Long-Term (5 Years) Effects of Bosentan in Patients With Pulmonary Arterial Hypertension

Right Ventricular Function Predicts Clinical Response to Specific Vasodilator Therapy in Patients with Pulmonary Hypertension

“…showed an initial positive clinical response to bosentan therapy in both adult and pediatric populations of PAH, response that diminished over time with a return of the clinical parameters to the baseline values in 1–2 years. Still, several more recent studies found a maintained benefit from vasodilator therapy for up to 5 years, although, even in these studies, a significant proportion of the patients experienced clinical deterioration during the follow‐up—up to 59% of patients being declared as treatment failure after 5‐year follow‐up . During the 14‐month follow‐up span of our study, 53% of patients improved or maintained a stable clinical course.…”

“…Still, few studies addressed the combined endpoint of mortality and morbidity. Avellana et al . found that Nt‐proBNP and especially levels of this biomarker after 1 year of bosentan therapy carry most significant predictive value with respect to maintained clinical response.…”

Bosentan therapy for pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension: A systemic review and meta‐analysis

“…The fact that whether these positive effects sustain in long time remained undefined. So far, some open‐label extension studies of the RCTs and retrospective cohort, which observed for 1–5 years, have confirmed long‐term benefit of bosentan; whereas, for CTEPH patients, our study showed that only CI and PVR were improved after bosentan treatment. There was no additional benefit for CTEPH from bosentan treatment in terms of exercise capacity, functional class, or reduction of clinical worsening.…”

“…Theoretically, the efficacy would be more evident in advanced disease stage than those with mild symptoms. Indeed, the effect of bosentan on walking distance improvement seemed minor in functional class‐II PAH patients from the ERALY study than in those with functional class‐III/IV from other trials . Third, only 2 RCTs concerning CTEPH were identified and small sample size might be an issue.…”

Congenital Heart Disease and Pulmonary Hypertension

“…This benefit is more robust in patients with PAH-CHD without Down syndrome. [118][119][120][121] The predominant adverse reaction of bosentan is increased hepatic transaminases, which can be seen up to w10% of patients. 105,116 However, the distinct benefit is that it is an oral agent and simple to administer.…”