November 6, 2025International Journal of Cardiology1 citationsOpen Access
European task force reclassification of dilated and non-dilated cardiomyopathies identifies a high arrhythmic-risk overlap phenotype
Key Result
A DCM-ALVC/ABVC overlapping phenotype had lower event-free survival (p=0.002), while LVEF (HR 0.96) and LGE extent (HR 1.04) independently predicted cardiac death or ventricular arrhythmias.
Study Design
Type
Cohort (n=306)
Structured PICO
Does reclassification using European Task Force criteria identify high arrhythmic-risk overlap phenotypes in patients with dilated and non-dilated left ventricular cardiomyopathies?
P
Population
306 patients with dilated or non-dilated left ventricular cardiomyopathy were retrospectively evaluated to characterize genotype-phenotype features and potential overlap.
E
Exposure
Reclassification using European Task Force criteria into isolated DCM, isolated arrhythmogenic left ventricular/biventricular cardiomyopathy (ALVC/ABVC), and a DCM-ALVC/ABVC overlapping phenotype.
C
Comparator
Comparison among the three reclassified groups (isolated DCM, isolated ALVC/ABVC, and DCM-ALVC/ABVC overlapping phenotype).
O
Outcome
Composite of cardiac death, sustained ventricular tachycardia, or ventricular fibrillation.composite
Reclassification of cardiomyopathies using European Task Force criteria identifies an overlapping DCM-ALVC/ABVC phenotype with higher arrhythmic risk and worse event-free survival.
Main Result
p-value: p=0.002
Abstract
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.