Pathophysiology of the right ventricle and of the pulmonary circulation in pulmonary hypertension: an update

Anton Vonk Noordegraaf; Kelly Marie Chin; François Haddad; Paul M Hassoun; Anna R Hemnes; Susan Roberta Hopkins; Steven Mark Kawut; David Langleben; Joost Lumens; Robert Naeije

doi:10.1183/13993003.01900-2018

Pathophysiology of the right ventricle and of the pulmonary circulation in pulmonary hypertension: an update

Eur Respir J. 2019 Jan 24;53(1):1801900. doi: 10.1183/13993003.01900-2018. Print 2019 Jan.

Authors

Affiliations

¹ Amsterdam UMC, Vrije Universiteit Amsterdam, Pulmonary Medicine, Amsterdam Cardiovascular Sciences, Amsterdam, The Netherlands.
² Division of Pulmonary and Critical Care Medicine, University of Texas Southwestern, Dallas, TX, USA.
³ Division of Cardiovascular Medicine, Stanford University and Stanford Cardiovascular Institute, Palo Alto, CA, USA.
⁴ Division of Pulmonary and Critical Care Medicine, Johns Hopkins University, Baltimore, MD, USA.
⁵ Division of Allergy, Pulmonary and Critical Care Medicine, Vanderbilt University Medical Center, Nashville, TN, USA.
⁶ Dept of Medicine, University of California, San Diego, La Jolla, CA, USA.
⁷ Penn Cardiovascular Institute, Dept of Medicine, and Center for Clinical Epidemiology and Biostatistics, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, USA.
⁸ Center for Pulmonary Vascular Disease, Cardiology Division, Jewish General Hospital and McGill University, Montreal, QC, Canada.
⁹ Maastricht University Medical Center, CARIM School for Cardiovascular Diseases, Maastricht, The Netherlands.
¹⁰ Université de Bordeaux, LIRYC (L'Institut de Rythmologie et Modélisation Cardiaque), Bordeaux, France.
¹¹ Dept of Cardiology, Erasme University Hospital, Brussels, Belgium.
¹² Laboratory of Cardiorespiratory Exercise Physiology, Faculty of Motor Sciences, Université Libre de Bruxelles, Brussels, Belgium.

Abstract

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.

Publication types

Review

MeSH terms

Adaptation, Physiological
Animals
Cardiac Catheterization
Echocardiography
Familial Primary Pulmonary Hypertension
Humans
Hypertension, Pulmonary / diagnosis
Hypertension, Pulmonary / physiopathology*
Hypertension, Pulmonary / therapy
Pulmonary Circulation*
Ventricular Dysfunction, Right / diagnosis
Ventricular Dysfunction, Right / physiopathology*
Ventricular Dysfunction, Right / therapy
Ventricular Function, Right*

Abstract

Publication types

MeSH terms

Grants and funding