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Pulmonary arteriovenous malformations may result in intrapulmonary shunting and hypoxemia, and often are treated by embolization or surgical resection. Previous reports have demonstrated effective treatment of hypoxia with inhaled nitric oxide in the acute setting. In this report, we describe a child with severe hypoxemia secondary to an inoperable pulmonary arteriovenous malformation that was initially managed with the use of nitric oxide followed by long term management with the phosphodiesterase-5 inhibitor, sildenafil.

Inhaled nitric oxide and sildenafil therapy for a pediatric patient with multiple pulmonary arteriovenous malformations