Clinical Analysis Of 143 Children With Cardiomyopathy And Their Outcomes

OBJECTIVE : The aim of this study is to retrospectively analyze the past11 years data of cardiomyopathy patients admitted in our hospital and summarize the clinical features and prognosis for better understanding of cardiomyopathy and provide reference for the diagnosis and treatment of children with cardiomyopathy.METHODS: we conducted a retrospective review of pediatric patients admitted with diagnosis of cardiomyopathy in the first affiliated hospital of Guangxi Medical University whose initial hospitalization fell between Jan 2006-Dec 2016.A total of 143 patients with cardiomyopathy diagnosed by China cardiomyopathy diagnosis and treatment recommendations of the Working Group and 1995 WHO/ISFC cardiomyopathy classification group were collected.Data such as age,sex,clinical symptoms,signs,cardiac enzymes,troponin I,ECG,cardiothoracic ratio,echocardiography,Genetic test results,NYHA heart function classification of older children,ROSSclassification in younger patients and other results were collected and summarized.Follow up was done by telephone or outpatient department with primary study endpoint included time to the combined outcome of cardiac transplantation or death.Comparative analysis of DCM and EFE.RESULTS: A total of 143 patients were collected in which 77 patients were male(53.8%),66 patients were female(46.2%),male:female ratio1.16:1,age in months from 1 to 168m(54.1±55.0)m.49 patients diagnosed DCM(34.3%),7patients diagnosed HCM(4.9%),9 patients diagnosed RCM(6.3%),4 patients diagnosed ARVC(2.8%),2 patients diagnosed LVNC(1.4%),7 patients diagnosed TIC(4.9%),and 65 patients diagnosed EFE(45.4%).Month age was significant difference between DCM and EFE(P < 0.05),gender difference was statistically insignificant(P>0.05).Majority of children were diagnosed with respiratory system symptoms,cardiac insufficiency,severe heart failure,accompanied by cardiac murmur,weakened heart sound,hepatomegaly,often associated with respiratory infection,33 patients were NYHA classification grade III\IV.8 patients were ROSS classification score more than 7 points.In laboratory examinations CK-MB were increased in 44 patients,Troponin I increased in 61 patients.There was no significant difference in the number of CK-MB and cTnI between the DCM group and EFE group(P > 0.05).ECG reports of 138 patients showed abnormal findings include ST-T segment changes,Pathological Q waves,Ventricular premature beats etc.99 cases of echocardiography showed EF<50%,114 cases showed FS<30%,and 8 caseswith intracardiac mural thrombus.The comparison of EF and FS between DCM and EFE group was statistically significant(P < 0.05).There was no significant difference between the two groups in pulmonary arterial pressure(P > 0.05).Cardiothoracic ratio >0.55 were found in 99 cases,DCM and EFE cardiothoracic ratio was statistically insignificant(P > 0.05).5 cases and their family relatives underwent cardiovascular gene detection,In 2 cases of HCM,MYH7 c.2156G> A(p.Arg719Gln)and MYBPC3 c.530G> A(p.Arg177His)two known pathogenic mutations were found;In 1 case of DCM,DMDc2473T> G(p.Trp825Gly)was found which is likely to be pathogenic mutations;One case of RCM and 1 case of TIC,gene mutation pathogenicity were not reported.By telephone or outpatient follow-up,among 142 discharged patients,only 94 patients were able to follow up completely,and follow-up time was 1-131 m,26patients died,48 patients were lost to follow up.In patients with DCM,30 patients were followed up,13 patients died,19 patients were lost to follow up;In patients with EFE,44 patients were followed up,20 patients was lost to follow up,6 patients died.None of the patients went through heart transplantation.CONCLUSION:(1).In children with cardiomyopathy,EFE is the most common type of cardiomyopathy with higher incidence in children less than 1year of age,especially in the infants less than 6 months of age.Incidence of DCM is higher mainly in older children.(2).The degree of cardiac function decline in the diagnosis of DCM is more severe than in the diagnosis of EFE.(3).Death rate of EFE is high during first 6 months after onset of disease,preventionof disease at this stage can cause better outcome and long-term prognosis.(4).In overall cardiomyopathy patients,the 1-year survival rate was 78.2%,and the3-year survival rate was 71.3%,and the 5-year survival rate was 71.3%.In patients with DCM,the 1-year survival rate was 68.2%,and the 3-year survival rate was 54.4%.In patients with EFE,the 1-year survival rate was 88.8%,and the 5-year survival rate was 88.8%.(5)Missense mutation of DMD c2473T>G(p.Trp825Gly)found in DCM might be a noval pathogenic mutation.