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J Am Coll Cardiol. 2013 Dec 24;62(25 Suppl):D22-33. doi: 10.1016/j.jacc.2013.10.027.

Right heart adaptation to pulmonary arterial hypertension: physiology and pathobiology.

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Department of Pneumology, VU University Medical Center, Amsterdam, the Netherlands. Electronic address:
Division of Cardiovascular Medicine, Department of Medicine, Stanford University, Stanford, California.
Department of Internal Medicine, Pulmonary Division, University of Texas Southwestern Medical Center, Dallas, Texas.
Pulmonary Hypertension and Right Heart Failure Program, Temple University Hospital, Philadelphia, Pennsylvania.
Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania.
CARIM School for Cardiovascular Diseases, Maastricht University, Maastricht, the Netherlands.
Department of Pathophysiology, Faculty of Medicine, Free University of Brussels, Brussels, Belgium.
Department of Medicine, Division of Allergy, Pulmonary and Critical Care Medicine, Vanderbilt University School of Medicine, Nashville, Tennessee.
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.
Pulmonary Hypertension Research Group, Centre de Recherche de l'Institut Universitaire de Cardiologie et de Pneumologie de Québec, Chemin Sainte-Foy, Québec, Canada.
Department of Pulmonary Circulation and Thromboembolic Diseases, Centre of Postgraduate Medical Education, ECZ, Otwock, Poland.
Division of Pulmonary and Critical Care Medicine and Victoria Johnson Lab for Lung Research, Virginia Commonwealth University, Richmond, Virginia; Johns Hopkins University, Baltimore, Maryland.


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.


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

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