The Clinical Feature And Prognosis Of Primary Endocardial Fibroelastosis In Children

Objective: To investigate the clinical features of primary endocardial fibroelastosis(EFE)related factors of its prognosis.Methods: A total of 150 hospitalized EFE patients(52 males and 98 females)in the Department of Cardiology of Children's Hospital of Chongqing Medical University from January 2008 to April 2018 were enrolled.The age and weight of the onset,clinical symptoms,and individuals history,auxiliary examination were collected,92 patients were followed up.According to the cardiac function and auxiliary examination results at the follow-up,they were divided into remission group(n=75)and deterioration group(n=17).U test,Chi square test analysis were used for statistical analysis.Logistic regression analysis were used to estimate the association of The age and weight of onset,the history of infection during maternal pregnancy,the initial diagnosis of cardiac function,the ratio of cardiothoracic,whether the treatment of IVIG,follow-up time and prognosis.Results: The median age of onset of 150 children was 163 days,the median weight was 7 kg,71.33% had pre-infection within two weeks,72% patients had respiratory symptoms,and cardiac function of 71.33% patients was grade?-?,cardiac function of 28.67% patiens was grade III,and 31.33% patiens of cardiothoracic ratio was ?0.70.The median endocardial thickness of cardiac ultrasound was 2.5 mm,the median FS was 18%,the mean LVD was 42.09 mm,and the mean EF was 38%.55.33% of virus antibodies were positive.(2)In the 92 children with regular follow-up,There were statistically significant differences in the age the onset,cardiothoracic ratio,follow-up time between remission group and deterioration group(P<0.05).There was no significant difference in gender,maternal infection,initial cardiac function,and IVIG therapy between the two groups(P>0.05).Using binary logistic regression to correct the confounding factors such as gender,age at onset,history of infection during maternal pregnancy,grade of cardiac function,and IVIG treatment,the probability of remission was significantly higher in children with cardiothoracic ratio of less than 0.7 [OR=6.89,95 %CI(1.54,25.68)],the probability of remission was significantly lower in children with a regular follow-up of less than 2 years [OR=0.11,95% CI(0.03,0.49)].Conclusion: EFE is more common in infants and young children.Most of the children have a history of pre-infection or a history of infection during pregnancy.Most patients start with respiratory symptoms,and the blood virus antibodies of some patients are positive.The initial cardiothoracic ratio was less than 0.7 and the follow-up time was longer than 2 years are protective factors for the remission of the disease.