STATE-OF-THE-ART PAPER
Diagnosis and Assessment of Pulmonary Arterial Hypertension
David B. Badesch, MD*,*,
Hunter C. Champion, MD, PhD ,
Miguel Angel Gomez Sanchez, MD ,
Marius M. Hoeper, MD ,
James E. Loyd, MD||,
Alessandra Manes, MD, PhD¶,
Michael McGoon, MD#,
Robert Naeije, MD, PhD**,
Horst Olschewski, MD,
Ronald J. Oudiz, MD and
Adam Torbicki, MD, PhD
* Divisions of Pulmonary Sciences and Critical Care Medicine and Cardiology, University of Colorado Health Sciences Center, Denver, Colorado
Division of Cardiology, Johns Hopkins University, Baltimore, Maryland
Heart Failure and Pulmonary Hypertension Unit, Hospital Universitario Doce de Octubre, Madrid, Spain
Department of Respiratory Medicine, University of Hannover Medical School, Hannover, Germany
|| Department of Medicine, Vanderbilt University School of Medicine, Nashville, Tennessee
¶ Institute of Cardiology, University of Bologna, Bologna, Italy
# Mayo Clinic College of Medicine, Rochester, Minnesota
** Departments of Pathophysiology and Cardiology, Erasme Academic Hospital, Free University of Brussels, Brussels, Belgium
Pulmonology Division, University Clinic of Internal Medicine, Medical University Graz, Graz, Austria
Liu Center for Pulmonary Hypertension, Los Angeles Biomedical Research Institute, Harbor-UCLA Medical Center, Torrance, California
Department of Chest Medicine, Institute of Tuberculosis and Lung Diseases, Medical University of Warsaw, Warsaw, Poland
Manuscript received February 6, 2009;
accepted April 15, 2009.
* Reprint requests and correspondence: Dr. David B. Badesch, Divisions of Pulmonary Sciences and Critical Care Medicine, Pulmonary Hypertension Center, University of Colorado Denver, Leprino Building, Room 536 or Box 957, 12401 East 17th Avenue, Aurora, Colorado 80045 (Email: David.Badesch{at}uchdenver.edu).
The diagnosis and assessment of pulmonary arterial hypertension is a rapidly evolving area, with changes occurring in the definition of the disease, screening and diagnostic techniques, and staging and follow-up assessment. The definition of pulmonary hypertension has been simplified, and is now based on currently available evidence. There has been substantial progress in advancing the imaging techniques and biomarkers used to screen patients for the disease and to follow up their response to therapy. The importance of accurate assessment of right ventricular function in following up the clinical course and response to therapy is more fully appreciated. As new therapies are developed for pulmonary arterial hypertension, screening, prompt diagnosis, and accurate assessment of disease severity become increasingly important. A clear definition of pulmonary hypertension and the development of a rational approach to diagnostic assessment and follow-up using both conventional and new tools will be essential to deriving maximal benefit from our expanding therapeutic armamentarium.
Key Words: pulmonary arterial hypertension • diagnosis • assessment
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Abbreviations and Acronyms
| | 6MWD = 6-min walk distance | | BNP = brain natriuretic peptide | | CMR = cardiac magnetic resonance | | CO = cardiac output | | CPET = cardiopulmonary exercise testing | | FC = functional class | | HR = heart rate | | IPAH = idiopathic pulmonary arterial hypertension | | LV = left ventricle/ventricular | | mPAP = mean pulmonary arterial pressure | | NIH = National Institutes of Health | | NT-proBNP = N-terminal pro-brain natriuretic peptide | | NYHA = New York Heart Association | | PAH = pulmonary arterial hypertension | | PAP = pulmonary arterial pressure | | PH = pulmonary hypertension | | PVR = pulmonary vascular resistance | | RA = right atrial/atrium | | RAP = right atrial pressure | | RCT = randomized controlled trial | | RHC = right heart catheterization | | RV = right ventricle/ventricular | | SSc = systemic sclerosis | | TAPSE = tricuspid annular plane systolic excursion | | TIPG = tricuspid insufficiency peak gradient | | TJV = tricuspid jet velocity | | ULN = upper limit of normal |
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C. G. Elliott, R. J. Barst, W. Seeger, M. Porres-Aguilar, L. M. Brown, R. T. Zamanian, and L. J. Rubin
Worldwide Physician Education and Training in Pulmonary Hypertension: Pulmonary Vascular Disease: The Global Perspective
Chest,
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[Abstract]
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I. Blanco, E. Gimeno, P. A. Munoz, S. Pizarro, C. Gistau, R. Rodriguez-Roisin, J. Roca, and J. A. Barbera
Hemodynamic and Gas Exchange Effects of Sildenafil in Patients with Chronic Obstructive Pulmonary Disease and Pulmonary Hypertension
Am. J. Respir. Crit. Care Med.,
February 1, 2010;
181(3):
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[Abstract]
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B. A. Boilson, J. A. Schirger, and B. A. Borlaug
Caveat medicus! Pulmonary hypertension in the elderly: a word of caution
Eur J Heart Fail,
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[Abstract]
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L. P. Badano, C. Ginghina, J. Easaw, D. Muraru, M. T. Grillo, P. Lancellotti, B. Pinamonti, G. Coghlan, M. P. Marra, B. A. Popescu, et al.
Right ventricle in pulmonary arterial hypertension: haemodynamics, structural changes, imaging, and proposal of a study protocol aimed to assess remodelling and treatment effects
Eur Heart J Cardiovasc Imaging,
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[Abstract]
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The Task Force for the Diagnosis and Treatment of, N. Galie, M. M. Hoeper, M. Humbert, A. Torbicki, J-L. Vachiery, J. A. Barbera, M. Beghetti, P. Corris, S. Gaine, et al.
Guidelines for the diagnosis and treatment of pulmonary hypertension
Eur. Respir. J.,
December 1, 2009;
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K. R. Stenmark, B. Meyrick, N. Galie, W. J. Mooi, and I. F. McMurtry
Animal models of pulmonary arterial hypertension: the hope for etiological discovery and pharmacological cure
Am J Physiol Lung Cell Mol Physiol,
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[Abstract]
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Authors/Task Force Members, N. Galie, M. M. Hoeper, M. Humbert, A. Torbicki, J.-L. Vachiery, J. A. Barbera, M. Beghetti, P. Corris, S. Gaine, et al.
Guidelines for the diagnosis and treatment of pulmonary hypertension: The Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS), endorsed by the International Society of Heart and Lung Transplantation (ISHLT)
Eur. Heart J.,
October 2, 2009;
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M. Humbert and L. J. Rubin
Combining evidence and experience in pulmonary hypertension
Eur. Respir. Rev.,
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