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Executive summary. Expert consensus statement on the diagnosis and treatment of paediatric pulmonary hypertension. The European Paediatric Pulmonary Vascular Disease Network, endorsed by ISHLT and DGPK
  1. Georg Hansmann1,
  2. Christian Apitz2,
  3. Hashim Abdul-Khaliq3,
  4. Tero-Pekka Alastalo4,5,
  5. Phillip Beerbaum1,
  6. Damien Bonnet6,
  7. Karl-Otto Dubowy7,
  8. Matthias Gorenflo8,
  9. Alfred Hager9,
  10. Anne Hilgendorff10,
  11. Michael Kaestner2,
  12. Martin Koestenberger11,
  13. Juha W Koskenvuo4,5,
  14. Rainer Kozlik-Feldmann12,
  15. Titus Kuehne13,
  16. Astrid E Lammers14,
  17. Heiner Latus15,
  18. Ina Michel-Behnke16,
  19. Oliver Miera17,
  20. Shahin Moledina18,
  21. Vivek Muthurangu19,
  22. Joseph Pattathu8,
  23. Dietmar Schranz15,
  24. Gregor Warnecke20,21,
  25. Peter Zartner22
  1. 1Department of Paediatric Cardiology and Critical Care, Hannover Medical School, Hannover, Germany
  2. 2Division of Paediatric Cardiology, Children's University Hospital Ulm, Ulm, Germany
  3. 3Department of Paediatric Cardiology, Saarland University Hospital, Homburg, Germany
  4. 4Blueprint Genetics, Biomedicum Helsinki, Helsinki, Finland
  5. 5Department of Paediatric Cardiology, Children's Hospital Helsinki, University of Helsinki, Helsinki, Finland
  6. 6Unité Médico-Chirurgicale de Cardiologie Congénital et Pédiatrique, Centre de reference Malformations Cardiaques Congénitales Complexes—M3C, Hôpital Necker Enfants Malades, APHP, Université Paris Descartes, Sorbonne Paris, Paris, France
  7. 7Department of Paediatric Cardiology and Congenital Heart Disease, Heart and Diabetes Centre NRW, Bad Oeynhausen, Germany
  8. 8Department of Paediatric Cardiology, University of Heidelberg, Heidelberg, Germany
  9. 9Department of Paediatric Cardiology and Congenital Heart Disease, German Heart Centre Munich and Technical University, Munich, Germany
  10. 10Perinatal Center Grosshadern, Dr. von Haunersches Children's Hospital, Ludwig-Maximilians-University, Munich, Germany
  11. 11Divison of Paediatric Cardiology, Department of Paediatrics, Medical University Graz, Graz, Austria
  12. 12Division of Paediatric Cardiology, University Heart Center, University Medical Center Hamburg-Eppendorf, Hamburg, Germany
  13. 13German Heart Institute Berlin (DHZB), Unit of Cardiovascular Imaging, Department of Congenital Heart Disease and Paediatric Cardiology, Berlin, Germany
  14. 14Department of Paediatric Cardiology, University of Münster, Münster, Germany
  15. 15Justus-Liebig-University Giessen, Pediatric Cardiology, Paediatric Heart Center, Giessen, Germany
  16. 16Paediatric Heart Center, Division of Paediatric Cardiology, University Hospital for Children and Adolescents, Medical University Vienna, Austria
  17. 17German Heart Institute Berlin (DHZB), Department of Congenital Heart Disease and Paediatric Cardiology, Berlin, Germany
  18. 18National Paediatric Pulmonary Hypertension Service UK, Great Ormond Street Hospital for Children, London, UK
  19. 19Cardiovascular MRI Department, Great Ormond Street Hospital for Children, London, UK
  20. 20Department of Cardiothoracic, Transplantation and Vascular Surgery, Hannover Medical School, Hannover, Germany
  21. 21German Centre for Lung Research, BREATH, Hannover, Germany
  22. 22Department of Paediatric Cardiology, German Paediatric Heart Centre, Sankt Augustin, Germany
  1. Correspondence to Professor Dr Georg Hansmann, Department of Paediatric Cardiology and Critical Care, Hannover Medical School, Carl-Neuberg-Str. 1, Hannover 30625, Germany; georg.hansmann{at}gmail.com

Abstract

The European Paediatric Pulmonary Vascular Disease (PVD) Network is a registered, non-profit organisation that strives to define and develop effective, innovative diagnostic methods and treatment options in all forms of paediatric pulmonary hypertensive vascular disease, including specific forms such as pulmonary arterial hypertension (PAH)-congenital heart disease, pulmonary hypertension (PH) associated with bronchopulmonary dysplasia, persistent PH of the newborn, and related cardiac dysfunction.

Methods The writing group members conducted searches of the PubMed/MEDLINE bibliographic database (1990–2015) and held five face-to-face meetings with votings. Clinical trials, guidelines, and reviews limited to paediatric data were searched using the terms ‘pulmonary hypertensioń’ and 5–10 other keywords, as outlined in the other nine articles of this special issue. Class of recommendation (COR) and level of evidence (LOE) were assigned based on European Society of Cardiology/American Heart Association definitions and on paediatric data only, or on adult studies that included >10% children.

Results A total of 9 original consensus articles with graded recommendations (COR/LOE) were developed, and are summarised here. The topics included diagnosis/monitoring, genetics/biomarker, cardiac catheterisation, echocardiography, cardiac magnetic resonance/chest CT, associated forms of PH, intensive care unit/ventricular assist device/lung transplantation, and treatment of paediatric PAH.

Conclusions The multipaper expert consensus statement of the European Paediatric PVD Network provides a specific, comprehensive, detailed but practical framework for the optimal clinical care of children with PH.

This is an Open Access article distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/

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