Abstract: AIM:To analyze the comprehensive preoperative evaluation, refined operative procedure, and postoperative intensive care to improve the healing rate of patients with congenital heart disease (CHD) with severe pulmonary hypertension (PH). METHODS: A retrospective analysis was conducted in 280 patients with ventricular septal defect (VSD) complicated by severe pulmonary hypertension (PH) who underwent surgical treatment with extracorporeal circulation from January 2009 to December 2010. Preoperative measures included oxygen therapy and depressurization of PH with hypotensive drugs such as sodium nitroprusside or PGE1. During surgery, special attention was given to myocardial and pneumopreservation with refined surgical technique, rectification of complicated abnormality and routine repair of the tricuspid valve. Adequate postoperative intensive care included proper prolonged intubation time under 4-6 PEEP, NO-inhalation and proper sedation. RESULTS: Of the 280 patients, five patients died due to circulatory failure, low-cardiac output, and lung infection (1.78%). Length of hospitalization after operation was (13.10±5.24) days and average pulmonary pressure significantly decreased from (71±14) mmHg to (58±11) mmHg (P<0.05) postoperation and to (38±5) mmHg (P<0.05) on discharge. There were only two cases of residual shunt. Two cases with moderate regurgitation and one patient with severe regurgitation of the tricuspid valve were observed in the 265 patients who were followed for 1 year postoperatively. CONCLUSION: Good preoperative evaluation, comprehensive surgical treatment and proper postoperative intensive care may effectively improve the healing rate and quality of life of patients with CHD with PH.