Paediatric pulmonary arterial hypertension: updates on definition, classification, diagnostics and management
- PMID: 30545978
- PMCID: PMC6351335
- DOI: 10.1183/13993003.01916-2018
Paediatric pulmonary arterial hypertension: updates on definition, classification, diagnostics and management
Abstract
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.
Copyright ©ERS 2019.
Conflict of interest statement
Conflict of interest: E.B. Rosenzweig reports institutional grants from Actelion, GSK, Gilead, Bayer and United Therapeutics/Lung Biotech, outside the submitted work. Conflict of interest: S.H. Abman reports grants from Shire and United Therapeutics, outside the submitted work. Conflict of interest: I. Adatia reports personal fees (for data and safety monitoring board work for selexipag trial in children, and for clinical oversight committee work for macitentan trial in children) from Actelion, personal fees (for data and safety monitoring board work for citruline trial in children) from Asklepion, and personal fees (for clinical oversight committee work for tadalafil trial in children) from Eli Lilly, outside the submitted work. Conflict of interest: M. Beghetti reports grants and personal fees from Actelion, grants, personal fees and non-financial support from Bayer Healthcare, and personal fees from GSK, Eli Lilly, MSD and Pfizer, during the conduct of the study. Conflict of interest: D. Bonnet reports personal fees from Actelion Pharmaceuticals Novartis, Bayer Healthcare and Eli Lilly, outside the submitted work. Conflict of interest: S. Haworth has nothing to disclose. Conflict of interest: D.D. Ivy: the University of Colorado School of medicine contracts with Actelion, Bayer, Lilly and United Therapeutics for D.D. Ivy to be a consultant and perform research studies. Conflict of interest: R.M.F. Berger: University Medical Center Groningen contracts with Actelion and Lilly for consultancy and advisory board activities of R.M.F. Berger.
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Comment in
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Still puzzling about a clear definition of pulmonary arterial hypertension in newborns.Eur Respir J. 2019 Mar 28;53(3):1900005. doi: 10.1183/13993003.00005-2019. Print 2019 Mar. Eur Respir J. 2019. PMID: 30923176 No abstract available.
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Response: Still puzzling about a clear definition of pulmonary arterial hypertension in newborns.Eur Respir J. 2019 Mar 28;53(3):1900135. doi: 10.1183/13993003.00135-2019. Print 2019 Mar. Eur Respir J. 2019. PMID: 30923178 No abstract available.
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