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Tex Heart Inst J. 2012;39(4):571-4.

Aberrant right subclavian artery-esophageal fistula and severe gastrointestinal bleeding after surgical correction of scimitar syndrome.

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Department of Internal Medicine, Midwestern University, Downers Grove, Illinois 60515, USA.


Scimitar syndrome and gastrointestinal bleeding from an aberrant right subclavian artery-esophageal fistula are each extremely rare. Although scimitar syndrome and aberrant right subclavian artery are typically asymptomatic in adults, fistulous connection between the aberrant artery and the esophagus is associated with a poor prognosis. Outcomes are contingent upon timely diagnosis and prompt surgical repair. Prolonged nasogastric and endotracheal intubation can lead to gastrointestinal bleeding in patients who have an aberrant right subclavian artery or other vascular ring. We recommend neither embolization nor the use of endovascular stents as anything other than a temporizing measure in the management of aberrant right subclavian artery injury. These methods can stop acute hemorrhage; however, sentinel bleeding will eventually occur and require definitive ligation. We report the case of a 57-year-old woman in whom an aberrant right subclavian artery-esophageal fistula developed after surgical correction of symptomatic scimitar syndrome. Massive gastrointestinal bleeding resulted from prolonged nasogastric and endotracheal intubation. To our knowledge, this is the first report of aberrant right subclavian artery and scimitar syndrome in the same patient, and the 4th report of a patient's surviving a fistula between the aberrant artery and the esophagus.


Esophageal fistula/complications/diagnosis/etiology/surgery; gastrointestinal hemorrhage/diagnosis/etiology; heart defects, congenital/pathology; hematemesis/etiology; intubation/adverse effects; scimitar syndrome/complications/diagnosis; subclavian artery/abnormalities/surgery; treatment outcome

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