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

Research output: Contribution to journalDebate/Note/Editorial

Abstract

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.

Details

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
  • Robert M.R. Tulloh
  • Gregor Warnecke
  • Håkan Wåhlander
  • Sven C. Weber
  • Peter Zartner
Organisations
External organisations
  • Hannover Medical School
  • Medical University of Graz
  • Blueprint Genetics
  • University Hospital of Ulm
  • Paris Descartes University
  • University Hospitals Leuven
  • Second University of Napels
  • Royal Brompton Hospital
  • Aga Khan University, Pakistan
  • University Medical Center Hamburg-Eppendorf
  • Amrita Hospital
  • University of Münster
  • German Heart Centre, Munich
  • Medical University of Vienna
  • German Heart Center Berlin
  • University of Cambridge
  • Bristol Royal Hospital for Children
  • Children's University Hospital, Zurich
  • Charité - University Medicine Berlin
  • Goethe University
  • University of Bristol
  • Queen Silvia Children’s Hospital
  • German Pediatric Heart Centre /Asklepios Klinik Sankt Augustin
  • Vanderbilt University
  • Necker-Enfants Malades Hospital
  • Monaldi Hospital
  • Addenbrooke's Hospital
  • University of Gothenburg
Research areas and keywords

Subject classification (UKÄ) – MANDATORY

  • Respiratory Medicine and Allergy
  • Pediatrics
  • Cardiac and Cardiovascular Systems

Keywords

  • children, congenital heart disease, European Pediatric Pulmonary Vascular Disease Network, guidelines, pediatric, pulmonary hypertension, pulmonary vascular disease, right heart failure, right ventricle
Original languageEnglish
Pages (from-to)879-901
Number of pages23
JournalJournal of Heart and Lung Transplantation
Volume38
Issue number9
Publication statusPublished - 2019
Publication categoryResearch
Peer-reviewedYes