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STATE-OF-THE-ART PAPER

Diagnosis, Assessment, and Treatment of Non-Pulmonary Arterial Hypertension Pulmonary Hypertension

Marius M. Hoeper, MD^_*,_*, Joan Albert Barberà, MD, Richard N. Channick, MD, Paul M. Hassoun, MD, Irene M. Lang, MD^||, Alessandra Manes, MD, PhD^¶, Fernando J. Martinez, MD^#, Robert Naeije, MD, PhD^_**, Horst Olschewski, MD, Joanna Pepke-Zaba, PhD, Margaret M. Redfield, MD, PhD, Ivan M. Robbins, MD^||||, Rogério Souza, MD^¶¶, Adam Torbicki, MD, PhD^## and Michael McGoon, MD^_***

^_* Department of Respiratory Medicine, University of Hannover Medical School, Hannover, Germany
Department of Respiratory Medicine, Hospital Clinic, CIBERES, University of Barcelona, Barcelona, Spain
Division of Pulmonary and Critical Care Medicine, University of California, San Diego, La Jolla, California
Department of Medicine, Pulmonary and Critical Care Medicine, Johns Hopkins University School of Medicine, Baltimore, Maryland
^|| Division of Cardiology, Medical University of Vienna, Vienna, Austria
^¶ Institute of Cardiology, University of Bologna, Bologna, Italy
^# Division of Pulmonary and Critical Care Medicine; University of Michigan Health System, Ann Arbor, Michigan
^_** Department of Pathophysiology, Free University of Brussels, Brussels, Belgium
Department of Pulmonology, Medical University of Graz, Graz, Austria
Pulmonary Vascular Disease Unit, Papworth Hospital, Cambridge, United Kingdom
Department of Cardiology, Mayo Clinic, Rochester, Minnesota
^|||| Department of Allergy, Pulmonary and Critical Care Medicine; Vanderbilt University Medical Center, Nashville, Tennessee
^¶¶ Pulmonary Department, Heart Institute, University of São Paulo Medical School, São Paulo, Brazil
^## Department of Chest Medicine, Institute of Tuberculosis and Lung Diseases, Medical University of Warsaw, Warsaw, Poland
^_*** Department of Cardiovascular Medicine, Mayo Clinic, Rochester, Minnesota

Manuscript received February 6, 2009; accepted April 15, 2009.

^_* Reprint requests and correspondence: Dr. Marius M. Hoeper, Department of Respiratory Medicine, Hannover Medical School, 30623 Hannover, Germany (Email: hoeper.marius{at}mh-hannover.de).

The 4th World Symposium on Pulmonary Hypertension was the first international meeting to focus not only on pulmonary arterial hypertension (PAH) but also on the so-called non-PAH forms of pulmonary hypertension (PH). The term "non-PAH PH" summarizes those forms of PH that are found in groups 2 to 5 of the current classification of PH, that is, those forms associated with left heart disease, chronic lung disease, recurrent venous thromboembolism, and other diseases. Many of these forms of PH are much more common than PAH, but all of them have been less well studied, especially in terms of medical therapy. The working group on non-PAH PH focused mainly on 4 conditions: chronic obstructive lung disease, interstitial lung disease, chronic thromboembolic PH, and left heart disease. The medical literature regarding the role of PH in these diseases was reviewed, and recommendations regarding diagnosis and treatment of PH in these conditions are provided. Given the lack of robust clinical trials addressing PH in any of these conditions, it is important to conduct further studies to establish the role of medical therapy in non-PAH PH.

Key Words: diagnosis • assessment • non-PAH pulmonary hypertension

Abbreviations and Acronyms   BNP = brain natriuretic peptide   COPD = chronic obstructive pulmonary disease   CT = computed tomography   CTEPH = chronic thromboembolic pulmonary hypertension   DLCO = diffusion capacity of the lung for carbon monoxide   ERA = endothelin receptor antagonist   FEV1 = mean forced expiratory volume in 1 s   ILD = interstitial lung disease   IPF = idiopathic pulmonary fibrosis   LHD = left heart disease   LV = left ventricular   mPAP = mean pulmonary artery pressure   PAH = pulmonary arterial hypertension   PCWP = pulmonary capillary wedge pressure   PDE-5 = phosphodiesterase-5   PEA = pulmonary endarterectomy   PH = pulmonary hypertension   PVR = pulmonary vascular resistance   RCT = randomized controlled trial   RHC = right heart catheterization   RV = right ventricular   SPAP = systolic pulmonary arterial pressure   TR = tricuspid regurgitation

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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; 30(20): 2493 - 2537. [Full Text] [PDF]