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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

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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