Objective To investigate the value of echocardiography in the diagnosis of double chambered ventricle by analyzing the data of children with double chambered ventricle and reviewing the related literatures.Methods 22 children with double chambered ventricle diagnosed by echocardiography were included in this study. We retrospectively analyzed the cardiac structure and hemodynamic change in echocardiography. We used two-dimensional echocardiography to evaluate the structure of the ventricle in order to identify the position, shape of the abnormal muscle bundles. And we used Doppler echocardiography to detect the direction and velocity of abnormal blood flow. Together with other diagnostic examinations and surgical results，the characteristics of double chambered ventricle were analyzed.Results 20 patients with double chambered right ventricle were diagnosed by echocardiography. All of them had VSD and 9 patients were also had other congenital heart disease. 15 of the 20 cases were confirmed by surgery. And another 5 cases were diagnosed as right ventricular outflow tract stenosis in operation. 2 patients were diagnosed as double chambered left ventricle by echocardiography，of which one case confirmed by MRI, the other one was diagnosed as dilated cardiomyopathy.Conclusion Echocardiography plays an important role in the diagnosis of double chambered ventricle.