Bosentan therapy for pulmonary arterial hypertension: a case report of a 16-month-old boy

ABSTRACT

The case of a 16-month-old child presenting with acute respiratory distress who was subsequently diagnosed as having idiopathic pulmonary arterial hypertension is reported. In addition to respiratory problems, the child had a history of developmental delay of postural acquisition and ponderal stagnation. Cardiac echography showed right ventricular dilatation with paradoxical bulging of the septum into the left ventricle and compression. Significant clinical and echocardiographic improvements following oxygen therapy and the use of furosemide allowed cardiac catheterisation under general anaesthesia which revealed an infrasystemic pulmonary hypertension with no vasoreactivity to oxygen or nitric oxide. The parents refused any aggressive treatment such as continuous infusion of epoprostenol, atrioseptostomy or pulmonary transplantation. Treatment with bosentan resulted in marked functional improvement and echographic stabilisation at 6 months that was maintained after 36 months of therapy.

Key words: :

• Bosentan
• Children
• Long-term therapy
• Paediatric PAH
• Pulmonaryarterial hypertension