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Update on pharmacotherapy for pulmonary hypertension

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Acronyms

6MWD 6-minute walk distance 

BMPR2 bone morphogenetic protein receptor type 2 

cGMP cyclic guanosine monophosphate 

CTEPH chronic thromboembolic pulmonary hypertension 

ERA endothelin receptor antagonist 

iPAH idiopathic pulmonary arterial hypertension 

NYHA New York Heart Association 

PAH pulmonary arterial hypertension 

PBS Pharmaceutical Benefits Scheme 

PDE5 phosphodiesterase type 5 

PEA pulmonary endarterectomy 

PH pulmonary hypertension 

PVR pulmonary vascular resistance 

RCT randomised controlled trial 

TCD time to clinical deterioration 

TGF-β transforming growth factor beta 

VQ ventilation–perfusion 

In contrast to the systemic circulation, the pulmonary circulation is a low-pressure circuit, which normally operates with a mean pressure below 20 mmHg. Pulmonary hypertension (PH) is defined haemodynamically by an elevated mean pulmonary artery pressure of ≥…

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