BACKGROUND To summarize our clinical experience in performing transthoracic balloon pulmonary valvuloplasty for the treatment of patients suffering from congenial pulmonary atresia with intact ventricular septum (PA/IVS). MATERIAL AND METHODS Between April 2009 and April 2016, 38 patients with PA/IVS underwent transthoracic balloon pulmonary valvuloplasty in our hospital. All of them were combined with patent ductus arteriosus, tricuspid insufficiency, and atrial septal defect or patent foramen ovale. The valvuloplasty was performed from the right ventricular outflow tract through a median sternotomy incision under TEE guidance for all cases. RESULTS Thirty-five patients were successfully discharged, and 3 patients died after the operation. The 35 surviving patients were followed up. Spo2 in the 35 patients was 88-96% after the operation. The transpulmonary valvular gradient pressure was less than or equal to 30 mmHg in 31 patients and between 36 and 52 mmHg in the other 4 patients. After the surgery, tricuspid regurgitation was significantly reduced. We found only 4 patients with moderate regurgitation, 5 patients with mild to moderate regurgitation, and mild regurgitation in the remaining 26 patients. Five patients underwent a second-stage operation, including biventricular repair in 4 patients and ligation of ductus arteriosus in 1 patient. CONCLUSIONS The application of transthoracic balloon pulmonary valvuloplasty for the treatment of PA/IVS is minimally invasive and safe, which has great significance for improving the curative effect for this condition and reducing operation mortality.