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Bosentan for chronic thromboembolic pulmonary hypertension
Marco Confalonieri1, Metka Kodric, Cinzia Longo
1Department of Pneumology, University Hospital of Trieste, Str. Di Fiume 447, 34149 Trieste, Italy. marco.confalonieri@aots.sanita.fvg.it
Insights
Bosentan, an endothelin receptor antagonist, shows safety and potential for inoperable chronic thromboembolic pulmonary hypertension (CTEPH). While not improving walking distance in trials, its use is encouraged pending further research for this severe condition.
Area of Science:
- Cardiology
- Pulmonary Medicine
- Pharmacology
Background:
- Chronic thromboembolic pulmonary hypertension (CTEPH) is a severe, underdiagnosed condition with pulmonary hypertension due to organized thrombi.
- CTEPH shares arteriolar lesions with idiopathic pulmonary arterial hypertension (PAH).
- Pulmonary endarterectomy is the primary treatment, but not suitable for all CTEPH patients, and no specific drug is currently licensed.
Purpose of the Study:
- To evaluate the efficacy and safety of bosentan, an endothelin receptor antagonist, in patients with chronic thromboembolic pulmonary hypertension (CTEPH).
- To assess bosentan's role as a potential therapeutic option for inoperable CTEPH cases.
Main Methods:
- Review of preliminary uncontrolled/unblinded studies and randomized controlled trials (RCTs) investigating bosentan in CTEPH.
- Analysis of hemodynamic effects and 6-minute walking distance as primary endpoints in RCTs.
Main Results:
- Bosentan demonstrated a positive hemodynamic effect in CTEPH patients.
- The drug failed to show significant improvement in the 6-minute walking distance in one key RCT.
- Bosentan was found to be safe for use in CTEPH patients.
Conclusions:
- Bosentan is considered safe and its use is encouraged for inoperable chronic thromboembolic pulmonary hypertension (CTEPH) based on available data.
- Further randomized controlled trials are necessary to establish bosentan as a standard treatment for CTEPH.
Abstract:
Chronic thromboembolic pulmonary hypertension (CTEPH) is a severe and underdiagnosed disease characterized by progressive hypertension secondary to organized thrombi in pulmonary vessels. The arteriolar lesions of CTEPH are similar to those seen in idiopathic pulmonary arterial hypertension (PAH). Surgical disobliteration of the vessels by pulmonary endarterectomy is the therapy of choice but this is not suitable for all cases. To date, there is no licensed specific drug for CTEPH. Endothelin-1, a vasoconstrictor and promoter of cell proliferation, is involved in the pathogenesis of both CTEPH and PAH. Bosentan, the first oral dual endothelin receptor antagonist, has been shown to be effective in PAH. Preliminary uncontrolled and/or unblinded studies reported efficacy of bosentan in CTEPH, and the only randomized, controlled trials showed a positive hemodynamic effect but failed its other co-primary end point, namely the improvement of 6-min walking distance. Nevertheless, bosentan has been shown to be safe and the data from most literature encourage its use for inoperable CTEPH. However, further randomized controlled trials are needed to definitively establish bosentan as a standard drug for CTEPH.
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