Heart failure & cardiomyopathies

Clinical Characteristics and Outcomes of Patients With Biventricular and Left-Dominant Arrhythmogenic Cardiomyopathy With Ring-Like Late Gadolinium Enhancement Pattern

JACC Clin ElectrophysiolPublished observational cohort study

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
Methodological detailsconsecutive patients
  • Consecutive patients

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 = 0.042
Nonsustained 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

Composed by AI from the published abstract and checked against it. How Briefs are made →

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