Long-term oral bosentan treatment in patients with pulmonary arterial hypertension related to congenital heart disease: a 2-year study

Abstract

Long-term bosentan treatment in patients with PAH related to CHD is safe and induces clinical stability and improvement, but the objective exercise values appear to slowly return to baseline. Larger studies on long-term endothelin receptor antagonism including quality of life assessment are needed to evaluate the therapeutic role of bosentan in this population.

Pulmonary Hypertension in Congenital Shunts

“…Long-term treatment with bosentan improved symptoms and aortic oxygen saturation, WHO functional class, maximal and sub-maximal exercise capacity, Borg dyspnoea index, mean PAP, pulmonary blood flow and PVR in a patient with plastic bronchitis following Fontan. 79 In a recent small study, oxygen saturation improved in 5/9 patients during a 16-week treatment period with bosentan. 80 The single endothelin receptor antagonists ambrisentan and sitaxentan are also used in the management of PAH; however, no data are available describing their use in Fontan patients.…”

Adult patients with pulmonary arterial hypertension due to congenital heart disease: a review on advanced medical treatment with bosentan

“…30 Monitoring of the international normalized ratio in the study of Apostolopoulou did not show interference. 34 …”

“…32 In the other two studies with a long-term follow-up no significant rises in liver transaminases were reported. 33,34 Furthermore, the induction of hepatic enzymes can make hormonal contraception unreliable. To conclude, aminotransaminases levels of more than three times upper normal limits need further evaluation.…”

“…Functional class also improved during follow-up (P = 0.001). Two long-term studies that showed a short-term efficacy followed by gradual return to baseline were performed by Apostolopoulou et al and Duffels et al 26,34 The first study showing stabilization was by Apostolopoulou et al with two year follow-up using treadmill exercise testing, using an exercise protocol described Northridge et al 35 Most patients (68%) were cyanotic and all patients had severe right ventricular dilatation and hypertrophy on echocardiography. After the initial improvement at 16 weeks of treatment in maximal and submaximal exercise, all exercise parameters at two years seem to be slowly returning to baseline.…”

Congenital Heart Disease and Pulmonary Hypertension

“…110 Long-term data have shown that the improvement on bosentan can be maintained without safety or tolerability issues. [111][112][113][114][115][116] A more recent randomized controlled trial of bosentan in patients with NYHA functional class II PAH included patients with PAH-CHD (n 5 32 of 185 individuals). At 6 months, PVR significantly decreased from baseline but 6MWT distance change did not reach statistical significance (mean treatment effect 19.1 m, P 5 .076).…”