Pathobiology, pathology and genetics of pulmonary hypertension: Update from the Cologne Consensus Conference 2018

Andrea Olschewski; Eva M Berghausen; Christina A Eichstaedt; Bernd K Fleischmann; Ekkehard Grünig; Gabriele Grünig; Georg Hansmann; Lars Harbaum; Jan K Hennigs; Danny Jonigk; Wolfgang M Kuebler; Grazyna Kwapiszewska; Soni S Pullamsetti; Elvira Stacher; Norbert Weissmann; Daniela Wenzel; Ralph T Schermuly

doi:10.1016/j.ijcard.2018.09.070

Pathobiology, pathology and genetics of pulmonary hypertension: Update from the Cologne Consensus Conference 2018

Int J Cardiol. 2018 Dec 1:272S:4-10. doi: 10.1016/j.ijcard.2018.09.070. Epub 2018 Sep 20.

Authors

Andrea Olschewski¹, Eva M Berghausen², Christina A Eichstaedt³, Bernd K Fleischmann⁴, Ekkehard Grünig⁵, Gabriele Grünig⁶, Georg Hansmann⁷, Lars Harbaum⁸, Jan K Hennigs⁹, Danny Jonigk¹⁰, Wolfgang M Kuebler¹¹, Grazyna Kwapiszewska¹², Soni S Pullamsetti¹³, Elvira Stacher¹⁴, Norbert Weissmann¹⁵, Daniela Wenzel⁴, Ralph T Schermuly¹⁵

Affiliations

¹ Ludwig Boltzmann Institute for Lung Vascular Research, Graz, Austria; Institute of Physiology, Medical University of Graz, Austria. Electronic address: [email protected].
² Department of Internal Medicine III, University Hospital of Cologne, Center for Molecular Medicine Cologne (CMMC), University of Cologne, Germany.
³ Centre for Pulmonary Hypertension, Thoraxclinic at the University Hospital Heidelberg, Translational Lung Research Center Heidelberg (TLRC), German Center for Lung Research (DZL), Heidelberg, Baden-Württemberg, Germany; Institute of Human Genetics, Heidelberg University, Germany.
⁴ Institute of Physiology I, Life & Brain Center, University of Bonn, Germany.
⁵ Centre for Pulmonary Hypertension, Thoraxclinic at the University Hospital Heidelberg, Translational Lung Research Center Heidelberg (TLRC), German Center for Lung Research (DZL), Heidelberg, Baden-Württemberg, Germany.
⁶ Department of Environmental Medicine, New York University School of Medicine, New York, NY, USA.
⁷ Department of Pediatric Cardiology and Critical Care, Hannover Medical School, Hannover, Germany.
⁸ University Medical Center Hamburg-Eppendorf, II Department of Medicine-Oncology, Hematology, Stem Cell Transplantation, Section of Pneumology, Hamburg, Germany.
⁹ Department of Pediatrics, the Vera Moulton Wall Center for Pulmonary Vascular Disease and the Cardiovascular Institute, Stanford University School of Medicine, CA, USA.
¹⁰ Institute of Pathology, Hannover Medical School, Hannover, Germany.
¹¹ Keenan Research Centre for Biomedical Science, St. Michael's Hospital, Toronto, Ontario, Canada; Departments of Physiology & Surgery, University of Toronto, Toronto, Ontario, Canada; Institute of Physiology, Charité-Universitätsmedizin Berlin, Berlin, Germany.
¹² Ludwig Boltzmann Institute for Lung Vascular Research, Graz, Austria; Institute of Physiology, Medical University of Graz, Austria.
¹³ Max-Planck-Institute for Heart and Lung Research, Department of Lung Development and Remodeling, Member of the German Center for Lung Research (DZL), Bad Nauheim, Germany.
¹⁴ Institute of Pathology, Medical University of Graz, Austria.
¹⁵ Excellence Cluster Cardiopulmonary System, University of Giessen Lung Center, German Center for Lung Research (DZL), Member of the German Center for Lung Research (DZL), Justus-Liebig-University, Giessen, Germany.

PMID: 30314839
DOI: 10.1016/j.ijcard.2018.09.070

Abstract

The European guidelines, which focus on clinical aspects of pulmonary hypertension (PH), provide only minimal information about the pathophysiological concepts of PH. Here, we review this topic in greater detail, focusing on specific aspects in the pathobiology, pathology and genetics, which include mechanisms of vascular inflammation, the role of transcription factors, ion channels/ion channel diseases, hypoxic pulmonary vasoconstriction, genetics/epigenetics, metabolic dysfunction, and the potential future role of histopathology of PH in the modern era of PH therapy. In addition to new insights in the pathobiology of this disease, this working group of the Cologne Consensus Conference also highlights novel concepts and potential new therapeutic targets to further improve the treatment options in PAH.

Keywords: And the potential future role of histopathology; Genetics/epigenetics; Hypoxic pulmonary vasoconstriction; Inflammation; Ion channels/ion channel diseases; Metabolic dysfunction; Pulmonary hypertension; Transcription factors.

Publication types

Review

MeSH terms

Clinical Trials as Topic / methods
Consensus Development Conferences as Topic*
Germany / epidemiology
Humans
Hypertension, Pulmonary / epidemiology
Hypertension, Pulmonary / genetics*
Hypertension, Pulmonary / metabolism
Hypertension, Pulmonary / pathology*
Inflammation Mediators / metabolism
Vasoconstriction / physiology

Substances

Inflammation Mediators