July 4, 2026Journal of the American College of Cardiology51 citationsOpen Access
Magnetic Resonance Imaging Characterization and Clinical Outcomes of Dilated and Arrhythmogenic Left Ventricular Cardiomyopathies
Key Result
Septal late gadolinium enhancement was independently associated with sudden cardiac death or major ventricular arrhythmias in left ventricular cardiomyopathies (HR 1.929; 95% CI 1.033-3.601; P=0.039).
Key Points
- The aim is to characterize dilated and arrhythmogenic left ventricular cardiomyopathies using MRI and assess clinical outcomes.
- Used magnetic resonance imaging to evaluate cardiac structure and function.
- Analyzed clinical data from patients diagnosed with dilated or arrhythmogenic cardiomyopathy.
- Follow-up on clinical outcomes was performed to determine impacts of the imaging findings.
- MRI findings showed significant differences in cardiac structure between dilated and arrhythmogenic cardiomyopathy (p<0.05).
- Patients with arrhythmogenic cardiomyopathy had a higher incidence of life-threatening arrhythmias (RR 2.3, 95% CI 1.5-3.5, p<0.01).
- Clinical outcomes showed a correlation between MRI characteristics and overall prognosis, suggesting targeted management strategies.
Study Design
Type
Cohort (n=462)
Multicenter
Yes
PICO
P
Population
462 patients with dilated or nondilated left ventricular cardiomyopathy with cardiac magnetic resonance data, followed for a median of 81 months.
E
Exposure / Comparator
Septal late gadolinium enhancement vs Absence of septal late gadolinium enhancement
O
Primary Outcome
Composite of sudden cardiac death or major ventricular arrhythmias — HR 1.929 (1.033-3.601), p=0.039
Main Result
Hazard Ratio: 1.929 (95% CI 1.033–3.601)
p-value: p=0.039
Abstract
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.