Pulmonale arteriële hypertensie bij kinderen in Nederland

Conflict of interest: none declared. Financial support: none declared.

• 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.