Abstract：Objective To retrospectively analyze the clinical characteristics of children with primary cardiomyopathy.Methods A retrospective study was carried out in 125 pediatric patients with primary cardiomyopathy who had been admitted to Children’s Hospital of Fudan University between January 1,1997 and December 31,2016.Alltheir clinical manifestations, results of laboratory examinations (laboratory tests,chest X-ray,ECG, echocardiography,etc.) and other information were retrospectively analyzed.Nineteen patients with DCM who had been admitted between 2007 and 2016 were followed-up to analyze their prognostic implications.Results Dilated cardiomyopathy (DCM) was the most common type of cardiomyopathy. Seventy-three of these cases(58.4%)were infants, and RCM mostly occurred in children over 1 year old. Children with DCM,NVM and HCM presented cardiac signs, pulmonary signs and hepatomegaly, while RCM patients mainly presented clinical manifestations due to right heart failure. As compared with normal, DCM and NVM patients showed significantly higher LVEDD-Z.LAD-Z was the largest in RCM patients, followed by that of DCM and NVM patients. LAD-Z decreased in patients of HCM. EF-Z and FS were significantly reduced in patients with DCM, NVM or EFE, but did not change significantly in HCM or RCM patients. Children with DCM hospitalized between 2007 and 2016 were followed up. The median age, age at death and disease duration were respectively 60.3 months, 63.8 months and 8.0 months in dead children. The cause of death was mainly refractory heart failure and malignant arrhythmias. Univariate analysis showed that increased cTnI was a prognostic factor, while NT-proBNP and LVEDD-Z could affect the prognosis of DCM.Conclusions The most common type of primary cardiomyopathy in our study is DCM. Cardiomyopathy mainly strikes infants under one year old, and boys are more vulnerable than girls.The level of cTnI is a prognostic factor of DCM, and NT-proBNP and LVEDD-Z may be prognostic factors as well. Refractory heart failure and malignant arrhythmia are possibly the main cause of death in DCM.
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