Reverse differential cyanosis (upper extremity oxygen saturation less than lower extremity oxygen saturation) is a rare, formerly fatal condition. Usually seen with dextrotransposition of the great arteries (with persistent pulmonary hypertension or aortic interruption/coarctation), it can be treated successfully with oxygen, prostaglandin E1, atrial septotomy, nitric oxide, bosentan, or extracorporeal membrane oxygenation, followed by the arterial switch operation.

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