Right heart adaptation to pulmonary arterial hypertension:  physiology and pathobiology

Vonk-noordegraaf, Anton; Haddad, François; Chin, Kelly M.; Forfia, Paul R.; Kawut, Steven M.; Lumens, Joost; Naeije, Robert; Newman, John; Oudiz, Ronald J.; Provencher, Steve; Torbicki, Adam; Voelkel, Norbert F.; Hassoun, Paul M.

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Right heart adaptation to pulmonary arterial hypertension: physiology and pathobiology [Turk Kardiyol Dern Ars]

Turk Kardiyol Dern Ars. 2014; 42(1): 29-44

Right heart adaptation to pulmonary arterial hypertension: physiology and pathobiology

Anton Vonk-noordegraaf¹, François Haddad², Kelly M. Chin³, Paul R. Forfia⁴, Steven M. Kawut⁵, Joost Lumens⁶, Robert Naeije⁷, John Newman⁸, Ronald J. Oudiz⁹, Steve Provencher¹⁰, Adam Torbicki¹¹, Norbert F. Voelkel¹², Paul M. Hassoun³
¹VU University Medical Center, Department of Pneumology, Amsterdam, Netherlands
²Stanford University School of Medicine, Department of Cardiovascular Medicine, Stanford, California, USA
³University of Texas Southwestern Medical Center, Department of Internal Medicine, Pulmonary Division, Dallas, Texas, USA
⁴Temple University Hospital, Pulmonary Hypertension and Right Heart Failure Program, Philadelphia, Pennsylvania, USA
⁵University of Pennsylvania, Perelman School of Medicine, Philadelphia, Pennsylvania, USA
⁶Maastricht University, Corum Cardiovascular Diseases School, Maastricht, The Netherlands
⁷Brussels Free University School of Medicine, Department of Pathophysiology, Brussels, Belgium
⁸Vanderbilt University School of Medicine, Allergy, Pulmonary and Critical Care Division, Nashville, Tennessee, USA
⁹UCLA David Geffen School of Medicine, Liu Center for Pulmonary Hypertension, UCLA Harbor Medical Center, Biomedical Research Institute, Department of Cardiology, Torrance, California, USA
¹⁰Pulmonary Hypertension Research Group, "Centre de Recherche de l
¹¹Graduate Medical Education Center, circulatory and pulmonary thromboembolism Diseases Department, Pharmaceutical, Otwock, Poland
¹²Virginia Commonwealth University, Pulmonary and Critical Care and Victoria Johnson Lung Research Laboratory, Richmond, Virginia, USA; Johns Hopkins University, Baltimore, Maryland, USA

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 uderlying 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. (J Am Coll Cardiol 2013;62: D22–33) ª 2013 by the American College of Cardiology Foundation.

Keywords: Echocardiography, heart failure, MRI, myocardium; pulmonary artery hypertension; right ventricle

Corresponding Author: Anton Vonk-noordegraaf, Netherlands
Manuscript Language: Turkish

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