Abstract
Management of desaturation following bidirectional superior cavopulmonary anastomosis can be complex. Causes of desaturation following bidirectional cavopulmonary anastomosis include anastomotic obstruction, presence of a decompressing vein from the cavopulmonary circuit to the inferior vena cava territory or to the atrium, high pulmonary vascular resistance, ventricular dysfunction, and, in rare cases, acute pulmonary arteriovenous malformations. While the cause of desaturation may be obvious in most patients, it may not be conclusive in some. If data suggests acute pulmonary arteriovenous malformations as the cause of desaturation in a 3-month-old baby, it becomes a management challenge because the accepted strategy of performing a completion Fontan operation is very high risk and inadvisable at this age. We present the case of a 3-month-old baby who had severe desaturation following bidirectional superior cavopulmonary anastomosis. Clinical data and angiography were suggestive of pulmonary arteriovenous malformations. We managed the patient successfully with a Blalock-Taussig shunt and he has since had a completion Fontan.
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