Why the study?
Does long-term home ventilation in combination with chronic intravenous epoprostenol improve pulmonary artery pressures and quality of life in a child with severe pulmonary hypertension associated with chronic lung disease?
Does long-term home ventilation in combination with chronic intravenous epoprostenol improve pulmonary artery pressures and quality of life in a child with severe pulmonary hypertension associated with chronic lung disease?
The combination of chronic intravenous epoprostenol and home mechanical ventilation may be an effective aggressive treatment strategy for severe pulmonary hypertension associated with chronic lung disease in children.
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.
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Zaidi et al. (2005) studied this question.
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