ObjectiveTo study the diagnosis and treatment of aortopulmonary window (APW) associated with severe pulmonary hypertension.MethodsThe clinical data of 23 patients with APW undergoing surgical treatment in The First Affiliated Hospital of Air Force Medical University from 2010 to 2018 were retrospectively reviewed. There were 9 male and 14 female patients. The age was 3-132 (4.63±2.14) months. The weight was 3.3-35.0 (17.3±3.6) kg.ResultsWindows were situated in the proximal of semilunar valve (type Ⅰ) in 8 patients, and distal of the aorta (type Ⅱ) in 14 patients, from proximal to distal (type Ⅲ) in only 1 patient. Eleven patients were isolated APW, the others were combined with cardiac defects. The mean pulmonary artery pressure was 68.4±7.5 mm Hg. All patients underwent surgical correction under general anesthesia and hypothermia cardiopulmonary bypass. All patients were discharged uneventfully, with an average follow-up time of 4 years. The patients showed good outcomes and no residual shunt after surgery, and the pulmonary artery pressure decreased to normal.ConclusionAPW is an uncommon congenital cardiac anomaly. The clinical presentation is an excessive left-to-right shunt, and most patients present early in life. Development of pulmonary hypertension and pulmonary vascular resistance is usually rapid. Operative treatment is indicated as soon as the diagnosis is established, regardless of the patient’s age, and most patients after surgery have a good long-term outcome.