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

• Published in: Journal of the American College of Cardiology Vol. 54; no. 1; pp. S85 - S96
• Main Authors: Hoeper, Marius M., Barberà, Joan Albert, Channick, Richard N., Hassoun, Paul M., Lang, Irene M., Manes, Alessandra, Martinez, Fernando J., Naeije, Robert, Olschewski, Horst, Pepke-Zaba, Joanna, Redfield, Margaret M., Robbins, Ivan M., Souza, Rogério, Torbicki, Adam, McGoon, Michael
• Format: Journal Article
• Language: English
• Published: United States Elsevier Inc 30.06.2009; Elsevier Limited
• Subjects: Airway management; Algorithms; assessment; Cardiology; Cardiovascular; Cardiovascular disease; Chronic obstructive pulmonary disease; Comorbidity; diagnosis; Drug therapy; Emphysema; Fees & charges; Forced Expiratory Volume; Grants; Heart failure; Heart Failure - epidemiology; Hemodynamics; Humans; Hypertension, Pulmonary - classification; Hypertension, Pulmonary - diagnosis; Hypertension, Pulmonary - epidemiology; Hypertension, Pulmonary - physiopathology; Hypertension, Pulmonary - therapy; Internal Medicine; Lung diseases; Lung Diseases, Interstitial; Mortality; non-PAH pulmonary hypertension; Pharmaceutical industry; Pulmonary arteries; Pulmonary Disease, Chronic Obstructive - epidemiology; Pulmonary Disease, Chronic Obstructive - physiopathology; Pulmonary Embolism; Pulmonary hypertension; Risk Factors; Sarcoidosis; Studies; Travel; ILD; mPAP; RCT; RV; BNP; LHD; LV; RHC; PVR; assessment; DLCO; diagnosis; PCWP; SPAP; PEA; non-PAH pulmonary hypertension; CT; ERA; PAH; PH; FEV1; CTEPH; IPF; PDE-5; TR; COPD; left heart disease; systolic pulmonary arterial pressure; mean pulmonary artery pressure; pulmonary hypertension; brain natriuretic peptide; tricuspid regurgitation; interstitial lung disease; left ventricular; right ventricular; phosphodiesterase-5; idiopathic pulmonary fibrosis; chronic thromboembolic pulmonary hypertension; FEV 1; diffusion capacity of the lung for carbon monoxide; pulmonary endarterectomy; endothelin receptor antagonist; pulmonary vascular resistance; right heart catheterization; mean forced expiratory volume in 1 s; pulmonary arterial hypertension; computed tomography; pulmonary capillary wedge pressure; randomized controlled trial; chronic obstructive pulmonary disease
• ISSN: 0735-1097; 1558-3597; 1558-3597
• DOI: 10.1016/j.jacc.2009.04.008

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.