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Scimitar syndrome in an adult: diagnosis and surgical treatment

^a Department of Thoracic and Cardiovascular Surgery, University of Essen, Hufelandstrasse 55, 45122 Essen, Germany
^b Department of Internal Medicine I and Cardiology, Alfried Krupp Hospital, Essen, Germany

^* Corresponding author. Tel.: +49-201-723-3402; fax: +49-201-723-5931
markus.kamler{at}uni-essen.de

A 51-year-old woman, presenting with increasing dyspnoea on exertion was admitted for surgery of a right sided partial anomalous pulmonary venous return. Contrast enhanced electron-beam tomography (EBT) presented a hypoplastic right lower lobe and confirmed the diagnosis of a so-called scimitar syndrome. Surgery consisted of creation of an atrial septal defect, transposition of the anomalous vein and reocclusion of the artificial septal defect with a Dacron patch. One year after surgery, the patient's physical strength increased to a normal level without dyspnoea, while three-dimensional imaging reconstructed from EBT demonstrated an intact operative situs.

Key Words: Scimitar syndrome; Three-dimensional computed tomography; Adult congenital surgery; Partial anomalous pulmonary venous return