Abstract: Objective To investigate the clinical features and long-term outcomes of childhood Takayasu arteritis patients with heart failure. Methods A total of 25 consecutive childhood Takayasu arteritis patients complicated with heart failure who were hospitalized at Fuwai Hospital, Chinese Academy of Medical Sciences from January 1, 2003 to December 31, 2017 were enrolled. The demographic, clinical, laboratory, imaging features and treatment plans were collected. The outcome was first onset of acute heart failure or readmission due to worsening heart failure or all-cause death. Kaplan-Meier survival curve was used to assess the overall and event-free survival. Results The average onset age of childhood Takayasu arteritis was（13.50±3.04） years and 96% were female. The median(P₂₅-P₇₅) diagnostic delay time was 1.04（0.46-3.52） years. The common clinical manifestations included dyspnea（100%）, bruits（88%）, hypertension（68%） and blood pressure asymmetry in the extremities（60%）. The median(P₂₅-P₇₅) N-terminal pro-brain natriuretic peptide（NT-proBNP） was 3 504.0（1 215.8-9 439.9）ng/L. Renal vascular hypertension（48%） and descending aortic stenosis（32%） were the most common causes of heart failure in childhood Takayasu arteritis patients. For treatment, 80% of patients received glucocorticoid-based therapy. Only 40% patients received the revascularization of the target arteries. At a median(P₂₅-P₇₅) follow-up of 6.65（2.97-10.52） years, 7（28%） patients were rehospitalized or died due to the exacerbation of heart failure, and 4（16%） cases died, the cause of death was all heart failure. The 5-year event-free and overall survival rate were 72.2% and 91.7%, respectively. Conclusions The most common causes of heart failure in childhood Takayasu arteritis are renovascular hypertension and descending aortic stenosis. The 5-year event-free survival rate is low. Enhancing the early recognition of this disease and deepening the understanding of the disease process may help to identify the disease timely, so as to intervene in time and then improve the prognosis.