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BMJ Case Reports 2017; doi:10.1136/bcr-2017-219952
  • Rare disease
  • CASE REPORT

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

  1. Mohammad Salih Almalki
  1. Internal Medicine Department, King Abdulaziz Specialist Hospital, Taif City, Saudi Arabia
  1. Correspondence to Dr Sarah Ali Althomali, sara_ali8089{at}yahoo.com
  • Accepted 28 April 2017
  • Published 15 May 2017

Summary

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.

Footnotes

  • Contributors All persons who meet authorship criteria are listed as authors, and all authors certify that they have participated sufficiently in the work to take public responsibility for the content. Each author certifies that this material has not been and will not be submitted to or published in any other publication before its appearance in the BMJ Case Reports. Detailed Contributorship : 1. Conception of study: SAA, AAA. 2. Acquisition of data: SAA, AAA. 3. Interpretation of data: SAA, MSA. 4. Drafting the manuscript: SAA, MSA. 5. Revising the manuscript: MSA.

  • Competing interests None declared.

  • Patient consent Obtained.

  • Provenance and peer review Not commissioned; externally peer reviewed.

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