EDITORIAL

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

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
Journal of Heart and Lung Transplantation 2019, 38 (9): 879-901
31495407
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.

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