Uncommon presentation of adult-form scimitar syndrome associated with single left pulmonary vein in a pregnant woman

BMJ Case Rep. 2017 May 15:2017:bcr2017219952. doi: 10.1136/bcr-2017-219952.

Abstract

Scimitar syndrome is the constellation of malformations including an abnormal venous drainage of the right lung into the inferior vena cava, associated with the right lung and systemic supply to the right lung. The anomalous vein looks like the curved, Turkish sword (scimitar), hence the name.The adult form of scimitar syndrome is rare, and it is usually an incidental diagnosis based on the characteristic finding on radiological imaging since the patients are usually asymptomatic or with minimal symptoms.Our patient presented with a rare presentation of scimitar syndrome, which is tachyarrhythmia (sinus tachycardia, with episodes of supraventricular tachycardia). The diagnosis of scimitar syndrome was made based on the typical radiological finding of the anomalous venous drainage on CT angiography. Our patient does not have the full spectrum of the scimitar syndrome; therefore, she did not suffer from the usual complication (pulmonary hypertension). She was treated with ablation without surgical intervention.

Keywords: Arrhythmias; Radiology (diagnostics).

Publication types

  • Case Reports

MeSH terms

  • Ablation Techniques / methods
  • Adult
  • Computed Tomography Angiography / methods
  • Female
  • Humans
  • Incidental Findings
  • Lung / abnormalities
  • Lung / blood supply*
  • Lung / pathology
  • Pregnancy
  • Pulmonary Veins / abnormalities*
  • Pulmonary Veins / diagnostic imaging
  • Radiography, Thoracic / methods
  • Rare Diseases
  • Scimitar Syndrome / complications
  • Scimitar Syndrome / diagnostic imaging*
  • Tachycardia / diagnosis
  • Tachycardia / etiology
  • Tachycardia / surgery
  • Treatment Outcome
  • Vena Cava, Inferior / abnormalities*
  • Vena Cava, Inferior / diagnostic imaging