Treatment of pulmonary arterial hypertension in children

Pulmonary arterial hypertension (PAH) is a symptom of various pathologies and is frequently associated with progressive deterioration; PAH is an important cause of morbidity and mortality in childrenCurrent therapies for PAH target the vascular calcium channel as well as abnormalities in the endothelin, nitric oxide, and prostacyclin signalling pathwaysAdvances in basic and clinical research into PAH have led to improved understanding of disease pathogenesis and identification of novel therapeutic targetsOnly a minority of drugs for PAH are officially approved in children, because randomized controlled studies are limited by the small number of paediatric patients with PAHInterventional and surgical strategies are increasingly performed in children with severe PAH refractory to medical therapy, to relieve right ventricular pressure overload, improve left ventricular performance, and support systemic circulationDevelopments in PAH-specific therapy as well as surgical and interventional procedures for severe PAH might delay or even avoid the need for lung transplantation