Clinical Study Of Dilated Cardiomyopathy In Children

?Object?This study was to explore the etiology and prognosis of DCM in the following three aspects:the clinical features of children with dilated cardiomyopa--thy(DCM)patients;the relationship between the general situation of the known mutations and the prognosis in children with idiopathic DCM;the relationship between the proinflammatory cytokines and UCG index and its founction of evaluation prognostic with newly diagnosed DCM.?Methods?To collect DCM patients' the clinical data who were hospitalized in our hospital in January 2010 to December 2014 was done to selecte patients by two-dimensional ultrasound cardiogram(UCG)for the detection of cardiac function to three groups:cardiac function recovery group,not restore group and death group.The recovery group refers to two successive review(interval greater than or equal to 1month)LVEF?55%;the death group:a variety of causes of death in follow-up;the group not recovery: in addition to the death,LVEF did not reach the recovery standard were included in the group;To collect the clinical data of DCM patients who were visited from October 2014 to February 2016 and were selected for DCM related gene examination and UCG examination;To collect the idiopathic DCM patients who were treated from July 2015 to January 2016.Collecting the data of UCG and the detection of proinflammatory cytokines.Proinflammatory cytokines include TNF-alpha,IL-1,IL-4,IL-6,IL-8,IL-10.According to the different grades of cardiac function,and LVEF<30% and ?30%,up from 2 months to LVEF improved ?5% and<5%,compare the correlation.?Outcome?1.In January 2010 to December 2014,there were 194 cases of DCM were selected and included in the study.Male were 113 cases(58.2%).The median age at onset was 1years.The recovery group was 45 cases(23.20%),no recovery group was 65 cases(33.51%),84 cases in death group(43.29%).There were 133 cases(68.56%)?2 years old,and the recovery group,the no recovery group,the death group each accounted for32.3%,32.3% and 35.3%.> 2 years of the recovery group,the no recovery group,the death group each accounted for about 3.3%,36.1% and 60.7%.Onset age?2 years old,the recovery group were significantly more than those without recovery group and death group(P <0.05).The cardiac function of III ~ IV respectively accounted for about 2%,22.4% and 75.5%(P=0.000)at the first diagnosis.2.Left ventricular end diastolic diameter(LVDD),left ventricular end systolic diameter(LVDS)in recovery group was significantly lower than that of non recovery group and death group(P < 0.05).LVEF,left ventricular short axis shortening rate(LVFS)of recovery group were significantly higher than that of the no recovery group and the death group(P all <0.05),there was no significant difference between the recovery group and the death group(P>0.05).There were 2 cases and 9 cases of severe reflux in the no recovery group and the death group respectively.And the difference was not significant(P>0.05).3.There were 162 cases(83.51%)patients with ST-T change in 194 cases,46 cases with ventricular arrhythmia(23.71%).The ventricular arrhythmias in recovery group was significantly less than that of the patients in the no recovery group and the death group(P all <0.05).There was no significant difference between the no recovery group and the death group(P>0.05).4.87(44.85%)of 194 patients received CMR examination.The incidence of LGE in the recovery group,non recovery group and death group were 0%,5.41% and 21.74%(P=0.252),respectively.After a week of treatment,the serum NT proBNP according to whether less than division of 3500pg/ml.Among the three groups compared no statistical difference(P=0.367);cTnI according to whether less than division of0.2ug/L.Among the three groups compared no statistical difference(P = 0.610).5.All patients were followed up for an average of 19.76 ±16.22 months.Recovery time was 9.62±6.73 months(2 to 25.33 months).The recovery group LVEF increased 5%for an average of 1.94 months,no recovery group was 5.69 months.In recovery group,LVEF followed up for 6 months and 1 year recovery rates were 42.22% and 77.78%.6.Multivariate analysis showed that age >2.0 years old(OR=17.064,95%CI3.494-83.171,P=0.000),cardiac