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

5 hours ago
  • #ventricular arrhythmias
  • #late gadolinium enhancement
  • #arrhythmogenic cardiomyopathy
  • RL-LGE (ring-like late gadolinium enhancement) is a distinctive imaging marker for arrhythmogenic cardiomyopathy (ACM) with left ventricular involvement.
  • The study included 149 patients with biventricular or left-dominant ACM, with RL-LGE identified in 49% of cases, more frequently in biventricular ACM (70%) than left-dominant ACM (30%).
  • RL-LGE was associated with DSP genetic variants and independently predicted arrhythmic events (HR: 2.47).
  • Over a median follow-up of 31 months, 24 patients experienced the primary endpoint (sudden cardiac arrest, sustained ventricular tachycardia, or ICD interventions), with 67% having RL-LGE.
  • Incorporating RL-LGE into risk models improved predictive performance for arrhythmic events, aiding in early identification of high-risk patients.
  • Genetic status did not provide additional prognostic information beyond RL-LGE and nonsustained ventricular tachycardia.