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

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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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