| Objective:By summarizing the clinical date of children admitted to our hospital for 11 years with endocardial fibroelastosis(EFE),we can improve the cognition of EFE and provide the basis for the early diagnosis and treatment of endocardial fibroelastosis.Methods:This study conducted a retrospective review of 66 pediatric patients admitted with diagnosis of endocardial fibroelastosis in our hospital fell between May 2009 to December 2019.The diagnosis of all included patients accord with the diagnosis of Viral myocarditis(draft)by the 1980 National nine provinces and municipalities myocarditis Cooperation Group.Date such as age,sex,clinical symptoms,cardiac biomarkers,BNP,NT-pro BN,electrocardiogram,chest X-ray,echocardiography,treatment and prognosis,modified Ross classification of younger children.According to the prognosis,the children were divided into the death group and the survival group,and SPSS21.0statistical software was used to analyze the general situation,clinical manifestations andsigns,auxiliary examination results,treatment methods and prognosis of the children.In addition,30 cases of congenital heart disease merging endocardial thickening were divided into 4 cases of congenital heart disease with obstruction of left ventricular outflow tract and 26 cases of congenital heart disease with left to right shunt.By comparing the two groups of clinical manifestations and auxiliary examination results,we can analyze whether endocardial thickening was caused by primary disease.Results:1.General information : There were 66 EFE children,32 males(48.5%)and 34(51.5%)females,the male to female ratio of 1:1.06,the age of onset ranged from 21 days to 3.2 years,the average 8.23 ± 6.89 months.The average age of survival group with 55 cases was 8.15±7.08 months,the average age of death group with 11 cases was8.65±6.09 months,and there was no significant statistical difference in two groups(P >0.05).30 cases of congenital heart disease merging the endocardial thickening was divided into 26 cases of volume load increased group(16 cases of atrial septal defects,3cases of ventricular septal defect,2 cases of patent ductus arteriosus,5 cases of atrial septal defects combined with patent ductus arteriosus)and 4 cases of pressure load increased group(2 cases of aortic stenosis,2 cases of aortic valve stenosis).15 cases of men and 15 cases of women,the age of onset ranged from 15 days-2.7 years,the average age 5.40±3.42 months in pressure load increased group and the average 4.71±6.45 months in volume load increased group.2.Clinical manifestations and signs: Respiratory symptoms and circulatory symptoms performance were the most common initial symptoms,32 cases(48.5%)had cough,20 cases(30.3%)had shortness of breath,and 8 cases(12.1%)had cyanosis,8cases(12.1%)had anorexia,7 cases(10.6%)had fever,7 cases(10.6%)had wheezing,5 cases(7.6%)had oliguria/edema and 5 cases(7.6%)had obstruction/runny nose.7cases(10.6%)only had abnormal imaging findings without any other symptoms.There were other rare causes including moaning,yellowing of the skin,hissing,sweating,pale complexion in 34 cases.The clinical sign mainly included hepatomegaly(63.6%).The main complications were respiratory tract infection(39 cases,59.1%),arrhythmia(32cases,48.5%)and cardiogenic shock(6 cases,9.1%).The modified Ross classification method was used in 66 children,including 16 cases children without heart failure,24 cases with mild heart failure,17 cases with moderate heart failure and 9 cases with severe heart failure.In 30 cases of congenital heart disease merging the endocardial thickening,pressure load increased group were mainly characterized by shortness of breath(75%),cough(50%),wheezing(25%),anorexia(25%),moaning(25%),cry noisy(25%),fever(25%),while the volume load increased group were mainly characterized by shortness of breath(34.6%),cough(34.6%),feeding difficulties(23.1%),wheezing(19.2%),cyanosis(15.4%),heart enlargement(15.4%),fever(15.4%),etc.Hepatomegaly was common in the clinical signs,while cardiogenic shock was also common in the volume load increased group(23.1%).3.Laboratory dates:(1)Cardiac biomarkers(CKMB?CTn I?CTn I),B-type natriuretic peptide(BNP)and N-terminal pro B type natriuretic peptide(NT-pro BNP): In laboratory examination CKMB increased in 32 patients while 23 cases in survival groups and 9 cases in death group.CKMB in survival group was lower in the death group(P < 0.05).Cardiac Troponin increased in 48 patients while 40 cases in survival groups and 8 cases in death group.There were no statistically significant differences between two groups(P > 0.05).BNP increased in 8 patients,and NT-pro BNP increased in 49 patients while 39 cases in survival groups and 10 cases in death group.The level of NT-pro BNP in death group was significantly higher than that in survival group(P < 0.05).(2)Chest radiography: There were 64 cases(97.0%)of heart shadow enlargement among 66 cases and average cardiothoracic ratio was 0.67(0.5-0.86),among which 7cases(10.6%)of cardiothoracic ratio in 0.55-0.59,12 cases(18.2%)in 0.60-0.65,25cases(37.9%)in 0.66-0.70,and 20 cases(30.0%)of cardiothoracic ratio > 0.70.More than half of the cases had a cardiothoracic ratio greater than 0.65.There were 2 cases of pulmonary congestion(3.0%)and 1 case of pleural effusion(1%).Among the 30 cases of congenital heart disease merging the endocardial thickening,the cardiothoracic ratio in the pressure load increased group was 0.64-0.69 with an average cardiothoracic ratio of 0.67±0.021.In the volume load increased group,the cardiothoracic ratio was0.47-0.76 with an average cardiothoracic ratio of 0.66±0.062.