Congenital heart disease (CHD), which has an incidence of eight new cases per 1000 live births, represents one of the most common groups of congenital malformations. Pulmonary arterial hypertension (PAH) is a serious and dreaded complication, and occurs in approximately 15% of patients with CHD [1]. In fact, more than half of the cases of PAH in children are secondary to CHD (PAH-CHD) [2,3].