Advertisement

Pediatric Radiology

, Volume 34, Issue 5, pp 409–413 | Cite as

Scimitar vein draining to the left atrium and a historical review of the scimitar syndrome

  • Peter D. Holt
  • Walter E. Berdon
  • Zvi Marans
  • Sylvia Griffiths
  • Daphne Hsu
Case Report

Abstract

Scimitar vein draining to the left atrium is the rarest of the pulmonary venolobar anomalies with less than a dozen reported cases. A case is reported of a patient whose plain film radiographic findings showed a typical scimitar vein but whose echocardiography showed normal pulmonary venous drainage. Cardiac catheterization confirmed drainage of the scimitar vein to the left atrium and systemic arterial supply which was embolized. The case described is used to review the history of “scimitar syndrome” with reflections on the significant contributions of Halasz and colleagues, who in 1956 helped define the anatomy, and Neill and colleagues, who in 1960 described the familial occurrence and clinical spectrum of the condition.

Keywords

Scimitar syndrome Left atrium Historical review 

References

  1. 1.
    Woodring JH, Howard TA, Kanga JF (1994) Congenital pulmonary venolobar syndrome revisited. Radiographics 14:349–369PubMedGoogle Scholar
  2. 2.
    Cooper G (1836) Case of malformation of the thoracic viscera consisting of imperfect development of the right lung and transposition of the heart. London Med Gazette 18:600–601Google Scholar
  3. 3.
    Park EA (1912) Defective development of the right lung due to anomalous development of the right pulmonary artery and vein, accompanied by dislocation of the heart simulating dextrocardia. Proc NY Pathol Soc 12:88–93Google Scholar
  4. 4.
    Halasz NA, Halloran KH, Liebow AA (1956) Bronchial and arterial anomalies with drainage of the right lung into the inferior vena cava. Circulation 14:826–846PubMedGoogle Scholar
  5. 5.
    Neill CA, Ferencz C, Sabiston DC, et al (1960) the familial occurrence of hypoplastic right lung with systemic arterial supply and venous drainage “scimitar syndrome.” Bull Johns Hopkins Hosp 107:1–21Google Scholar
  6. 6.
    Charaf L, Abon P, Lundell B (1992) [The man behind the syndrome. Nicholas A. Halasz—the man behind a rare abnormality. The scimitar syndrome—a condition with the scimitar formed shadow] (in Swedish). Lakartidningen 89:565–566PubMedGoogle Scholar
  7. 7.
    Dotter CT, Hardisty NM, Steinberg I (1949) Anomalous right pulmonary vein entering the inferior vena cava: two cases diagnosed during life by angiography and cardiac catheterization. Am J Med Sci 218:31–36Google Scholar
  8. 8.
    Roehm JOF, Jue KL, Amplatz K (1966) Radiographic features of the scimitar syndrome. Radiology 86:856–859PubMedGoogle Scholar
  9. 9.
    Kiely B, Filler J, Stone S, et al (1967) Syndrome of anomalous venous drainage of the right lung to the inferior vena cava: a review of 67 reported cases and three new cases in children. Am J Cardiol 20:102–116Google Scholar
  10. 10.
    Morgan JR, Forker AD (1971) Syndrome of hypoplasia of the right lung and dextroposition of the heart: “scimitar sign” with normal pulmonary venous drainage. Circulation 43:27–30PubMedGoogle Scholar
  11. 11.
    Goodman LR, Jamshidi A, Hipona FA (1972) Meandering right pulmonary vein simulating the scimitar syndrome. Chest 62:510–512PubMedGoogle Scholar
  12. 12.
    Felson B (1973) Chest roentgenology. Saunders, PhiladelphiaGoogle Scholar
  13. 13.
    Dupuis C, Charaf LAC, Breviere GM, et al (1992) The “adult” form of the scimitar syndrome. Am J Cardiol 70:502–507PubMedGoogle Scholar
  14. 14.
    Dupuis C, Charaf LAC, Breviere GM, et al (1993) “Infantile” form of the scimitar syndrome with pulmonary hypertension. Am J Cardiol 71:1326–1330PubMedGoogle Scholar
  15. 15.
    Yong-An G, Burrows PE, Benson LN, et al (1993) Scimitar syndrome in Infancy. J Am Coll Cardiol 22:873–882PubMedGoogle Scholar
  16. 16.
    Marco de Lucas E, Canga A, Sadaba P, et al (2003) Scimitar syndrome: complete anatomical and functional diagnosis with gadolinium-enhanced and velocity-encoded cine MRI. Pediatr Radiol 33:716–718CrossRefPubMedGoogle Scholar

Copyright information

© Springer-Verlag 2004

Authors and Affiliations

  • Peter D. Holt
    • 1
  • Walter E. Berdon
    • 1
  • Zvi Marans
    • 2
  • Sylvia Griffiths
    • 2
  • Daphne Hsu
    • 2
  1. 1.Department of Radiology, Columbia University, College of Physicians and Surgeons, Children’s Hospital of New York-Presbyterian Babies Hospital 3NNew YorkUSA
  2. 2.Department of Cardiology, Columbia University, College of Physicians and Surgeons, Children’s Hospital of New York-PresbyterianBabies Hospital 3NNew YorkUSA

Personalised recommendations