| Objective:To summarize and analyze the clinical effect and prognosis of long term follow up of long term treatment of children with EFE,and to investigate the incidence,treatment and outcome of children with EFE.From the clinical symptoms after treatment,left ventricular size,left ventricular ejection fraction(LVEF),left ventricular endocardial thickness,high voltage recovery and growth is affected by several aspects were analyzed.Methods:A retrospective analysis from January 2006 to October 2016 in the second hospital,hebei medical university pediatrics clear diagnosis of endocardial elastic fiber hyperplasia and 52 cases hospitalized with clinical cases of late and outpatient follow-up data.Results: There were 27 boys in this group,25 girls,Onset age from 2months to 6 years,the average age of onset was 8 months,0-6 months of onset in 20 cases(38.5%),6 months-1 years age of onset in 19 cases(36.5%),more than 1 years old patients with onset in 13 cases of disease(25.0%).Admitted to the hospital for respiratory tract infections(mostly pulmonary infection)and(or)cardiac dysfunction,hospitalized at the time of diagnosis.There is no specific treatment for EFE,clinical use of strong heart,diuretic,vasodilator and other joint anti heart failure treatment,at the same time,the use of glucocorticoid and nutritional myocardial drug support treatment,children under 2 years old with Human Immunoglobulin for Intravenous Injection immunomodulator(IVIG)treatment.The mortality rate of the disease is high,and the course of disease is long,need to take medication for a long time,regular follow-up review,patients with high compliance requirements.After discharge to continue regular medication treatment in our hospital pediatric outpatient follow-up review 46 patients on a regular basis,the follow-up rate was 88.5%(46/52),out of 10 cases,the rate of missed diagnosis was 11.5%(6/52).Outpatient follow-up time was 1 months after treatment,2 months,3months,6 months,9 months,1 years,1 years and 6 months,2 years,2 years and 6 months,3 years,3 years / 1 times a year.During follow-up,22 patients(22/46)had left ventricular high voltage,left ventricular high voltage recovery in most children at the last follow-up.Arrhythmia occurred in 17 cases,with the improvement of the condition of EEF and symptomatic treatment good recovery after reasonable,the follow-up treatment in 15 cases(15/22)left ventricular high voltage recovery,recovery rate(68.2%),7 cases were not restored,the ECG was mainly left atrium,left ventricular high voltage,ST segment and T wave inversion,performance as cardiomyopathy.Pearson test was used to analyze the correlation between EF and recovery of children with left ventricular high voltage,The correlation coefficient is equal to 0.214,P=0.257,the result is P>0.05,the results showed no correlation,and the results did not exclude the smaller sample size,can not deny the high voltage and no correlation between left ventricular EF,day after the accumulation of more clinical cases,expand the sample size can be analyzed.46 cases of echocardiography showed left ventricular enlargement,decreased left ventricular ejection fraction,43 cases showed thickening echo enhancement,the left ventricular ejection fraction and left ventricular ejection fraction were significantly improved after treatment,2 cases of children with mitral valve prolapse,mitral regurgitation,mitral regurgitation,combined with this symptom in children with EFE clinical recovery time is long,the treatment effect is poor,and ultimately died.Treatment of left ventricular size after 1 years(LV)returned to normal in 16 cases,the recovery rate was 34.8%(16/46),after 3 years of treatment LV returned to normal in 27 cases,the recovery rate was 58.7%(27/46),after 6 years of treatment LV returned to normal in 35 cases,the recovery rate was 76.1%(35/46),11 cases had not returned to normal in 4 cases of death,the other 7 cases,the left ventricular ejection fraction is slightly larger,but the normal daily life,general activity is not affected.1 years after treatment,left ventricular ejection fraction(LVEF)of33 cases returned to normal,the average LVEF number is 57.78% ±4.66,the 3year after treatment,left ventricular ejection fraction was recovered in 39 patients,the average LVEF number is 59.79% ± 4.28,follow up to 6 year after treatment,the left ventricular ejection fraction was recovered in 40 patients,the average LVEF number is 62.48% ± 4.01.The 1 year after treatment,the recovery rate of LVEF was 71.7(33/46),the 3 year after treatment,the recovery rate was 84.8%(39/46),the 6 year after treatment,the recovery rate was 87%(40/46).After 1 years of treatment,the recovery rate of endocardial thickness was 32.6%(15/46 cases),after 3 years of treatment,the recovery rate of endocardial thickness was 47.8%(22/46),all patients were followed up to 6 years after treatment,and the normal rate of intimal thickness was 87.0%(40/46),after long term treatment,the thickness of endocardium gradually became thin and returned to normal.Pearson test was used to analyze the correlation between endocardial thickness and EF,the correlation coefficient is equal to 0.214,P=0.257,the result is P>0.05,results there was no correlation,the results do not rule out with small sample size,day after accumulation of more clinical cases,the correlation analysis can be carried out after enlarging the sample size.The treatment initial height and weight of children were slightly lower than those of normal children,there was no significant difference between the final height and weight of children with normal age,after 3 years of treatment,the height and weight of children were compared with those of the same age children,and the standard deviation was P>0.05,the difference was not statistically significant.After 10 years of age,height,weight and normal height and weight of children of the same age,the standard deviation was P>0.05,the difference was not statistically significant.The whole group of children with 1 cases of EFE patients with glycogen storage disease,1 patients with propionic acidemia,children with heritage metabolic disease recovery than children in general less,17 cases with arrhythmia,with the improvement in treatment of arrhythmia in EFE patients is also gradually improved,disappeared.All the patients were followed up for 46 cases of children with a total of 5cases of death,among the 3 patients in the treatment 2 months after death,3 months after the treatment to a higher risk for death time,death within 3months after treatment,3 cases were in high risk,not to spend time with high mortality rate,the other 2 cases children were complicated with congenital anterior mitral valve prolapse,prolong life of the patients after treatment,treatment for a long time,but because of the congenital valvular malformation of the end failed to reverse.After 1 years of treatment,0 cases were cured,43 cases improved,the improvement rate was 93.5%(43/46),3 cases died within1 years,for 1 years the mortality rate was 7.5%(3/46).After 3 years of treatment,15 cases were cured,the cure rate was 32.6%(15/46),1 to 3 years no death in children,treatment of 3 year mortality rate is still 7.5%(3/46).After 6 years of treatment,24 cases were cured,the cure rate was52.2%(24/46),5 cases died,the mortality rate was 10.9% for 6 years(5/46),the total cure rate of 89.1%(41/46).Conclusion:1 Primary endocardial fibroelastosis occurs mostly in children under 1years old,with respiratory tract infection(a lung infection)and(or)heart failure treatment.2 The EFE specification improved after treatment is the earliest clinical symptoms,LVEF,followed by LV,left ventricular high voltage,thickness of endocardium returned late,endocardial thickness and left ventricular high voltage may not be associated with EF,the results do not rule out the small sample and the relevant,for days after the accumulation of clinical cases,expand the sample size can be analyzed.3 With congenital heart valve problems in treatment for a long time,poor effect,high mortality rate,some patients with arrhythmia,With the treatment of EFE and antiarrhythmic treatment of arrhythmia after a good recovery,a very small number of patients with glycogen storage disease,genetic metabolic disease propionic acidemia,combined genetic and metabolic disease in children with poor prognosis than in general.4 EFE for a long time,the condition improved above 1 years,recovery ofmore than 3 years,3 months after the treatment in the highest risk of death,EFE children adhere to long-term reasonable and standardized medication for regular outpatient follow-up,the long-term prognosis effect is good. |
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