Comparing clinical performance of current implantable cardioverter-defibrillator implantation recommendations in arrhythmogenic right ventricular cardiomyopathy

Laurens P Bosman*, Claire L Nielsen Gerlach, Julia Cadrin-Tourigny, Gabriela Orgeron, Crystal Tichnell, Brittney Murray, Mimount Bourfiss, Jeroen F van der Heijden, Sing-Chien Yap, Katja Zeppenfeld, Maarten P van den Berg, Arthur A M Wilde, Folkert W Asselbergs, Hariskrishna Tandri, Hugh Calkins, J Peter van Tintelen, Cynthia A James, Anneline S J M Te Riele

*Corresponding author for this work

Research output: Contribution to journalArticleAcademicpeer-review

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Abstract

Aims Arrhythmogenic right ventricular cardiomyopathy (ARVC) patients have an increased risk of ventricular arrhythmias (VA). Four implantable cardioverter-defibrillator (ICD) recommendation algorithms are available The International Task Force Consensus ('ITFC'), an ITFC modification by Orgeron et al. ('mITFC'), the AHA/HRS/ACC guideline for VA management ('AHA'), and the HRS expert consensus statement ('HRS'). This study aims to validate and compare the performance of these algorithms in ARVC. Methods and results We classified 617 definite ARVC patients (38.5 +/- 15.1 years, 52.4% male, 39.2% prior sustained VA) according to four algorithms. Clinical performance was evaluated by sensitivity, specificity, ROC-analysis, and decision curve analysis for any sustained VA and for fast VA (>250 b.p.m.). During 6.4 [2.8-11.5] years follow-up, 282 (45.7%) patients experienced any sustained VA, and 63 (10.2%) fast VA. For any sustained VA, ITFC and mITFC provide higher sensitivity than AHA and HRS (94.0-97.8% vs. 76.7-83.5%), but lower specificity (15.9-32.0% vs. 42.7%-60.1%). Similarly, for fast VA, ITFC and mITFC provide higher sensitivity than AHA and HRS (95.2-97.1% vs. 76.7-78.4%) but lower specificity (42.7-43.1 vs. 76.7-78.4%). Decision curve analysis showed ITFC and mITFC to be superior for a 5-year sustained VA risk ICD indication threshold between 5-25% or 2-9% for fast VA. Conclusion The ITFC and mITFC provide the highest protection rates, whereas AHA and HRS decrease unnecessary ICD placements. ITFC or mITFC should be used if we consider the 5-year threshold for ICD indication to lie within 5-25% for sustained VA or 2-9% for fast VA. These data will inform decision-making for ICD placement in ARVC.

Original languageEnglish
Article numbereuab162
Pages (from-to)296-305
Number of pages10
JournalEuropace
Volume24
Issue number2
Early online date2-Sept-2021
DOIs
Publication statusPublished - 2-Feb-2022

Keywords

  • Arrhythmogenic right ventricular cardiomyopathy
  • Prognosis
  • Risk stratification
  • Implantable cardioverter-defibrillator
  • Ventricular arrhythmias
  • HYPERTROPHIC CARDIOMYOPATHY
  • TASK-FORCE
  • DYSPLASIA/CARDIOMYOPATHY
  • DIAGNOSIS
  • RISK

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