PULMONARY ARTERIAL HYPERTENSION IN CHILDREN

Abstract

Pulmonary arterial hypertension (PAH) in the paediatric age group may be associated with a variety of underlying diseases and contributes significantly to morbidity and mortality. Pulmonary vascular remodeling in this disease leads to high pulmonary vascular resistance and subsequently to progressive right ventricular failure, low cardiac output, and death. Without treatment, median survival in children with PAH has been reported to be less than one year. In recent decades advances in basic pulmonary vascular biology have led to novel therapies for this lifethreatening disease, which include prostacyclin analogues, endothelin receptor antagonists (ERAs) and phosphodiesterase-5 inhibitors (PDE5 inhibitors).Pulmonary arterial hypertension (PAH) in the paediatric age group may be associated with a variety of underlying diseases and contributes significantly to morbidity and mortality. Pulmonary vascular remodeling in this disease leads to high pulmonary vascular resistance and subsequently to progressive right ventricular failure, low cardiac output, and death. Without treatment, median survival in children with PAH has been reported to be less than one year. In recent decades advances in basic pulmonary vascular biology have led to novel therapies for this lifethreatening disease, which include prostacyclin analogues, endothelin receptor antagonists (ERAs) and phosphodiesterase-5 inhibitors (PDE5-inhibitors).