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

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.

Right ventricular function is a predictor for sustained ventricular tachycardia requiring anti-tachycardic pacing in arrhythmogenic ventricular cardiomyopathy: insight into transvenous vs. subcutaneous implantable cardioverter defibrillator insertion

“… 4 , 16 Severe RV dysfunction is also one of the Class I indications for an ICD in patients with ARVC in accordance with four stratification algorithms. 17 In this study, it has also been shown that RV dysfunction is also an independent predictor of VT resulting in ATP.…”

Arrhythmias as Presentation of Genetic Cardiomyopathy

“…Indications for ICD are based on the ARVC disease criteria 105 and specific risk factors for VA (Table 1). 154 The course is usually dominated by VA, as symptomatic HF developed in only 13% of index cases in this large series, with only 4% going to heart transplantation.…”

Implantable defibrillators in primary prevention of genetic arrhythmias. A shocking choice?

“…Recently, the comparison of the clinical performance of current algorithms for ICD implantation in ARVC confirmed that both the 2015 ITFC and the modified ITFC recommendations provide the highest ICD protection rates. 114 , 115 The 2015 ITFC remain the best performing algorithm to indicate an ICD in ARVC, if one considers a threshold of >6% 5-year risk (similar to the threshold for HCM patients) of fast VT. 115 …”