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

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...

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Main Authors: Olschewski, Andrea (Author) , Berghausen, Eva M. (Author) , Eichstaedt, Christina (Author) , Fleischmann, Bernd K. (Author) , Grünig, Ekkehard (Author) , Grünig, Gabriele (Author) , Hansmann, Georg (Author) , Harbaum, Lars (Author) , Hennigs, Jan K. (Author) , Jonigk, Danny (Author) , Kübler, Wolfgang Michael (Author) , Kwapiszewska, Grazyna (Author) , Pullamsetti, Soni S. (Author) , Stacher, Elvira (Author) , Weissmann, Norbert (Author) , Wenzel, Daniela (Author) , Schermuly, Ralph T. (Author)
Format: Article (Journal) Conference Paper
Language:English
Published: 20 September 2018
In: International journal of cardiology
Year: 2018, Volume: 272, Pages: 4-10
ISSN:1874-1754
DOI:10.1016/j.ijcard.2018.09.070
Online Access:Verlag, Volltext: https://doi.org/10.1016/j.ijcard.2018.09.070
Verlag, Volltext: http://www.sciencedirect.com/science/article/pii/S0167527318356614
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Author Notes: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

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520 |a 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. 
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