OBJECTIVE: To investigate the improvement of left ventricular outflow tract obstruction and aortic valve function in patients with complete transposition of the great arteries with left ventricular outflow tract obstruction after operation pulse reversal. Methods A total of 549 children underwent arterial reversal at Fu Wai Cardiovascular Hospital from 2002 to 2013, of whom 42 patients had combined left ventricular outflow tract obstruction, including 31 males and 11 females, median age 12 months (7 d to 96 months); median weight 6.5 (3.5 to 26.0) kg, peripheral transcutaneous oxygen saturation 52% to 85%; left ventricular outflow tract lesion types including abnormal pulmonary valve, subvalvular septum, tunnel-like stenosis, myocardial stenosis, accessory valve tissue, and composite lesions. During the operation, different methods were adopted according to the lesion types: junctional dissection was performed for junctional adhesions, subvalvular septum was excised, hypertrophic muscle bundle or part of the ventricular septum was excised for simple myocardial stenosis, fibrous tissue and hypertrophic muscle was excised for annular or tunnel-like stenosis, paravalvular tissue or non-functional tendons were excised, and the tendons crossing to the left ventricle through the septal defect were excised and replanted. Results Mean extracorporeal circulation time ranged from 147 to 344 (193.5±73.1) min, aortic block time from 139 (109 to 305) min, ventilator use time from 36 (3 to 960) h, and ICU stay from 5 (1 to 48) d. Extracorporeal membrane oxygenation (ECMO) assisted 3 cases, all of which were successfully withdrawn. There were 2 early deaths, 1 case of multi-organ failure and 1 case of severe infection. One case died during follow-up for unknown reasons, three cases were lost to follow-up, and 36 patients were followed up for 24 (3-116) months; one case of recurrent left ventricular outflow tract obstruction was due to subvalvular confined thickened fibrous tissue, one case of mild neoaortic stenosis, 11 cases of small amount of neoaortic regurgitation, and two cases of moderate regurgitation; the median left ventricular-aortic pressure difference was 4 (2-49) mmHg at follow-up. There was a significant improvement (Z=-5.153) from the preoperative [37.2 (12.1 to 70.6) mmHg]. 91%±5% of cardiac events were waived at 1 year and 78%±8% at 5 years. Conclusion In complete transposition of the great arteries with combined left ventricular outflow tract obstruction, the severity of the obstruction needs to be assessed in conjunction with the anatomical situation and pressure difference, and with appropriate indications, satisfactory medium- and long-term results can be obtained with operational pulse transposition.