An analysis of the factors related to the prognosis of primary endocardial fibroelastosis

Abstract:

Objective：To investigate the clinical features of primary endocardial fibroelastosis（EFE） and the factors affecting its prog－nosis. Methods：A total of 150 children with EFE（52 males and 98 females） who were hospitalized in the Department of Cardiology in Children’s Hospital of Chongqing Medical University from January 2008 to April 2018 were enrolled. The age，body weight，clinical symptoms，personal history，and auxiliary examinations of the children at disease onset were collected. Among these patients，92 were followed up after discharge from the hospital and were divided into remission group（n=75） and deterioration group（n=17） according to their cardiac function and auxiliary examination results at follow-up. The chi-square test and U test were used for statistical analysis. Logistic regression was used to analyze the association of age and body weight of the patients at disease onset，maternal infection his－tory，cardiac function，and cardiothoracic ratio at initial diagnosis，status of gamma-globulin treatment，and duration of follow-up with prognosis. Results：The median age and median body weight of the 150 children at disease onset were 163 days and 7 kg，respectively；71.33% of the patients had a prodromal infection within two weeks，72% had respiratory symptoms as their earliest manifestations，71.33% had a cardiac function of grade Ⅰ-Ⅱ，28.67% had a cardiac function of grade Ⅲ，and 31.33% had a cardiothoracic ratio of ≥0.70 at initial diagnosis. The median endocardial thickness，mean LVDd，median LVFS，and mean LVEF were 2.5 mm，42.09 mm，18%，and 38%，respectively. Fifty-five point three-three percent of the patients had positive viral antibody test results. Among the 92 children being regularly followed up，there were signifi－cant differences in age at disease onset，duration of follow-up，and cardiothoracic ratio between the remission group and the deterioration group（P<0.05），but there were no significant differences in sex，maternal infection history，cardiac functional grading at initial diagnosis，or status of gamma-globulin treatment between the two groups（P>0.05）. After correction of the confounding factors such as sex，age at disease onset，maternal infection history，cardiac functional grading at initial diagnosis，and status of gamma-globulin treatment using binary logistic regression，the percentage of remitted children with a cardiothoracic ratio of <0.7 at initial diagnosis increased significantly（OR=6.89，95%CI=1.54 to 25.68），and the percentage of remitted children with a regular follow-up of <2 years decreased significantly（OR=0.11，95%CI=0.03 to 0.49）. Conclusion：EFE is prevalent in infants and young children，most of whom have a history of prodromal infection or maternal infection. An initial cardiothoracic ratio of <0.7 and a follow-up of >2 years are protective factors for remission of the disease.