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Scimitar syndrome
  1. G P Georghiou,
  2. B A Vidne,
  3. R Hirsch

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Scimitar syndrome is a rare condition characterised by partial or complete anomalous pulmonary venous drainage to the inferior vena cava. The term derives from the curvilinear shadow created by the anomalous pulmonary vein on the chest radiograph. The shadow extends medially from the lateral superior position of the right lung and increases in calibre as it descends toward the cardiophrenic angle.

An 18 year old man was referred for evaluation of recurrent pleuritis. The chest radiograph showed prominent central pulmonary arteries with normal tapering into the periphery and a typical scimitar shaped shadow in the right lung (panel A, arrows). Magnetic resonance imaging in the transverse plane showed the scimitar vein at the level of its connection with the inferior vena cava, just below the level of the right atrium (panel B: IVC, inferior vena cava; SV, scimitar vein). The coronal plane revealed the entire length of the scimitar vein (panel C: AO, ascending aorta; LV, left ventricle; MPA, main pulmonary artery; RA, right atrium; RPA, right pulmonary artery; RV, right ventricle). The right lung and airways were of normal size. On both echocardiography and first pass nuclear scan, the presence of a large left to right shunt (Qp/Qs  =  2) was confirmed, caused by the anomalous pulmonary venous drainage through the scimitar vein.

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At surgery, a pericardial baffle was created to redirect the blood from the anomalous vein through a surgically created atrial septal defect into the left atrium. Surgically treated scimitar syndrome has an excellent long term prognosis and most patients are asymptomatic.

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