Paediatric pulmonary arterial hypertension (PAH) is a challenge for the paediatric anaesthetist. Due to its high morbidity and mortality, support should be provided by a dedicated team. Understanding the pathophysiology of PAH allows performing an appropriate therapeutic approach. In case of high vascular pulmonary resistance, the main objectives of anaesthetic management are to maintain an optimal pulmonary flow and to avoid the decrease in systemic arterial pressure. Haemodynamic monitoring is essential to detect the onset of an acute PAH crisis but also to give direct information on the efficacy of treatment.
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