EP News: Pediatric and Congenital Electrophysiology

  • Martin J. LaPage
    Address reprint requests and correspondence: Dr Martin J. LaPage, Division of Pediatric Cardiology, Department of Pediatrics, University of Michigan, 1540 E Hospital Dr, 11th floor Pediatric Cardiology, Ann Arbor, MI 48109.
    Division of Pediatric Cardiology, Department of Pediatrics, University of Michigan, Ann Arbor, Michigan
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Published:September 02, 2022DOI:
      Jorda et al (Eur Heart J 2022;43:3041, PMID 35766180) validated the prediction model in an observational, retrospective, longitudinal study in an external multicenter cohort and compared its performance to current guideline recommendations for implantable cardioverter-defibrillator (ICD) use on the basis of decision curve analysis. (The calculator is an online algorithm published in 2019 that intends to predict the occurrence of sustained ventricular arrhythmias [including sudden cardiac death or arrest] within 5 years of arrhythmogenic right ventricular cardiomyopathy diagnosis in patients aged 14 years.) The validation cohort included 429 patients (mean age 43 years), of whom 103 (24%) experienced sustained ventricular tachycardia during a median follow-up of 5 years. Algorithm validation showed good agreement (C-index 0.7; 95% confidence interval 0.65–0.75) between predicted and observed outcomes at 1 and 5 years. The algorithm was superior to current guidelines–based risk assessment to predict the net clinical benefit of ICD use. The authors conclude that the calculator provided reliable prognostic data to predict VAs and outperformed clinical statements in predicting ICD benefit.
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