A single center experience in pediatric cardiomyopathy. Risk factors, outcomes and the effect of levosimendan

Cardiomyopathies are the leading cause of heart failure (HF) in children with anatomically intact hearts. A retrospective data analysis of a tertiary cardiac surgery and cardiology center cohort was performed. Our objectives were to analyze demographic, clinical, echocardiographic and hemodynamic data of children with HF due to cardiomyopathy- myocarditis, identify risk factors predictive of outcome and evaluate the possible effect of levosimendan administration. A total of 75 patients were included in the study. Median follow up was 24.1 months [interquartile range (IQR) 8.3-85.9]. Forty nine patients (71%) presented with significant HF (stage III/IV), with dilated cardiomyopathy (DCM) being the predominant diagnosis (74%). Twenty five patients (36%) experienced adverse outcome (defined as the composite endpoint of deterioration, transplantation listing and death), 18 (26%) died and 19 (27%) fully recovered. Severe HF at presentation (stage III/IV), presence of fibrosis on endomyocardial biopsy, intubation during admission at presentation and NT-proBNP values were identified as risk factors for death. Sixteen patients received repeated 24-hour levosimendan infusions [median 12 infusions/patient (IQR 9-24)]. All received a loading dose but one. No hypotensive episodes were recorded during loading or the first 24-hour infusion. Levosimendan administration was associated with significant improvement of left ventricular fractional shortening (LVFS, p =.003) and significant reduction of NT-proBNP values (p =.033). No difference was detected in survival time (combined endpoint of death or transplantation) between patients who received levosimendan and those who did not (log-rank test p-value =.645). To conclude, the majority of children in our study presented with significant HF (stage III/IV) with DCM being the predominant diagnosis. During follow up 27% fully recovered while 26% died. Several factors were associated with death. Levosimendan infusions were safe to administrate and associated with improvement of LVFS and reduction of NT-proBNP values but no survival benefit.