Pulmonary arterial hypertension (PAH) is a common complication in patients with congenital heart disease (CHD), particularly in those with prominent left-to-right (systemic-to-pulmonary) shunt. However, PAH can also develop after partially successful repair (due to remaining residual lesions) or with full repair of cardiac defect if it is not done in a timely manner. Persistent exposure of the pulmonary vasculature to increased blood flow and pressure may result in pulmonary obstructive arteriopathy which histopathologically is similar to those observed in idiopathic or other associated forms of PAH.
