Abstract: Objective To investigate the clinical features of Takayasu arteritis (TA) with heart failure in children. Methods TA patients complicated with heart failure admitted to Affiliated Children's Hospital of Capital Institute of Pediatrics from January 2013 to December 2019 were retrospectively analyzed. Results A total of 5 female patients were included and the age of onset was from 2 to 14 years old. Four patients started with the manifestations of heart failure including chest distress, palpitation, dizziness, fatigue and sweat. Vascular murmur, weak pulse or pulseless and hypertension were found in 5, 4 and 4 patients respectively. All 5 patients had valve involvement with 3 of multi-valve involvement. There were 4 cases of mitral valve regurgitation, 3 cases of aortic valve regurgitation with 2 of ascending aortic dilatation, 3 cases of tricuspid regurgitation and 3 cases of coronary artery dilatation. The clinical type of 5 patients was type Ⅲ. Four patients received treatment of glucocorticoid and/or immunosuppressive agents and 2 patients were treated with Infliximab injection. One patient was lost to follow-up and 4 patients were followed up for 6 to 48 months who had improved to varying degrees with normal inflammatory indicators and no new lesions, except for the irreversible heart injury. One case was complicated with intractable hypertension and prepared to receive surgical treatment. Conclusion To get early diagnosis of cardiac involvement and treatment in time, echocardiography should be performed regularly in children with TA. In addition to the primary heart disease, alert should be taken to the possibility of cardiac involvement of TA for patients starting with heart symptoms. TA patients with aortic and renal artery stenosis were always complicated with hypertension.