Insight into Pulmonary Arterial Hypertension Associated with Congenital Heart Disease (PAH-CHD): Classification and Pharmacological Management from a Pediatric Cardiological Point of View

Compared with adult patients with pulmonary hypertension (PH), pulmonary vascular disease is characterized by complex heterogeneity in pediatric patients. The Nice PH classification does not completely characterize or individualize any subgroup of pediatric PH. This is in contrast to the Panama classification, in which prenatal and fetal origins of many pulmonary vascular diseases in neonates and children, perinatal pulmonary vascular maladaptation, prenatal and postnatal pulmonary vascular mal-development, and pulmonary vascular hypoplasia are included. Currently, the updated treatment algorithm for adults with pulmonary arterial hypertension (PAH), including PAH associated with congenital heart disease (PAH-CHD) and idiopathic PAH, etc. has been reported. It has been suggested to treat FC III patients with Eisenmenger syndrome (ES) with bosentan. However, there is no evidence-based treatment algorithm for children with PAH-CHD. Moreover, it is necessary to develop a more comprehensive algorithm in which multiple specific pediatric risk factors are determined, and the critical goal of treatment should be to permit normal activities without the need to self-limit in children with PAH-CHD. Together, the beneficial data on specific-target pharmacologic interventions are still quite preliminary, and large trials are warranted. Specifically, the extrapolation of the other forms of the disease, such as ES, should be undertaken carefully.