(3)Electrocardiogram(ECG): 59 cases(89.4%)of the patients were abnormal including ST-T segment changes(35 cases,53.0%),left ventricular hypertrophy(27cases,40.0%),ventricular premature contraction(20 cases,30.3%),atrial premature contraction(21 cases,26.5%),right ventricular hypertrophy(13 cases,19.7%),QT prolongation(7 cases,10.6%),atrial tachycardia(7 cases,10.6%),atrial flutter(5 cases,7.6%),pre-excitation syndrome(5 cases,7.6%)and atrial fibrillation(4 cases,6.1%).Among the 30 cases of congenital heart disease merging the endocardial thickening,the pressure load increased group mainly manifested as left ventricular hypertrophy(75%),right ventricular hypertrophy(75%),ST-T segment changes(50.0%)and QT prolongation(25%),while the volume load increased group was mainly manifested as ST-T segment changes(61.5%),left ventricular hypertrophy(23.1%),premature ventricular contraction(23.1%),premature atrial contraction(23.1%),right ventricular hypertrophy(19.2%),QT prolongation(15.4%)and atrial tachycardia(11.5%).(4)Ultrasonic cardiogram(UCG): 59 cases(89.4%)had decreased LVEF,52 cases(96.3%)had FS less than 30%.Left ventricular enlargement and endocardial thickeningwere observed in all cases,with a thickness of 2.9-7.0mm and diffuse thickening of the endocardium were observed in 15 cases.Atrioventricular valve regurgitation was found in 64 cases(97.0%),mainly in the mitral and tricuspid valves.Pulmonary hypertension ranged from 30 to 81 mm Hg in 13 patients(19.7%).Pericardial effusion was found in 6patients(9.1%).In comparison between the two groups,LVPWd of the survival group was significantly lower than that of the death group(P < 0.05),while LVEF and FS of the survival group were significantly higher than that of the death group(P <0.05).There was no significant difference in LVDd,LVDs,IVSd and endocardial thickness between the two groups(P > 0.05).There was a certain correlation between endocardial thickness with LVDd(r=0.316,P < 0.05)and LVPWd(r=0.271,P < 0.05),but no significant correlation with LVEF and FS(P >0.05).Among the 30 cases of congenital heart disease merging the endocardial thickening,endocardial thickness of the pressure load increased group was 2.5-4.6mm,with an average of 3.53±0.95 mm,mainly with 2 cases of the posterior wall of the left ventricle and 2 cases of diffuse thickening of the endocardium.The endocardial thickness of volume load increased group was 2.7-6.8mm,with an average of 4.60±1.18 mm.The frequency of thickening endocardium in different areas mainly was the posterior wall of the left ventricle(23cases),diffuse thickening of the endocardium(3 cases),lateral wall(2 cases),and anterior wall(1 case).LVEF decreased in 2 cases(50%)of the pressure load increased group and 16 cases(61.5%)of the volume load increased group.In the pressure load increased group,2 patients(50%)had pulmonary arterial hypertension with pulmonary artery pressure of 31-72 mm Hg,and in the volume load increased group,8 patients(30.8%)had pulmonary arterial hypertension with pulmonary artery pressure of41-75 mm Hg.4.Treatment: The patients accepted treatments including digitalis,diuretics,ACEI,vasodilator agents,adrenocorticotropic hormone,IVIG,?-adrenergic blocker,anti-arrhythmia drugs and anti-infection drugs.5.Prognosis: When 66 children were discharged,55 of them survived and 11 died.The age of onset in the death group ranged from 3.7 months to 1.8 years.Fifty-five patients were followed up at the outpatient or by telephone for 5 months to 10 years,among which 5 died(9.1%),8 lost to follow-up(14.5%)and 42 survived.Among the dead children,4 died within 1 year after discharge,and 1 died due to sudden convulsions and respiratory failure in the 5th year after discharge.15 cases(35.7%)were clinically cured and the drug was discontinued.15 cases(35.7%)showed clinical improvement and 5 patients stopped the drug by themselves whose medical compliance was poor.Among the 30 cases of congenital heart disease merging the endocardial thickening,1 of the 4 patients in the pressure load increased group died(25%),and 8 of the 26 patients in the volume load increased group died(30.8%),with an average age of onset 7.04±11.24 months.Conclusion:1.The onset age of EFE is mostly within 1 year old,especially in infants less than6 months old.2.EFE has a variety of clinical manifestations.The most common of first symptoms in EFE are respiratory symptoms and circulatory symptoms.The clinical manifestations often have no specificity,so early diagnosis is difficult.3.The cardiac biomarkers and NT-pro BNP often increase in EFE which often indicate poor prognosis.4.EFE mainly involves in the left ventricle,which is characterized by enlargement of the left ventricle,decrease of LVEF,thickening of the endocardium which often involves in the posterior wall of the left ventricle.There was a positive correlation between endocardial thickness and heart size.5.The treatment of EFE mainly includes digitalis,diuretics,hormone and IVIG.And the treatment time of children with EFE is long.If long-term standard treatment can be adhered to,the long-term prognosis will be better.6.Congenital heart disease(atrial septal defect,ventricular septal defect,patent ductus arteriosus,aortic stenosis,aortic valve stenosis)can be combined with primary endocardial fibroelastosis with a high mortality. |
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