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Magnetic Resonance Imaging Characterization and Clinical Outcomes of Dilated and Arrhythmogenic Left Ventricular
Matteo Castrichini1, Antonio De Luca2, Giulia De Angelis2
1Cardiothoracovascular Department, Division of Cardiology, Azienda Sanitaria Universitaria Giuliano Isontina, University of Trieste, Trieste, Italy, member of European Reference Network for Rare, Low-Prevalence, or Complex Diseases of the Heart (ERN GUARD-Heart); Department of Cardiovascular Medicine, Mayo Clinic, Rochester, Minnesota, USA; University of Colorado Cardiovascular Institute, Anschutz Medical Campus, Aurora, Colorado, USA.
Insights
Nondilated left ventricular cardiomyopathy (NDLVC) patients show higher genetic risks for arrhythmias and preserved function compared to dilated cardiomyopathy (DCM). Septal LGE predicts major arrhythmic events in both conditions.
Area of Science:
- Cardiology
- Genetics
- Cardiovascular Imaging
Background:
- Nondilated left ventricular cardiomyopathy (NDLVC) is distinct from dilated cardiomyopathy (DCM).
- Comprehensive characterization using cardiac magnetic resonance (CMR) and genetic testing is lacking.
Purpose of the Study:
- Characterize NDLVC and DCM in a large multicenter cohort.
- Assess clinical outcomes and identify predictors of adverse events in these patient groups.
Main Methods:
- Retrospective analysis of 462 patients (227 DCM, 235 NDLVC) with CMR data from 4 centers.
- Genetic testing for pathogenic variants.
- Composite endpoint: sudden cardiac death or major ventricular arrhythmias.
Main Results:
- NDLVC showed higher arrhythmogenic gene variants (40% vs 23%), preserved LV ejection fraction (51% vs 36%), and more free-wall LGE (27% vs 14%) than DCM.
- DCM had more nonarrhythmogenic gene variants (23% vs 12%) and septal LGE (45% vs 32%).
- Septal LGE independently predicted major arrhythmic events (HR: 1.929), along with LV dilatation, age, NYHA class, and ventricular arrhythmias.
Conclusions:
- Septal LGE is a key predictor of major arrhythmic events in NDLVC and DCM.
- LV dilatation, age, disease severity, and ventricular arrhythmias also predict adverse outcomes.
Background:
Nondilated left ventricular cardiomyopathy (NDLVC) has been recently differentiated from dilated cardiomyopathy (DCM). A comprehensive characterization of these 2 entities using cardiac magnetic resonance (CMR) and genetic testing has never been performed.
Objectives:
This study sought to provide a thorough characterization and assess clinical outcomes in a large multicenter cohort of patients with DCM and NDLVC.
Methods:
A total of 462 patients with DCM (227) or NDLVC (235) with CMR data from 4 different referral centers were retrospectively analyzed. The study endpoint was a composite of sudden cardiac death or major ventricular arrhythmias.
Results:
In comparison to DCM, NDLVC had a higher prevalence of pathogenic or likely pathogenic variants of arrhythmogenic genes (40% vs 23%; P < 0.001), higher left ventricular (LV) systolic function (LV ejection fraction: 51% ± 12% vs 36% ± 15%; P < 0.001) and higher prevalence of free-wall late gadolinium enhancement (LGE) (27% vs 14%; P < 0.001). Conversely, DCM showed higher prevalence of pathogenic or likely pathogenic variants of nonarrhythmogenic genes (23% vs 12%; P = 0.002) and septal LGE (45% vs 32%; P = 0.004). Over a median follow-up of 81 months (Q1-Q3: 40-132 months), the study outcome occurred in 98 (21%) patients. LGE with septal location (HR: 1.929; 95% CI: 1.033-3.601; P = 0.039) was independently associated with the risk of sudden cardiac death or major ventricular arrhythmias together with LV dilatation, older age, advanced NYHA functional class, frequent ventricular ectopic activity, and nonsustained ventricular tachycardia.
Conclusions:
In a multicenter cohort of patients with DCM and NDLVC, septal LGE together with LV dilatation, age, advanced disease, and frequent and repetitive ventricular arrhythmias were powerful predictors of major arrhythmic events.
