RT Journal Article SR Electronic T1 Paediatric pulmonary arterial hypertension: updates on definition, classification, diagnostics and management JF European Respiratory Journal JO Eur Respir J FD European Respiratory Society SP 1801916 DO 10.1183/13993003.01916-2018 VO 53 IS 1 A1 Rosenzweig, Erika B. A1 Abman, Steven H. A1 Adatia, Ian A1 Beghetti, Maurice A1 Bonnet, Damien A1 Haworth, Sheila A1 Ivy, D. Dunbar A1 Berger, Rolf M.F. YR 2019 UL https://publications.ersnet.org//content/53/1/1801916.abstract AB Paediatric pulmonary arterial hypertension (PAH) shares common features of adult disease, but is associated with several additional disorders and challenges that require unique approaches. This article discusses recent advances, ongoing challenges and distinct approaches for the care of children with PAH, as presented by the Paediatric Task Force of the 6th World Symposium on Pulmonary Hypertension. We provide updates of the current definition, epidemiology, classification, diagnostics and treatment of paediatric PAH, and identify critical knowledge gaps. Several features of paediatric PAH including the prominence of neonatal PAH, especially in pre-term infants with developmental lung diseases, and novel genetic causes of paediatric PAH are highlighted. The use of cardiac catheterisation as a diagnostic modality and haemodynamic definitions of PAH, including acute vasoreactivity, are addressed. Updates are provided on issues related to utility of the previous classification system to reflect paediatric-specific aetiologies and approaches to medical and interventional management of PAH, including the Potts shunt. Although a lack of clinical trial data for the use of PAH-targeted therapy persists, emerging data are improving the identification of appropriate targets for goal-oriented therapy in children. Such data will likely improve future clinical trial design to enhance outcomes in paediatric PAH.State of the art and future perspectives in paediatric pulmonary hypertension with special emphasis on classification, diagnosis and treatment http://ow.ly/uVPo30mksOj