Response to bosentan in children with pulmonary hypertension

Abstract

Objective: To describe an early experience of treating 40 children with the dual endothelin receptor antagonist bosentan, which is known to be safe and effective in adults with pulmonary hypertension (PH). Design: In this retrospective, observational study the UK Service for Pulmonary Hypertension for children treated 40 children with bosentan, 20 with idiopathic pulmonary arterial hypertension (IPAH) (mean age 8.03 years, range 1.2-17) and 20 with PH associated with other conditions (congenital heart disease, parenchymal lung or connective tissue disease, or HIV). Their mean age was 8.3 years (range 0.6-16 years). Patients: 39 patients were in World Health Organization (WHO) class III and IV, and all had shown recent deterioration. In IPAH the mean pulmonary vascular resistance (PVR) was 21.7 units?m 2 (range 5.6-42.8). In secondary PH the mean PVR was 18 units?m 2 (range 4.9-49). No child had a positive response to vasodilator testing with nitric oxide. Interventions: Bosentan was given as first line treatment to 25. Nine were given intravenous epoprostenol. Children were treated for a mean of 12.7 months (range 2-24 months). Main outcome measures: Response to treatment was judged by WHO functional class, six minute walk test, weight, ECG and echocardiographic findings, and need to add additional treatment. Results: Bosentan was well tolerated. In the IPAH group 19 (95%) stabilised with bosentan treatment but 12 (60%) patients needed combined treatment with epoprostenol. In secondary PH, WHO class, six minute walk test, and weight gain improved significantly. Conclusion: Bosentan helped stabilise children with IPAH but intravenous epoprostenol was also needed by 60%. Children with secondary PH improved.

Paediatric pulmonary hypertension: monitoring progress and identifying unmet needs

“…Other less well-powered, open-label studies in smaller numbers of patients, often cases series or retrospective studies, have reported on the use of bosentan in paediatric patients with PAH [20][21][22][23][24]. In a study specifically carried out in PAH patients with systemic-to-pulmonary shunt, bosentan therapy produced short-term improvement (4 months) with respect to WHO functional class and 6-min walking distance both in 10 children and 25 adults [25].…”

Anesthetic Management of Pulmonary Hypertension Secondary to Pediatric Congenital Cardiac Disease

“…Nonselektif ETA/ETB antagonisti olarak kullanılan bosentan kronik PHT tedavisinde tercih edilmektedir. OPAB ve PDD'ni azalttığı ve idiopatik PHT hastalarında yaşam kalitesini arttırdığı gösterilmiştir (17,18) . Eisenmenger sendromunda oksijen satürasyonunu azaltmadan egzersiz kapasitesini düzelttiği gösterilmiştir (19) .…”

Successful Treatment with Bosentan for Pulmonary Hypertension and Reduced Peripheral Circulation in Juvenile Systemic Sclerosis

“…Furthermore, it has been shown that bosentan ameliorates decreased skin perfusion and digital ulceration secondary to SSc [5,6]. A previous report showed that bosentan was effective for treatment of children with primary and secondary PAH [3]. However, the effectiveness and safety of bosentan for treatment of juvenile SSc still remains unclear.…”