Development of Cardiac Events and Functional Recovery Prediction Models for Pediatric Dilated Cardiomyopathy.

Abstract

Background: Since both the risk of death and the probability of spontaneous functional recovery (FR) coexist in association with pediatric dilated cardiomyopathy (DCMP), management should be based on individualized outcome predictions.Methods: A single-center retrospective review of 105 pediatric patients (age at presentation ≤ 18 years) with DCMP, managed between 1994 and 2017, was performed. Logistic regression was conducted to identify variables associated with FR and cardiac events (CEs), i.e., death or heart transplantation (HTPL), within 2 years after initial presentation. Two outcome prediction models were formulated using these variables.Results: Twenty-six (24.8%) and 51 patients (48.6%) experienced FR and CE, respectively, within 2 years after initial presentation. Predictors of mortality without HTPL were earlier era at presentation (HR: 4.13; 95% CI: 1.88–9.06; p < 0.001) and significant TR (≥moderate; HR: 4.31; 95% CI: 1.26–14.77; p = 0.020) in multivariable Cox regression model. Predictors of FR were recent era (HR: 4.49; 95% CI: 1.40–14.44; p = 0.0012), younger age at initial presentation (HR: 0.98 per 1 month increase; 95% CI: 0.97–0.99, p < 0.001), post-myocarditis DCMP (HR: 4.29; 95% CI: 1.32–13.93; p = 0.015), and arrhythmia-mediated DCMP (HR: 26.88; 95% CI: 2.61–276.70; p = 0.006). Risk factors for CEs was idiopathic DCMP (HR: 2.95; 95% CI: 1.32–6.56, p = 0.008). The low-risk group who had higher probability of FR than CE in prediction model had a slightly higher overall survival rate (71.4 vs. 52.2% at 10 years after presentation; log-rank p = 0.09) and a significantly higher HTPL-free survival rate (67.5 vs. 24.9% at 10 years after presentation; log-rank p < 0.001) than the high-risk group.Conclusions: Prognostication and management strategies for pediatric DCMP may be enhanced by risk stratification using outcome prediction modeling.