Efficacy of Bosentan Treatment in Children with Eisenmenger Syndrome and Primary Pulmonary Hypertension

  • Dr Metin Sungur, Ondokuz Mayis University, Faculty of Medicine, Pediatric Cardiology Department, Turkey
  • Dr Emine Azak, Ondokuz Mayis University, Faculty of Medicine, Pediatric Cardiology Department, Turkey
  • Dr Pelin Ayyildiz, Ondokuz Mayis University, Faculty of Medicine, Pediatric Cardiology Department, Turkey
  • Prof Nazlihan Gunal, Turkey
  • Prof Kemal Baysal, Ondokuz Mayis University, Faculty of Medicine, Pediatric Cardiology Department, Turkey

    • Pulmonary arterial hypertension (PAH) in the pediatric population include mainly familial PAH and secondary to congenital heart disease.
    Objective: To describe an early experience of treating 11 children with bosentan, known to be safe and effective in adults with PAH.
    Design: In this study on 11 children treated with bosentan, three had idiopathic pulmonary arterial hypertension (IPAH) with a mean age of 8.6 years, (3-12 years), and eight had PH associated with congenital heart disease. Their mean age was 11.5 years (2.5-19 years).
    Patients: Eleven patients were in WHO class III and IV, and all had shown recent deterioration. In IPAH, the mean pulmonary vascular resistance (PVR) was 23.6 units.m2 (16-33). In Eisenmenger Syndrome patients, the mean PVR was 17.8 units.m2 (8-24). Mean pulmonary arterial pressures measured as 58 mmHg (45-68) in IPAH group, and as 73.5 mmHg (50-105) in Eisenmenger Syndrome group. Mean pulmonary arterial pressure was 69 mmHg (45-105). All patients had a positive response to vasodilator testing.
    Interventions: All children were treated with Bosentan for a mean of 10 months (6-22 months)
    Results: The drug was well tolerated by all patients. One patient with IPAH died during follow-up. Functional class and the 6 minute walk test distance improved. Arterial oxygen saturation remained stable. The ECG and echocardiographic findings did not change significantly.
    Conclusion: Bosentan appears to be safe and well tolerated in children with Eisenmenger Syndrome and IPAH. Moreover, in agreement with the observed improvement in objective exercise capacity, symptomatic status improved during treatment.