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2019 updated consensus statement on the diagnosis and treatment of pediatric pulmonary hypertension: The European Pediatric Pulmonary Vascular Disease Network (EPPVDN), endorsed by AEPC, ESPR and ISHLT

Review article
Authors Georg Hansmann
Martin Koestenberger
Tero Pekka Alastalo
Christian Apitz
Eric D. Austin
Damien Bonnet
Werner Budts
Michele D'Alto
Michael A. Gatzoulis
Babar S. Hasan
Rainer Kozlik-Feldmann
R. Krishna Kumar
Astrid E. Lammers
Heiner Latus
Ina Michel-Behnke
Oliver Miera
Nicholas W. Morrell
Guido Pieles
Daniel Quandt
Hannes Sallmon
Dietmar Schranz
Karin Tran-Lundmark
Robert M.R. Tulloh
Gregor Warnecke
Håkan Wåhlander
Sven C. Weber
Peter Zartner
Published in The Journal of Heart and Lung Transplantation
Volume 38
Pages 879-901
ISSN 1053-2498
Publication year 2019
Published at Institute of Clinical Sciences, Department of Pediatrics
Pages 879-901
Language en
Keywords children, congenital heart disease, European Pediatric Pulmonary Vascular Disease Network, guidelines, pediatric, pulmonary hypertension, pulmonary vascular disease, right heart failure, right ventricle
Subject categories Pediatrics, Thoracic surgery, Respiratory Medicine and Allergy, Cardiac and Cardiovascular Systems, Paediatric cardiology


© 2019 The European Pediatric Pulmonary Vascular Disease Network is a registered, non-profit organization that strives to define and develop effective, innovative diagnostic methods and treatment options in all forms of pediatric pulmonary hypertensive vascular disease, including pulmonary hypertension (PH) associated with bronchopulmonary dysplasia, PH associated with congenital heart disease (CHD), persistent PH of the newborn, and related cardiac dysfunction. The executive writing group members conducted searches of the PubMed/MEDLINE bibliographic database (1990–2018) and held face-to-face and web-based meetings. Ten section task forces voted on the updated recommendations, based on the 2016 executive summary. Clinical trials, meta-analyses, guidelines, and other articles that include pediatric data were searched using the term “pulmonary hypertension” and other keywords. Class of recommendation (COR) and level of evidence (LOE) were assigned based on European Society of Cardiology/American Heart Association definitions and on pediatric data only, or on adult studies that included >10% children or studies that enrolled adults with CHD. New definitions by the World Symposium on Pulmonary Hypertension 2018 were included. We generated 10 tables with graded recommendations (COR/LOE). The topics include diagnosis/monitoring, genetics/biomarkers, cardiac catheterization, echocardiography, cardiac magnetic resonance/chest computed tomography, associated forms of PH, intensive care unit/lung transplantation, and treatment of pediatric PH. For the first time, a set of specific recommendations on the management of PH in middle- and low-income regions was developed. Taken together, these executive, up-to-date guidelines provide a specific, comprehensive, detailed but practical framework for the optimal clinical care of children and young adults with PH.

Page Manager: Webmaster|Last update: 9/11/2012

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