Erika B. Rosenzweig, Steven H. Abman, Ian Adatia, Maurice Beghetti, Damien Bonnet, Sheila Haworth, D. Dunbar Ivy, Rolf M. F. Berger
Columbia University and New York Presbyterian Hospital. University of Colorado and Children’s Hospital Colorado. Glenwood Children’s Heart Clinic. University Hospitals of Geneva and Lausanne. Hôpital Necker Enfants Malades, AP-HP, Université Paris Descartes. University College London. University Medical Center Groningen and University of Groningen.
United States, Canada, Switzerland, France, United Kingdom and Netherlands
European Respiratory Journal
Eur Respir J 2019; 53:
DOI: 10.1183/13993003.01916-2018
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.
Category
Review Articles Concerning Pulmonary Vascular Disease
Consensus Guidelines for Pulmonary Vascular Disease
Diagnostic Testing for Pulmonary Vascular Disease. Non-invasive Testing
Diagnostic Testing for Pulmonary Vascular Disease. Invasive Testing
Age Focus: Pediatric Pulmonary Vascular Disease
Fresh or Filed Publication: Filed (PHiled). Greater than 1-2 years since publication
Article Access
Free PDF File or Full Text Article Available Through PubMed or DOI: Yes