Scimitar syndrome

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[edit] Discussion

  • AKA venolobar syndrome
  • AKA hypogenetic lung syndrome
  • Rare congenital cardiovascular anomaly involving the right lung.

Consists of:

  • Ipsilateral anomalous pulmonary drainage of part or all of the right lung into the inferior vena cava
  • Hypoplasia of the right lung
  • Dextrorotation of the heart
  • Hypoplasia or other malformation of the right pulmonary artery
  • Anomalous systemic arterial supply to the lower lobe of the right lung from the subdiaphragmatic aorta or its main branches
  • Connection to the bronchial tree distinguishes this entity from pulmonary sequestration

  • Clinical significance and prognosis of scimitar sydrome depend to a large extent on the amount of the resulting left-right shunt; at least 40% of patients are asymptomatic.
  • Clinical symptoms usually manifest in the 2nd to 3rd decade of life, with fatigue and dyspnea with exertion being most common and recurrent pulmonary infection less common.

[edit] Imaging Findings

[edit] Images

Patient #1

[edit] See Also

[edit] External Links

Goldminer: Scimitar syndrome

[edit] References

Teresa Berrocal, Carmen Madrid, Susana Novo, Julia Gutiérrez, Antonia Arjonilla, and Nieves Gómez-León. Congenital Anomalies of the Tracheobronchial Tree, Lung, and Mediastinum: Embryology, Radiology, and Pathology. RadioGraphics 2004 24: 17e.