| Sub-title | |
| Authors | V Grech R Xuereb A Manche K Schembri J V DeGiovanni |
| Abstract | Scimitar syndrome 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 down to the inferior vena cava (IVC). This descending vein is visible on CXR as a curvilinear density along the right heart border and resembles the curved Arabic sword that gives the condition its name. Scimitar syndrome 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 a young lady who presented incidentally, with a murmur, at 16 years of age. Full investigation including angiography showed a large atrial septal defect with right heart dilation and scimitar syndrome. She was operated locally with uneventful and complete correction by baffling of the scimitar vein from its entry into the inferior vena to the left atrium through the atrial septal defect. |
Published in: | |
| Journal | Malta Medical Journal |
| Volume | Volume 15 (suppl) |
| Pages | - |
| Date | |
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| Key words | scimitar syndrome, pulmonary venous drainage, congenital heart disease, paediatrics |