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Scimitar syndrome (congenital pulmonary venolobar syndrome)
  1. A Khan,
  2. N J Ring,
  3. P D Hughes
  1. Department of Respiratory and General Medicine, Derriford Hospital, Plymouth, UK
  1. Correspondence to:
 Dr A Khan
 Department of Respiratory and General Medicine, Derriford Hospital, Plymouth PL6 8DH, UK; Ayaz.khanphnt.swest.nhs.uk

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A 19 year old woman was referred for left sided chest pain. There were no other symptoms but a day earlier she had been lifting heavy boxes. Her examination was entirely normal except for left sided localised chest wall tenderness. A clinical diagnosis of musculoskeletal chest pain was made. Her ECG and the rest of the baseline laboratory tests were normal. Her chest radiograph (fig 1 (A)) shows a small right hemithorax and an anomalous pulmonary vein on the right side (arrows). The magnetic resonance imaging scan (fig 1 (B)) shows that the anomalous vein (arrows) is draining into the right atrium (RA). The hypoplastic right lung and anomalous pulmonary vein confirmed that she had congenital pulmonary venolobar syndrome also known as scimitar syndrome. The name scimitar comes from the anomalous pulmonary vein that courses along the right cardiac margin as a curvilinear shadow and is said to resemble a “scimitar,” or Turkish sword. The anomalous venous return is usually to the inferior vena cava but it can also be to the portal vein, a hepatic vein, or the right atrium. Presentation and clinical course depends on the size of the shunt and varies from heart failure in infancy to asymptomatic adulthood. About 25% of the patients have associated congenital heart disease, most commonly septal defects. Our patient did not have any such defect. She remained well and active and did not need any further intervention.

Figure 1

 (A) Chest radiograph. (B) Magnetic resonance imaging scan. RA, right atrium; LV, left ventricle; PA, pulmonary artery; IVC, inferior vena cava.

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