Epoprostenol and home mechanical ventilation for pulmonary hypertension associated with chronic lung disease

Pulmonary hypertension (PH) can be associated with bronchopulmonary dysplasia (BPD) of infancy and mortality in these pediatric patients is high without aggressive medical treatment. Continuous intravenous epoprostenol (prostacyclin) was shown to lower pulmonary artery pressures (PAP) in children with idiopathic pulmonary arterial hypertension (PAH), formerly referred to as primary pulmonary hypertension. We report on the first case of long-term home ventilation in combination with chronic intravenous epoprostenol in a child with severe pulmonary hypertension associated with chronic lung disease. This aggressive combination resulted in significant improvement in pulmonary artery pressures, substantial improvement in quality of life, and eventual discontinuation of home ventilation.