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Riociguat (Adempas) : management of inoperable chronic thromboembolic pulmonary hypertension.
- Author(s):
- Canadian Agency for Drugs and Technologies in Health, issuing body
- Title(s):
- Riociguat (Adempas) : management of inoperable chronic thromboembolic pulmonary hypertension.
- Series:
- Common drug review
- Country of Publication:
- Canada
- Publisher:
- Ottawa (ON) : Canadian Agency for Drugs and Technologies in Health, 2015 Jul.
- Description:
- 1 online resource : illustrations.
-
Language:
- English
- Electronic Links:
- https://www.ncbi.nlm.nih.gov/books/NBK349365/
- Summary:
- Chronic thromboembolic pulmonary hypertension (CTEPH) is a disease subtype of pulmonary hypertension. It is a progressive disease characterized by the presence of non-resolving or recurrent thrombi distributed within the pulmonary arteries. This can obstruct or occlude the luminal space, eventually leading to increased pulmonary vascular resistance (PVR), pulmonary hypertension, and right-sided heart failure. Although the exact etiology of CTEPH remains poorly understood, it may arise following an initial episode of acute pulmonary embolism (PE); however, up to 60% of CTEPH patients have not had any antecedent episode of acute PE. The epidemiology of CTEPH is likewise not well established. Some surveillance data estimate CTEPH to occur in 0.1% to 0.5% of patients surviving an initial episode of acute PE; however, the true incidence of CTEPH is likely to be higher, owing to an unknown number of undetected cases either presenting occultly or latently in the setting of acute PE or through non-venous thromboembolism etiologies. The current gold standard treatment intervention in CTEPH is surgery, specifically, pulmonary endarterectomy (PEA), which has the potential to be curative; however, there is no universally accepted criteria for operability. For patients who are not eligible for surgical intervention (up to 50%) or who refuse it, there are several classes of medical therapies indicated for pulmonary arterial hypertension (PAH) that have been used off-label as monotherapy in CTEPH patients. These include endothelin receptor antagonists (ERAs), phosphodiesterase-5 (PDE-5) inhibitors, and prostanoids (including prostacyclins and prostacyclin analogues). Riociguat is a first-in-class drug of the soluble guanylate cyclase stimulator class of drugs, which works by producing vasorelaxation independent of the endogenous vasodilatory effects of nitrous oxide. It is also the first drug to be marketed in Canada for the treatment of CTEPH. Riociguat is initiated at a dose of 1.0 mg orally three times a day and adjusted by 0.5 mg increments every two weeks (according to systemic systolic blood pressure readings) to a maximum dose of 2.5 mg three times a day. Riociguat has a Health Canada indication for the management of inoperable CTEPH (World Health Organization [WHO] Group 4), or persistent or recurrent CTEPH after surgical treatment in adults aged e 18 years with WHO functional class II or III pulmonary hypertension. Reimbursement is being sought by the manufacturer in accordance with this indication. The objective of this systematic review was to evaluate the beneficial and harmful effects of riociguat for the treatment of patients with WHO functional class II or III CTEPH who are deemed inoperable or have persistent or recurrent CTEPH after surgical treatment.
- MeSH:
- Canada
Chronic Disease
Hypertension, Pulmonary/drug therapy*
Pulmonary Embolism/drug therapy*
Pyrazoles/therapeutic use
Pyrimidines/therapeutic use
Risk Assessment
Treatment Outcome
- Publication Type(s):
- Review
- Notes:
- Includes bibliographical references.
- Other ID:
- (OCoLC)949986788
- NLM ID:
- 101680385 [Electronic Resource]