State-of-the-Art Paper
Future Perspectives for the Treatment of Pulmonary Arterial Hypertension

https://doi.org/10.1016/j.jacc.2009.04.014Get rights and content
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Over the past 2 decades, pulmonary arterial hypertension has evolved from a uniformly fatal condition to a chronic, manageable disease in many cases, the result of unparalleled development of new therapies and advances in early diagnosis. However, none of the currently available therapies is curative, so the search for new treatment strategies continues. With a deeper understanding of the genetics and the molecular mechanisms of pulmonary vascular disorders, we are now at the threshold of entering a new therapeutic era. Our working group addressed what can be expected in the near future. The topics span the understanding of genetic variations, novel antiproliferative treatments, the role of stem cells, the right ventricle as a therapeutic target, and strategies and challenges for the translation of novel experimental findings into clinical practice.

Key Words

treatment
pulmonary arterial hypertension
PAH

Abbreviations and Acronyms

ANP
atrial natriuretic peptide
BMP
bone morphogenetic protein
BMPR2
bone morphogenetic protein receptor type 2
BNP
brain natriuretic peptide
EGFR
epidermal growth factor receptor
EPC
endothelial progenitor cell
eNOS
endothelial nitric oxide synthase
ET
endothelin
ETA
ET-1 receptor A
ETB
ET-1 receptor B
Gαs
Gα subunit
GPCR
G-protein–coupled receptor
5-HT
serotonin
IPAH
idiopathic pulmonary arterial hypertension
Kv
voltage-dependent potassium channel
MCT
monocrotaline
MI
myocardial infarction
NADPH
nicotinamide adenine dinucleotide phosphate
NO
nitric oxide
PAH
pulmonary arterial hypertension
PASMC
pulmonary artery smooth muscle cell
PDGF
platelet-derived growth factor
PET
positron emission tomography
PGI2
prostacyclin
PH
pulmonary hypertension
ROS
reactive oxygen species
RV
right ventricular
SERT
serotonin transporter
SMC
smooth muscle cell
TGF
transforming growth factor
VEGF
vascular endothelial growth factor

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