Heart failure & cardiomyopathiesCohort study
Clinical Characteristics and Outcomes of Patients With Biventricular and Left-Dominant Arrhythmogenic Cardiomyopathy With Ring-Like Late Gadolinium Enhancement Pattern
- observational cohort study
- consecutive patients
Authors' conclusion
RL-LGE independently predicts arrhythmic events in ACM, regardless of genotype status, and refines the prognostic performance of traditional risk models. Its detection may aid in early recognition of high-risk patients and inform primary-prevention ICD therapy.
Study context
- Population
- Patients with a diagnosis of biventricular or left-dominant arrhythmogenic cardiomyopathy (BIV-ACM or LD-ACM)mean age 36 ± 12 years, 66% male
- Participants
- 149 patients
- Exposure
- RL-LGE as a predictor of the primary endpoint
- RL-LGEn = 73
- Outcome
- Composite of:
- Sudden cardiac arrest
- Sustained ventricular tachycardia
- Implantable cardioverter-defibrillator (ICD) interventions
- Follow-up
- Median 31 months
Main finding
As reported in the abstract
Hazard ratio (95% CI)P value
RL-LGEmultivariable Cox regression; independent predictor
2.47(1.10–6.02)
P = 0.042nonsustained ventricular tachycardiamultivariable Cox regression
2.62(1.17–6.41)
P = 0.033- Patients who experienced the primary endpoint
- 24
- Of the 24 patients with the primary endpoint, share who had RL-LGE
- 67%
Observational data: associations, not effects.
Further findings
Prevalence of RL-LGE
- RL-LGE identified
- 73 (49%)
- Prevalence in BIV-ACM
- 70%
- Prevalence in LD-ACM
- 30%
- Most frequently in association with DSP variants
Incorporating RL-LGE into the arrhythmogenic right ventricular cardiomyopathy risk model
- Significantly improved predictive performance (likelihood ratio test)
- P = 0.006
Original publication
Clinical Characteristics and Outcomes of Patients With Biventricular and Left-Dominant Arrhythmogenic Cardiomyopathy With Ring-Like Late Gadolinium Enhancement Pattern. JACC Clin Electrophysiol. Published 16 March 2026. doi:10.1016/j.jacep.2026.02.019
Summarised from the published abstract; every number is checked against it