State-of-the-Art Paper
Diagnosis, Assessment, and Treatment of Non-Pulmonary Arterial Hypertension Pulmonary Hypertension

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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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