Objective:To summarize the effect of one and a half ventricle repair in the treatment of Ebstein’s Anomaly.Methods:The data of 149 patients diagnosed with Ebstein’s Anomaly and received with surgical treatment in Pediatric Cardiac Surgery Department of Beijing Anzhen Hospital affiliated to Capital Medical University from January 2010 to December 2018 were continuously collected, and the survival rate, reoperation rate and tricuspid regurgitation were followed up in the medium-long term.Results:There were 68 males and 81 females, with a median age of 5.58 years. Patients were divided into Biventricular repair group and one and a half ventricle repair group. The operative age was significantly younger in the one and a half ventricle repair group (4.15 years vs. 6.71 years, P=0.019). There were also significant differences in patiens’ body length[(107.70±31.28)cm vs. (123.20±35.22)cm, P=0.014]and body weight[(19.69±12.22)kg vs. (29.65±20.41)kg, P=0.001], between the two groups, which may be related to the severity of the disease and the need for early surgical intervention. However, there was no significant difference between the two groups in preoperative cyanosis, hemoglobin level, cardiac function, arrhythmia, and common cardiac malformations such as atrial septal defect. Notably, the proportion of preoperatively complicated pulmonary stenosis (10.81% vs. 1.79%, P=0.016) and right ventricular dysplasia (16.22% vs. 3.57%, P=0.008) was significantly higher in the one and a half ventricle repair group. In intraoperative and postoperative indicators, as one and a half ventricle repair under the collateral circulation, extracorporeal circulation time was slightly longer [(125.51±37.35)min vs. (100.44±25.24)min, P<0.001], and other indicators such as aortic cross-clamp time, endotracheal intubation time, length of hospital stay, and mid-term follow-up results, including mid-term mortality, reoperation rate, cardiac function and valvular regurgitation, there was no significant difference between the two groups. However, the proportion of hospital mortality in half ventricular therapy group was slightly higher, which may be related to the poor right heart function and postoperative recovery difficulties. Conclusion:Good follow-up results have been achieved in the treatment of two surgical therapy. Patients with right ventricular dysplasia and pulmonary artery stenosis should be paid more attention to. Pulmonary artery pressure and pulmonary vascular development should be evaluated before surgery to make preparations for one and a half ventricle repair.