RESUMO
Progressive pulmonary arterial hypertension (PAH) is a rare condition with high morbidity and mortality. Paediatric PAH distinguishes itself from PAH in adults, but is still poorly characterized. Paediatric PAH presents itself with non-specific symptoms which often results in later diagnosis. Determination of the correct underlying diagnosis in paediatric PAH is complex, and must therefore take place at specialized centres. Paediatric progressive PAH is usually either idiopathic or associated with congenital heart disease. Pediatric PAH frequently co-occurs with dysmorphic abnormalities, which may point towards aetiological mechanisms. Recent reports suggest an improved survival and exercise tolerance due to treatment with new second-generation drugs for paediatric PAH. In the Netherlands, the care for children with PAH is centralised to guarantee the optimization of diagnosis and treatment in accordance with the most recent scientific insights.