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J Thorac Cardiovasc Surg 2008;135:446-448
© 2008 The American Association for Thoracic Surgery

Brief Communication

Use of sildenafil and nitric oxide in the management of hypoxemia owing to pulmonary arteriovenous fistulas after total cavopulmonary connection

^a Kids Heart Research and Adolph Basser Cardiac Institute, The Children's Hospital at Westmead, Sydney, Australia
^b Helen MacMillan Paediatric Intensive Care, The Children's Hospital at Westmead, Sydney, Australia
^c Discipline of Paediatrics and Child Health, Faculty of Medicine, The University of Sydney, Sydney, Australia

Received for publication August 13, 2007; revisions received October 4, 2007; accepted for publication October 15, 2007.

^_* Address for reprints: Associate Professor David Winlaw, Paediatric Surgeon and Head, Kids Heart Research, The Children's Hospital at Westmead, Locked Bag 4001, Westmead NSW 2145, Australia. (Email: davidw@chw.edu.au).

The first 20% of the full text of this article appears below.

Hypoxemia after total cavopulmonary connection is a predictable outcome in children with pulmonary arteriovenous fistulas (PAVF). Fontan completion is often performed earlier than routine in these children with a view to recruiting hepatic venous blood, which contains hepatic factor. This has an antiangiogenic effect and is associated with regression of PAVF in the medium term. Before this occurs, hypoxemia may develop, and the purpose of this report is to highlight the time frame over which hypoxemia may develop and the utility of inhaled nitric oxide and orally administered pulmonary vasodilators. We present the case histories of 2 children with PAVF who underwent total cavopulmonary connection. Both patients had significant postoperative hypoxemia associated with the presence of PAVF. Pulmonary vasodilatation occurring as a result of anesthetic agents and vasodilating infusions in the immediate postoperative period may delay the onset of hypoxemia, which may respond dramatically to inhaled nitric oxide. Sildenafil was used in both patients with normalization of systemic oxygen saturations over the short term and therapy was later ceased. Although not proven by the cases presented, we suggest that the benefit derived from sildenafil relates to vasodilatation of resistance vessels within the pulmonary parenchyma, which diminishes flow through PAVF, increasing systemic oxygenation until regression of PAVF, induced by hepatic factor, can take place.

Clinical Summaries

Patient 1
This child was born at 33 weeks with mitral atresia, ventricular septal defect, hypoplastic left ventricle, and aortic coarctation. Her condition was stabilized on a prostaglandin infusion and initial palliation included pulmonary artery banding with coarctation repair through a left thoracotomy. Atrial septectomy was . . . [Full Text of this Article]