title: Right heart adaptation to pulmonary arterial hypertension: physiology and pathobiology.
abstract: Survival in patients with pulmonary arterial hypertension (PAH) is closely related to right ventricular (RV) function. Although pulmonary load is an important determinant of RV systolic function in PAH, there remains a significant variability in RV adaptation to pulmonary hypertension. In this report, the authors discuss the emerging concepts of right heart pathobiology in PAH. More specifically, the discussion focuses on the following questions. 1) How is right heart failure syndrome best defined? 2) What are the underlying molecular mechanisms of the failing right ventricle in PAH? 3) How are RV contractility and function and their prognostic implications best assessed? 4) What is the role of targeted RV therapy? Throughout the report, the authors highlight differences between right and left heart failure and outline key areas of future investigation.
authors.affiliation: Department of Pneumology, VU University Medical Center, Amsterdam, the Netherlands. Electronic address: a.vonk@vumc.nl.
authors.forename: Anton
authors.initials: A
authors.lastname: Vonk-Noordegraaf
authors.affiliation: Division of Cardiovascular Medicine, Department of Medicine, Stanford University, Stanford, California.
authors.forename: François
authors.initials: F
authors.lastname: Haddad
authors.affiliation: Department of Internal Medicine, Pulmonary Division, University of Texas Southwestern Medical Center, Dallas, Texas.
authors.forename: Kelly M
authors.initials: KM
authors.lastname: Chin
authors.affiliation: Pulmonary Hypertension and Right Heart Failure Program, Temple University Hospital, Philadelphia, Pennsylvania.
authors.forename: Paul R
authors.initials: PR
authors.lastname: Forfia
authors.affiliation: Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania.
authors.forename: Steven M
authors.initials: SM
authors.lastname: Kawut
authors.affiliation: CARIM School for Cardiovascular Diseases, Maastricht University, Maastricht, the Netherlands.
authors.forename: Joost
authors.initials: J
authors.lastname: Lumens
authors.affiliation: Department of Pathophysiology, Faculty of Medicine, Free University of Brussels, Brussels, Belgium.
authors.forename: Robert
authors.initials: R
authors.lastname: Naeije
authors.affiliation: Department of Medicine, Division of Allergy, Pulmonary and Critical Care Medicine, Vanderbilt University School of Medicine, Nashville, Tennessee.
authors.forename: John
authors.initials: J
authors.lastname: Newman
authors.affiliation: The David Geffen School of Medicine at UCLA, Liu Center for Pulmonary Hypertension, Division of Cardiology, Biomedical Research Institute at Harbor-UCLA Medical Center, Torrance, California.
authors.forename: Ronald J
authors.initials: RJ
authors.lastname: Oudiz
authors.affiliation: Pulmonary Hypertension Research Group, Centre de Recherche de l'Institut Universitaire de Cardiologie et de Pneumologie de Québec, Chemin Sainte-Foy, Québec, Canada.
authors.forename: Steve
authors.initials: S
authors.lastname: Provencher
authors.affiliation: Department of Pulmonary Circulation and Thromboembolic Diseases, Centre of Postgraduate Medical Education, ECZ, Otwock, Poland.
authors.forename: Adam
authors.initials: A
authors.lastname: Torbicki
authors.affiliation: Division of Pulmonary and Critical Care Medicine and Victoria Johnson Lab for Lung Research, Virginia Commonwealth University, Richmond, Virginia; Johns Hopkins University, Baltimore, Maryland.
authors.forename: Norbert F
authors.initials: NF
authors.lastname: Voelkel
authors.affiliation: Department of Internal Medicine, Pulmonary Division, University of Texas Southwestern Medical Center, Dallas, Texas.
authors.forename: Paul M
authors.initials: PM
authors.lastname: Hassoun
mesh_terms: D000222:Adaptation, Physiological / Q000502:physiology*; D000818:Animals; D065627:Familial Primary Pulmonary Hypertension; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis / Q000503:physiopathology*; D018497:Ventricular Dysfunction, Right / Q000175:diagnosis / Q000503:physiopathology*; D016278:Ventricular Function, Right / Q000502:physiology*; D020257:Ventricular Remodeling / Q000502:physiology
keywords: B type natriuretic peptide; BNP; CO; Ea; Ees; LV; MHC; MPAP; MRI; PAC; PAH; PH; PVR; RAP; RHF; RNA; RV; RVEF; arterial elastance; cardiac output; echocardiography; heart failure; left ventricular; mean pulmonary arterial pressure; myocardium; myosin heavy chain; pulmonary arterial compliance; pulmonary arterial hypertension; pulmonary artery hypertension; pulmonary hypertension; pulmonary vascular resistance; ribonucleic acid; right atrialpressure; right heart failure; right ventricle; right ventricular; right ventricular ejection fraction; ventricular elastance