Clinical Research Directory

Inclusion Criteria: * Male or female aged 18 to 65 * Patients with congenital heart disease and pulmonary hypertension with clear diagnosis * Pulmonary vascular resistance index (PVR) in hemodynamics of right heart catheterization before surgical repair: hemodynamics of right heartcatheterization before surgery conforms to "gray zone standard" (PVR: 4-8 Wood\* m2, QP/QS \> 1.5), acute vascular reaction test meets the positive standard (PVR: \< 4 Wood\* m2) * Successfully performed surgical repair of congenital heart disease to close the defect * Sign informed consent form Exclusion Criteria: * Other causes of PAH include pulmonary hypertension associated with left heart disease (valve disease) * Other types of CHD-PAH, including Eisenmenger syndrome, PAH with small defects, etc. * After evaluating the acute pulmonary vasodilation test by right heart catheterization, the result shows that the surgical prognosis is poor, and those who are not suitable for surgical treatment should not undergo surgical treatment. * There are other surgical contraindications, including severe systemic diseases, severe mental disorders, severe cognitive dysfunction, and physical conditions that cannot tolerate surgery. * Cannot provide informed consent