Introduction: Intravenous epoprostenol is frequently administered in adults and children for treatment of pulmonary hypertension. Although generally safe, pulmonary edema has been described in a few case reports of adult patients with pulmonary veno-occlusive disease.
Case report: We present an infant who had an operation for scimitar syndrome and abnormal drainage of the right pulmonary veins into the inferior vena cava who developed pulmonary edema while receiving a prostacyclin infusion. The typical partial anomalous pulmonary venous drainage was operatively corrected at 6 days of age, and an accompanying coarctation was resected. At 7 months of age, diagnostic cardiac catheterization was performed to evaluate suspected pulmonary hypertension. Pulmonary pressure was elevated to supra-systemic values, and obstructed venous drainage of the right hypoplastic lung was demonstrated. To decrease pulmonary hypertension during weaning and extubation, epoprostenol infusion was initiated. Sixty minutes after extubation, massive acute pulmonary edema lead to reintubation. Mean airway pressure of 16 mm Hg (21 mbar) with pure oxygen ventilation was initially required, with an oxygenation index of 14, a ventilation index of 36, and an alveolar-arterial oxygen tension difference of 541 mm Hg. After discontinuation of epoprostenol, weaning and extubation was successful.
Conclusion: Pulmonary edema caused by prostacyclin infusion in patients with impaired postcapillary pulmonary drainage may also be encountered in children and has to be anticipated.