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. 2022 Feb 2;24(2):296-305.
doi: 10.1093/europace/euab162.

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

Laurens P Bosman  1   2 , Claire L Nielsen Gerlach  2 , Julia Cadrin-Tourigny  3 , Gabriela Orgeron  4 , Crystal Tichnell  4 , Brittney Murray  4 , Mimount Bourfiss  2 , Jeroen F van der Heijden  2 , Sing-Chien Yap  5 , Katja Zeppenfeld  6 , Maarten P van den Berg  7 , Arthur A M Wilde  8 , Folkert W Asselbergs  1   2   9   10 , Hariskrishna Tandri  4 , Hugh Calkins  4 , J Peter van Tintelen  1   11 , Cynthia A James  4 , Anneline S J M Te Riele  1   2
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Comparing clinical performance of current implantable cardioverter-defibrillator implantation recommendations in arrhythmogenic right ventricular cardiomyopathy

Laurens P Bosman et al. Europace. .

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.

Keywords: Arrhythmogenic right ventricular cardiomyopathy; Implantable cardioverter-defibrillator; Prognosis; Risk stratification; Ventricular arrhythmias.

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Figures

Figure 1
Figure 1
Schematic overview of the four ICD placement algorithms. ICD, implantable cardioverter-defibrillator; LVEF, left ventricular ejection fraction; PVC, premature ventricular complex; RVEF, right ventricular ejection fraction; VA, ventricular arrhythmia; VT, ventricular tachycardia.
Figure 2
Figure 2
Kaplan–Meier plots with 95% CI for survival free from any sustained VA for each of the four ICD placement algorithms; ITFC (A), mITFC (B), AHA (C), and HRS (D). Survival is significantly worse concordant with the class of ICD indication. ICD, implantable cardioverter-defibrillator; VA, ventricular arrhythmia.
Figure 3
Figure 3
Kaplan–Meier plots with 95% CI for survival free from fast VA for each of the four ICD placement algorithms; ITFC (A), mITFC (B), AHA (C), and HRS (D). Only HRS showed a significantly different survival between ICD indication classes but only between class IIa and none (IIb/III). ICD, implantable cardioverter-defibrillator; VA, ventricular arrhythmia.
Figure 4
Figure 4
Clinical performance measures of the four ICD placement recommendation algorithms at a 5-year time point for (A) any sustained VA, and (B) fast VA. Bar chart shows the proportion of patients correctly classified (blue) and incorrectly classified (orange), with dark colouring for those with events and light colouring for those without. Table on the right shows total proportion with ICD, sensitivity (Se), specificity (Sp), and time-dependent area under the curve (AUC). ICD, implantable cardioverter-defibrillator; VA, ventricular arrhythmia.
Figure 5
Figure 5
Decision curve analysis with the 5-year risk threshold for ICD placement on the X-axis and net benefit on the Y-axis. (A) For any sustained VA, this graph demonstrates that when the risk threshold justifying ICD placement lies between 5–25%, ITFC and mITFC algorithms had the best performance, while AHA and HRS perform best if the risk threshold is >25%. (B) For fast VA, mITFC performs best when the threshold lies between 2–4%, ITFC when between 4–9%, and AHA/HRS when >9%. ICD, implantable cardioverter-defibrillator; VA, ventricular arrhythmia.
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