Paediatric pulmonary arterial hypertension: updates on definition, classification, diagnostics and management.

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State: Public
Version: Final published version
License: CC BY-NC 4.0
Serval ID
serval:BIB_7F24E41BA134
Type
Article: article from journal or magazin.
Publication sub-type
Review (review): journal as complete as possible of one specific subject, written based on exhaustive analyses from published work.
Collection
Publications
Institution
Title
Paediatric pulmonary arterial hypertension: updates on definition, classification, diagnostics and management.
Journal
The European respiratory journal
Author(s)
Rosenzweig E.B., Abman S.H., Adatia I., Beghetti M., Bonnet D., Haworth S., Ivy D.D., Berger RMF
ISSN
1399-3003 (Electronic)
ISSN-L
0903-1936
Publication state
Published
Issued date
01/2019
Peer-reviewed
Oui
Volume
53
Number
1
Pages
1801916
Language
english
Notes
Publication types: Journal Article
Publication Status: epublish
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.
Pubmed
Web of science
Open Access
Yes
Create date
10/01/2019 17:40
Last modification date
20/10/2020 5:23
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