Clinical efficacy of glucocorticoid on primary endocardial fibroelastosis in children

Objective To study the clinical efficacy of glucocorticoid in treatment of primary endocardial fibroelastosis (EFE) in children. Methods Fifty-eight primary EFE cases admitted in the Children's Hospital of Chongqing Medical University from Jan. 2006 to Dec. 2013 were treated and followed-up for more than 3 years. Based on whether the application of glucocorticoid and the length of time of glucocorticoid therapy, cases were divided into three groups: no glucocorticoid group (n=15), glucocorticoid 0.5-1 year group (n=17) and glucocorticoid 1-2 year group (n=26). The following indicators were followed up and observed: the cardiac function indexes before and after treatment [Modified ROSS score, left ventricular ejection fraction (LVEF), left ventricular fractional shortening (LVFS)], cardiac size [cardiothoracic ratio (C/T)] and the proportion of death. The efficacy and safety of glucocorticoid were studied. Results No statistically significant difference of modified ROSS scores, LVEF, LVFS and C/T existed among the 3 groups before treatment (P>0.05). After treatment, the modified ROSS score, LVEF, LVFS and C/T in glucocorticoid 0.5-1 year group were 2.06±1.78, 59.29±8.34, 31.24±6.0 and 0.580±0.055, respectively, which were significantly different from those before treatment (5.06±1.92, 38.76±6.31, 18.47±3.14 and 0.67±0.05, respectively, P 0.05). Conclusion The glucocorticoid has good clinical effect on endocardial fibroelastosis, and no significant difference is found on the efficacy between the total course of glucocorticoid therapy 0.5-1 year group and 1-2 years group. DOI: 10.11855/j.issn.0577-7402.2018.04.14