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Dhillon S et al. – Oral bosentan therapy was beneficial and generally well tolerated in patients with mildly symptomatic PAH. In a well designed, placebo–controlled trial in adolescents and adults with mildly symptomatic PAH, pulmonary vascular resistance was significantly reduced with bosentan relative to placebo, but the 6–minute walk distance did not increase significantly. Similarly, pediatric patients (most of whom had mildly symptomatic PAH) in a small uncontrolled trial experienced some improvement in hemodynamic variables with bosentan, but did not experience a significant increase in exercise capacity. Adverse events associated with bosentan were consistent with those seen in other indications, with major concerns being the potential for teratogenicity and hepatotoxicity, for which regular liver function monitoring is recommended. Overall, considering the progressive nature of PAH, bosentan extends the treatment options available to patients with mildly symptomatic PAH.


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