TY - JOUR
T1 - Pathophysiology of the right ventricle and of the pulmonary circulation in pulmonary hypertension
T2 - An update
AU - Noordegraaf, Anton Vonk
AU - Chin, Kelly Marie
AU - Haddad, François
AU - Hassoun, Paul M.
AU - Hemnes, Anna R.
AU - Hopkins, Susan Roberta
AU - Kawut, Steven Mark
AU - Langleben, David
AU - Lumens, Joost
AU - Naeije, Robert
N1 - Funding Information:
Conflict of interest: A. Vonk Noordegraaf reports grants and speaker fees from Actelion, MSD and GSK, outside the submitted work. K.M. Chin reports personal fees for consulting work on clinical trials from Actelion, grants (paid to institution) from Ironwood and Sonivie, personal fees for consulting work for a clinical registry from University of California San Diego, and research grants from the NIH, outside the submitted work. F. Haddad has nothing to disclose. P.M. Hassoun has nothing to disclose. A.R. Hemnes reports personal fees from Actelion, Bayer, Complexa and United Therapeutics, and grants from the CMREF and NIH, outside the submitted work; and in addition has a patent issued: Annamometer (oral mechanism for detection of end-tidal CO2; not referenced in this work). S.R. Hopkins is funded by the NIH via research grants to study the pulmonary circulation. S.M. Kawut reports non-financial travel support from the ATS and Pulmonary Hypertension Association, grants from Actelion, United Therapeutics, Gilead, Lung Biotech, Bayer and Mallinkrodt, and grants and non-financial support from the CMREF, outside the submitted work and paid to his university; and has served in an advisory capacity (for grant review and other purposes) for United Therapeutics, Akros Pharmaceuticals, GSK and Complexa, Inc., without financial support or in-kind benefits. D. Langleben reports grants, personal fees and non-financial support from Actelion and Bayer, personal fees from United Therapeutics and Merck, and grants from Northern Therapeutics, outside the submitted work. J. Lumens has nothing to disclose. R. Naeije has nothing to disclose.
Publisher Copyright:
Copyright © ERS 2019. This article is open access and distributed under the terms of the Creative Commons Attribution Non-Commercial Licence 4.0.
PY - 2019/1/1
Y1 - 2019/1/1
N2 - The function of the right ventricle determines the fate of patients with pulmonary hypertension. Since right heart failure is the consequence of increased afterload, a full physiological description of the cardiopulmonary unit consisting of both the right ventricle and pulmonary vascular system is required to interpret clinical data correctly. Here, we provide such a description of the unit and its components, including the functional interactions between the right ventricle and its load. This physiological description is used to provide a framework for the interpretation of right heart catheterisation data as well as imaging data of the right ventricle obtained by echocardiography or magnetic resonance imaging. Finally, an update is provided on the latest insights in the pathobiology of right ventricular failure, including key pathways of molecular adaptation of the pressure overloaded right ventricle. Based on these outcomes, future directions for research are proposed.
AB - The function of the right ventricle determines the fate of patients with pulmonary hypertension. Since right heart failure is the consequence of increased afterload, a full physiological description of the cardiopulmonary unit consisting of both the right ventricle and pulmonary vascular system is required to interpret clinical data correctly. Here, we provide such a description of the unit and its components, including the functional interactions between the right ventricle and its load. This physiological description is used to provide a framework for the interpretation of right heart catheterisation data as well as imaging data of the right ventricle obtained by echocardiography or magnetic resonance imaging. Finally, an update is provided on the latest insights in the pathobiology of right ventricular failure, including key pathways of molecular adaptation of the pressure overloaded right ventricle. Based on these outcomes, future directions for research are proposed.
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U2 - 10.1183/13993003.01900-2018
DO - 10.1183/13993003.01900-2018
M3 - Article
C2 - 30545976
AN - SCOPUS:85060540102
SN - 0903-1936
VL - 53
JO - European Respiratory Journal
JF - European Respiratory Journal
IS - 1
M1 - 1801900
ER -