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
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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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