Surgical Repair of Scimitar Syndrome

Scimitar syndrome (SS) is a form of partial anomalous pulmonary venous drainage that is dramatically visible on plain chest radiography (CXR). In these individuals, the entire venous drainage from the right lung enters a single anomalous large vein that descends to the inferior vena cava. This descending vein is visible on CXR as a curvilinear density along the right heart border and resembles the curved Turkish sword that gives the condition its name. SS forms part of the large spectrum of associated conditions known as venolobar syndrome. These include right lung hypoplasia or sequestered segments of right lung, congenital heart disease and various others. We report, along with our other five cases, a special case of a young woman who presented incidentally, with a murmur, at 16 years of age. Full investigation including angiography showed a large atrial septal defect (ASD) with right heart dilation and SS. She underwent surgical correction with uneventful and complete correction by baffling of the scimitar vein from its entry into the inferior vena to the left atrium through the enlarged ASD.