The prostacyclin
(prostaglandin I2 (PGI2)) pathway is an important therapeutic target in
pulmonary arterial hypertension (PAH). Reduced levels of PGI2 are associated
with pathogenic changes within the lung vasculature and therapies that aim to
restore PGI2 signalling have been shown to play a key role in PAH management .
Evidence to support the use of therapies targeting the PGI2 pathway was first
generated in the 1990s when intravenous epoprostenol, was demonstrated to
provide symptomatic and haemodynamic benefits and to improve survival. As a
result, epoprostenol was the first drug to be approved for PAH. The European Society of Cardiology/European
Respiratory Society (ESC/ERS) guidelines cite different levels of
recommendation and evidence for each therapy, with epoprostenol having the
highest level (IA) in patients with WHO FC III or IV disease. Intravenous
prostanoids require continuous infusion via a central venous catheter due to
their short half-lives .Serious side-effects may
occur on interruption or withdrawal of i.v. prostanoids , which can result from
pump malfunctions or if the line is erroneously compromised. A compromised line
can lead to cardiovascular collapse