Chronic thromboembolic pulmonary hypertension that is
recurrent or persistent following surgery, or is
inoperable (initiated under specialist supervision) |
Monotherapy or in combination with an endothelin
receptor antagonist for idiopathic or hereditary
pulmonary arterial hypertension, or pulmonary arterial
hypertension associated with connective tissue disease
(initiated under specialist supervision)
▶BY MOUTH
▶Adult: Initially 1 mg 3 times a day for 2 weeks,
increased in steps of 0.5 mg 3 times a day, dose to be
increased every 2 weeks, increased to up to 2.5 mg
3 times a day (max. per dose 2.5 mg 3 times a day),
increase up to maximum dose only if systolic blood
pressure 95 mmHg and no signs of hypotension, if
treatment interrupted for 3 or more days, restart at
1 mg three times daily for 2 weeks and titrate as before,
during titration, reduce dose by 0.5 mg three times
daily if systolic blood pressure falls below 95 mmHg
and patient shows signs of hypotension
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DUE TO INTERACTIONS
▶Manufacturer advises consider initial dose of 0.5 mg
3 times daily in those stable on a strong multi-pathway
cytochrome P450 and P-glycoprotein (P-gp)/breast
cancer resistance protein (BCRP) inhibitor.
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History of serious haemoptysis.
previous bronchial artery embolisation. pulmonary
hypertension associated with idiopathic interstitial
pneumonias. pulmonary veno-occlusive disease
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Autonomic dysfunction. elderly (risk of
hypotension). hypotension (do not initiate if systolic
blood pressure below 95 mmHg). hypovolaemia. severe
left ventricular outflow obstruction
CAUTIONS, FURTHER INFORMATION
▶Smoking Smoking cessation advised (response possibly
reduced); dose adjustment may be necessary if smoking
started or stopped during treatment.
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Scottish Medicines Consortium (SMC) decisions
SMC No. 1001/14
The Scottish Medicines Consortium has advised (December
2014) that riociguat (Adempas ®) is accepted for restricted
use within NHS Scotland for the treatment of chronic
thromboembolic pulmonary hypertension in adults for
whom a phosphodiesterase type-5 inhibitor is
inappropriate, not tolerated, or ineffective, and should
only be prescribed by specialists in the Scottish Pulmonary
Vascular Unit. This advice is contingent upon the
continuing availability of the Patient Access Scheme in
NHS Scotland or a list price that is equivalent or lower.
SMC No. 1056/15
The Scottish Medicines Consortium has advised (July
2015) that riociguat (Adempas ®) is accepted for restricted
use within NHS Scotland for the treatment of pulmonary
arterial hypertension in adults as specific monotherapy as
an alternative treatment option to endothelin receptor
antagonist monotherapy and should only be prescribed by
specialists in the Scottish Pulmonary Vascular Unit or by
similar specialists. This advice is contingent upon the
continuing availability of the Patient Access Scheme in
NHS Scotland or a list price that is equivalent or lower.
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