Comparison of different prediction models for the indication of implanted cardioverter defibrillator in patients with arrhythmogenic right ventricular cardiomyopathy

Abstract

Aims: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is associated with a high risk of sudden cardiac death. Three different prediction models for the indication of implanted cardioverter defibrillator (ICD) are now available: the 5 year ARVC risk score, the International Task Force Consensus (ITFC) criteria, and the Heart Rhythm Society (HRS) criteria. We compared these three prediction models in a validation cohort of patients with definite ARVC.

Methods and results: In a cohort of 140 patients with definite ARVC, the 5 year ARVC risk score and the ITFC and HRS criteria were compared for the prediction of a major combined endpoint of sudden cardiac death, appropriate ICD intervention, resuscitated cardiac arrest, and sustained ventricular tachycardia. During the follow-up, 65 major events occurred. The 5 year ARVC risk score with a threshold >10%, derived from the maximally selected rank statistic, predicted 62 (95%) events [odds ratio (OR) 9.1, 95% confidence interval (CI) 2.6-32, P = 0.0006], the ITFC criteria 53 (81%, OR 4.8, 95% CI 2.2-10.3, P = 0.0001), and the HRS criteria 29 (45%, OR 4.2, 95% CI 1.9-9.3, P = 0.0003). At the analysis of decision curve for ICD implantation, a 5 year ARVC risk score >10% showed a greater net benefit than the ITFC and HRS criteria over a wide range of threshold probability of events. Finally, at multivariate analysis, the 5 year ARVC risk score >10% was the only independent predictor of major events.

Conclusions: The 5 year score with a threshold of >10% was more effective for predicting events than the ITFC and HRS criteria.

Keywords: 5 year ARVC risk score; Arrhythmogenic cardiomyopathy; Heart Rhythm Society criteria; International Task Force Consensus; Prognosis.

Figure 1

Maximally selected rank statistic of 5 year arrhythmogenic right ventricular cardiomyopathy (ARVC) risk score to predict major combined events: the optimal threshold of 5 year ARVC score was >10%.

Figure 2

Kaplan–Meier survival free from event curves: in the left panel, Kaplan–Meier curves showed that patients with 5 year ARVC risk score >10% had worse prognosis than those with lower values of risk score. As showed in the middle panel, patients satisfying the International Task Force Consensus (ITFC) criteria had worse prognosis than those without. Finally, Kaplan–Meier curve of right panel demonstrated the prognostic role of Heart Rhythm Society (HRS) criteria.

Figure 3

Time‐dependent area under the curve (AUC) for predicting major combined endpoint: the time‐dependent AUC curves of the 5 year ARVC score (continuous variable), of the International Task Force Consensus (ITFC) algorithm, and of the Heart Rhythm Society (HRS) criteria are showed in the upper panels, respectively, from left to right. In the lower panels, the time‐dependent AUC difference between 5 year ARVC score and the ITFC, ARVC, and HRS criteria and between HRS and ITFC algorithm are showed.

Figure 4

Decision curve analysis of implanted cardioverter defibrillator (ICD) implantation for preventing the major combined endpoint. In the left panel, the net benefit curves of different thresholds of 5 year arrhythmogenic right ventricular cardiomyopathy (ARVC) risk score are compared, demonstrating that the >10% threshold had a higher net benefit compared with the other thresholds and to the ‘ICD to all the patients’ approach for a wide range of threshold probability, including the range of probability corresponding to the reported 5 year risk of sudden death in ARVC. In the right panels, the net benefit curves of the 5 year ARVC score >10%, of the International Task Force Consensus (ITFC) algorithm, and of the Heart Rhythm Society (HRS) criteria were compared. The 5 year ARVC score >10% had a greater net benefit than other models in a wide range of probability including the range of reported 5 year risk of sudden death of ARVC.