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

Julie Pantaleoni1, Kourosh Parsapour2, Alexander A Kon3

  • 1Department of Pediatrics, School of Medicine, Stanford University, Stanford, CA, USA.

Journal of Pediatric Intensive Care
|June 20, 2019
PubMed

Insights

Pulmonary arteriovenous malformations can cause hypoxemia. Sildenafil provided long-term management for a child with severe hypoxemia from an inoperable malformation.

Area of Science:

  • Cardiology
  • Pulmonology
  • Pediatric Medicine

Background:

  • Pulmonary arteriovenous malformations (PAVMs) can lead to intrapulmonary shunting and hypoxemia.
  • Standard treatments include embolization or surgical resection, which may not be suitable for all patients.

Observation:

  • A pediatric patient presented with severe hypoxemia due to an inoperable PAVM.
  • Initial management involved inhaled nitric oxide for acute symptom control.

Findings:

  • The patient was transitioned to long-term management with sildenafil, a phosphodiesterase-5 inhibitor.
  • Sildenafil effectively managed the severe hypoxemia associated with the inoperable PAVM.

Implications:

  • Sildenafil represents a potential long-term therapeutic option for managing hypoxemia in patients with inoperable PAVMs.
  • This case highlights the utility of phosphodiesterase-5 inhibitors beyond acute settings for complex pulmonary vascular conditions.

Keywords:
Arteriovenous malformationsnitric oxidephosphodiesterase-5 inhibitors

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