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Pulmonary artery hypertension (PAH) is a significant risk factor in patients with congenital heart disease. If left untreated, PAH can lead to reduced quality of life and functional class due to pulmonary vascular damage and right ventricular failure (RV).The prevalence of PAH in ACHD is estimated to be between 5% and 10%. In severe cases of PAH, treatment options are generally limited to palliative...