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

Abstract

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.

Juvenile polyposis syndrome and hereditary haemorrhagic telangiectasia caused by the SMAD4 gene mutation in a paediatric patient – a case report and a review of the literature

“…The only study that reports multiple arteriovenous malformations involving the liver was that of Chung et al [12]. We found only one paper by Pantaleoni et al describing a patient with severe hypoxaemia secondary to an inoperable pulmonary arteriovenous malformations treated by long-term management with sildenafil [13].…”

Successful use of calcium channel blocker for management of arterio-venous malformations following Fontan procedure

“…Its use has been well described in the literature as a complementary and alternative therapy to inhaled nitric oxide for the treatment of hypoxaemia resulting from arteriovenous malformations following Fontan procedure. 5,6 In our patient, serial trials of nitric oxide cessation were attempted after initiation of high-dose sildenafil failed and were associated with severe hypoxaemia and acidosis. As the patient responded to nitric oxide, calcium channel blockers were introduced with the hypothesis that it would dilate the pulmonary arterioles and divert blood flow away from the arteriovenous malformations, into the normal capillaries.…”

“…1 In presence of severe arteriovenous malformations, which cause significant arterial desaturation following a Fontan procedure, several therapeutic options have been reported. [4][5][6] Percutaneous or surgical interruption of individual arteriovenous malformations has been described when the vessels were large and accessible, and lobectomy has been reported for localised arteriovenous malformations. 7 However, when arteriovenous malformations are small and diffuse, only medical treatment is available.…”

“…Phosphodiesterase-5 inhibitor, sildenafil, is commonly used to wean inhaled nitric oxide and is continued until resolution of the arteriovenous malformations following Fontan procedure and restoration of the hepatic blood flow into the pulmonary circulation. Its use has been well described in the literature as a complementary and alternative therapy to inhaled nitric oxide for the treatment of hypoxaemia resulting from arteriovenous malformations following Fontan procedure 5 6 …”