Nomogram for predicting cardiac death and heart transplantation in arrhythmogenic right ventricular cardiomyopathy

In this study, we aimed to develop and validate a competing risk nomogram for predicting cardiac death and heart transplantation (HT) in patients with arrhythmogenic right ventricular cardiomyopathy (ARVC).

We retrospectively enrolled 149 consecutive patients with ARVC diagnosed at our institution between 2008 and 2022. Cox proportional hazards model was primarily used to identify variables associated with cardiac death and HT. On the basis of these indicators, a competing risk nomogram was developed to predict the 1, 3, and 5 year probabilities of cardiac death and HT. The area under the receiver operating characteristic curve (AUC), Harrell's C-index, and calibration curves were used to evaluate and internally validate the performance of the model. Decision curve analysis was performed to assess the clinical utility of the nomogram.

Of the 149 patients with ARVC, the mean age was 38.77 ± 15.94 years, and most of the patients were men (67.11%, 100/149). Fourteen patients experienced cardiac death and nine underwent HT, during a median follow-up period of 5.8 years (interquartile range, 0.62-5.56 years). Multivariable COX analysis revealed that extent of TWI in the anterior and inferior leads (P = 0.0057), right atrial diameter on transthoracic echocardiography (P = 0.0498), RVEF (P = 0.1036), and LVEF (P < 0.001) all showed statistical significance. The 1-, 3-, and 5-year cumulative incidence of cardiac death and HT were 3.35%, 8.05%, and 11.4%, respectively. The area under the receiver operating characteristic curve of the nomogram for predicting cardiac death and HT at 1, 3, and 5 years after diagnosis of ARVC were 0.860, 0.935, and 0.956. The value of Harrell's C-index is 0.9273 (95% confidence interval 0.8954-0.9590; P < 0.001), indicating that the model had good discriminative ability in internal validation. Decision curve analysis revealed that our model was clinically useful within the entire range of potential treatment thresholds in most cases. The cumulative incidence of the primary outcomes was significantly different between the three risk groups according to nomogram-derived scores (P < 0.001).

On the basis of a retrospective review of patients with ARVC at a single centre, we developed a novel nomogram for predicting the risk of cardiac death and HT after ARVC diagnosis. This competing risk nomogram based on four readily available clinical parameters (right atrial diameter, right and left ventricular ejection fraction, and T-wave inversion) is a potentially useful tool for individualized prognostic assessment in patients with ARVC.