function III ~ IV level(OR=17.711,95%CI2.229-140.704,P= 0.007),MR severe reflux(OR=3.762,95%CI1.209-11.706,P = 0.022)are the independent risk factors that the cardiac function can not be returned to normal.7.In October 2014 to February 2016,there were 71 patients with DCM met the inclusion criteria.The average follow-up time was 19.75±18.13 months(2~72months).56.34% were men.By 60 known DCM causative gene screening,we found that 59 patients(83.10%)with DCM have causative mutations,of which 80% of the existence of the phenomenon of parental genetic.Genetic mode is mainly autosomal dominant(69.49%);autosomal recessive inheritance(5.08%),X-autosomal inheritance(1.69%).Familial DCM were 10%.59 patients with recovered cardiac function in patients were 17 cases(28.8%),39 cases without recovery(66.1%),3cases in death group(5.1%).There were no significant difference compared with no genetic screening found patients(P > 0.05).8.We found 31 kinds of gene mutation.Ranked in the top three were MYH6(9.72%),MYH7(9.72%)and DSP(8.5%),including missense mutation 75.41%,base deletion 11.48%,nonsense 4.92% mutations,splice site mutations 4.92%,repeat mutations 3.28%.9.48 patients(11 cases recovered,34 cases not recover,3 cases died)were detected to a gene mutation,9 cases(5 cases of recovery,4 cases did not recover)were detected two mutations,2 cases(1 cases recovery,1 case did not recover)were examined to three gene mutation.10.Relatives of the patients who carrying the mutant gene,excepting 1 cases of male patients with the same gene mutation and also DCM patients,the rest of the patients were not in the incidence of relatives.11.In July 2015 to January 2016,24 cases were collectde,and 20 cases of normal control group were treated as the control group.The DCM group was significantly increased the level of tumor necrosis factor(TNF-?)(P<0.05)compared with the control group:TNF-? was significantly higher in heart function grade III-IV than the normal control group(P < 0.01),significantly higher than the heart function grade I-II group(P < 0.05).The level of TNF-? in heart function in I-II group was significantly higher than that in normal control group(P<0.05).12.There was a significantly negative correlation between the level of LVEF,LVFS and TNF-?(P<0.05).The level of TNF-? and interleukin-6(IL-6),interleukin-8(IL-8)and heart function grade into significant positive correlation(P<0.05).13.The proinflammatory cytokines(except IL-10)levels in the group of LVEF?30%were higher than that of group LVEF>30%.Among them,the level of TNF-? in the two groups was statistically significant(P<0.01).14.13 patients of 24 patients were followed up for more than 2 months.The LVEF results were compared with the LVEF results at the first visit,and the patients were divided into two groups according to the difference of LVEF?5% or not.There was no significant difference between the two groups.?Conclusion?1.After appropriate treatment,idiopathic DCM patients with LVEF returned to normal were 23.19%.Nearly 80% of patients in the recovery group in 1 years to return to normal LVEF.5.67% of patients with LVEF and LVDD were returned to normal.2.?2 year old DCM patients were about 32.33% LVEF normal;>2 years of age is only 3.28%;3.Multivariate analysis showed that age >2.0 years old,cardiac function III ~ IV level,MR severe reflux are the independent risk factors that the cardiac function can not be returned to normal.4.In idiopathic DCM patients,83.10% were found to carry a pathogenic gene DCM,the incidence rate is located in the top three were MYH6,MYH7 and DSP.29% of carrying the DCM gene in patients were with LVEF to return to normal.5.There were 80% of the patients in the DCM who had genetic mutations in the same gene mutations that were detected in their relatives.The detection rate of familial DCM was 10%.6.There was a significantly negative correlation between the level of LVEF,LVFS and TNF-?(P<0.05).The level of TNF-? and interleukin-6(IL-6),interleukin-8(IL-8)and heart function grade into significant positive correlation(P<0.05).7.Effect of proinflammatory cytokine in the evaluation of prognosis and whether can become for treatment on the basis of need longer follow-up times and accumulated more cases confirmed.