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C. Ciuca, L. Ragni, E. Angeli, G. Egidy Assenza, T. Hasan, G. Gargiulo, M. Bonvicini, P709
Outcome predictors of dilated cardiomyopathy in childhood, European Heart Journal, Volume 38, Issue suppl_1, August 2017, ehx501.P709, https://doi.org/10.1093/eurheartj/ehx501.P709Close - Share Icon Share
Extract
Purpose: To assess the magnitude and the predictors of either left ventricular recovery or failure leading to death or heart transplantation (HT) in paediatric patients with dilated cardiomyopathy (DCM) and severe left ventricular dysfunction (LVD)
Methods: 83 consecutive patients with DCM associated with severe LVD (left ventricular ejection fraction <35%, left ventricular dilatation >+2SD) and age <18 years were enrolled. Aetiology of DCM was: 29% idiopathic, 5% neuromuscular related, 15% associated with syndromic diseases, 8% anthracycline-related, 9% nutritional deficiency related, 12% myocarditis, 1% auto-immune myocarditis, 4% tachycardiomyopathy and 17% left ventricular non compaction (LVNC).
Results: Mean age was 4.9±6.3, 56.6% were male. Normalization of left ventricular function was achieved in 39.8% of all patients. At the multivariate logistic regression non idiopathic DCM (HR 13, 95% CI 25–72, p=0.002) younger age (HR 0.87, 95% CI 0.76–0.99, p=0.04) and less necessity of inotropic support (HR 0.36, 95% CI 0.10–0.89, p=0.04) predicted normalisation of left ventricular performance. Overall, one and 5-year survival free from HT was 83.6% respectively 69.8%,survival rate was different according to the type of DCM (figure 1). Familiar history of cardiomyopathy or sudden death (HR 2.8, 95% CI 1.1–6.9, p=0.02) and inotropic support during hospitalisation hospitalisation (HR 4.4, 95% CI 1.1–9.8, p<0.001) where associated with a higher risk of death or HT.
