A single center experience in pediatric cardiomyopathy. Risk factors, outcomes and the effect of levosimendan. (June 2020)

• Record Type: Journal Article
• Authors: Kourelis, Georgios; Apostolopoulou, Sotiria; Rallis, Dimitrios; Vagenakis, Georgios A.; Kakava, Felicia; Kyriakoulis, Konstantinos; Laskari, Cleo V.; Tsoutsinos, Alexandros; Ekmektzoglou, Konstantinos; Chalkias, Athanasios; Iacovidou, Nicoletta Μ.; Rammos, Spyridon
• Is Part Of: Progress in pediatric cardiology. Volume 57(2020)
• Publication Date: 2020-06
• Subjects: ATP adenosine triphosphate -- AVVR atrioventricular valve regurgitation -- BNP B-type natriuretic peptide -- CI Confidence Interval -- CO cardiac output -- CHD congenital heart disease -- DCM dilated cardiomyopathy -- ECMO extracorporeal membrane oxygenator -- EF ejection fraction -- EMB endomyocardial biopsy -- FDA Food and Drug Administration -- FS fractional shortening -- HF heart failure -- HR heart rate -- ICD implantable cardioverter – defibrillator -- ICU Intensive Care Unit -- IQR interquartile range -- IV intravenous -- LCOS low cardiac output syndrome -- LOS length of stay -- LV left ventricle -- LVEDP left ventricular end diastolic pressure -- LVEF Left Ventricular Ejection Fraction -- LVFS left ventricular fractional shortening -- MCS mechanical circulatory support -- NT-proBNP N-terminal pro b-type natriuretic peptide -- NYHA New York Heart Association -- OR Odds Ratio -- PCR Polymerase Chain Reaction -- SD standard deviation -- VAD ventricular assist device; Children -- Heart failure -- Cardiomyopathy -- Myocarditis -- Heart transplantation -- Levosimendan; Pediatric cardiology -- Periodicals; Cardiovascular Diseases -- Periodicals; Infant; Child; Cardiologie pédiatrique -- Périodiques; 618.9212005
• DOI: 10.1016/j.ppedcard.2020.101201
• Languages: English
• ISSNs: 1058-9813

Abstract

Abstract: 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. Highlights: Dilated cardiomyopathy is the most common form of cardiomyopathy in children. Endomyocardial biopsy and cardiac catheterization could add to risk assessment. Levosimendan infusions including a loading dose are safe to administer. Levosimendan is associated with improvement of cardiac function. Levosimendan administration wasn't associated with survival benefit.