolving definitions and overlapping phenotypes. The European Task Force Criteria, by incorporating structural, functional, arrhythmic, and genetic parameters, refine the diagnostic criteria for arrhythmogenic cardiomyopathy. This study aimed to characterize the genotype-phenotype features of patients with arrhythmogenic left ventricular/biventricular cardiomyopathy (ALVC/ABVC), dilated cardiomyopathy (DCM), and their potential overlap.
Methods:
We retrospectively evaluated 306 patients with DCM or non-dilated left ventricular cardiomyopathy (NDLVC). Following European Task Force criteria, patients were reclassified as: isolated DCM, isolated ALVC/ABVC and a DCM-ALVC/ABVC overlapping phenotype. NDLVC not fulfilling the criteria, and isolated arrhythmogenic right ventricular cardiomyopathy were excluded. The study endpoint was a composite of cardiac death, sustained ventricular tachycardia, or ventricular fibrillation.
Results:
Overall, 66, 79 and 76 patients were categorized as DCM, ALVC/ABVC, DCM-ALVC/ABVC, respectively. Genetic likely-pathogenic/pathogenic variants were more frequent in ALVC/ABVC than in DCM and DCM-ALVC/ABVC (50.6 % vs 28.8 % and 27.6 %, p = 0.004). DCM-ALVC/ABVC patients presented extensive LGE, higher NT-proBNP, and the highest arrhythmic burden (all p < 0.05). Moreover, they had significantly lower event-free survival (log-rank p = 0.002). In Cox analysis, LVEF (HR: 0.96, 95 % CI 0.94-0.99, p = 0.002) and LGE extent (HR: 1.04, 95 % CI 1.00-1.07, p = 0.016) independently predicted the endpoint.
Conclusions:
The European Task Force Criteria enabled the identification of an overlapping phenotype associated with increased arrhythmic risk, supporting the clinical utility of multiparametric diagnostic approaches to improve risk stratification and guide therapeutic decisions in patients with cardiomyopathies.
More Related Videos
09:16Isolation and Characterization of Cardiac Mesenchymal Stromal Cells from Endomyocardial Bioptic Samples of Arrhythmogenic Cardiomyopathy Patients
Published on: February 28, 2018
07:11Morphological and Functional Assessment of the Right Ventricle Using 3D Echocardiography
Published on: October 28, 2020
Related Concept Videos
Cardiomyopathy I: Introduction and Classification
Cardiomyopathy II: Dilated Cardiomyopathy
Cardiomyopathy III: Hypertrophic Cardiomyopathy
Cardiomyopathy IV: Restrictive Cardiomyopathy
Dysrhythmias II: Classification of Tachyarrhythmias
Cardiomyopathy V: Interprofessional Care