It is important to diagnose and treat children with PAH early because of their poor prognosis if they are left untreated. It is also vital that children are referred to a specialist centre that has experience with PAH and pediatric patients as soon as possible. The introduction of new therapeutic strategies has improved prognosis for PAH in children, even though a cure is still not possible.
Children typically undergo a diagnostic approach similar to that used for adults as described in the “How is PAH diagnosed”.
Once a diagnosis of PAH in children has been confirmed, each patient requires a unique management strategy, and patients should be carefully monitored with regular follow-up visits. This individual approach is essential because children with PAH are a vulnerable and heterogeneous patient population; infants, small children and adolescents grow and develop at different paces. Their pulmonary vasculature, for example, is particularly susceptible to change during growth.