European Journal of Pediatrics

, Volume 169, Issue 10, pp 1171–1177 | Cite as


Scimitar syndrome
  • Levent MidyatEmail author
  • Esen Demir
  • Memnune Aşkın
  • Figen Gülen
  • Zülal Ülger
  • Remziye Tanaç
  • Selen Bayraktaroğlu


Scimitar syndrome is a rare congenital anomaly, characterized by partial or complete anomalous pulmonary venous drainage of the right or left lung into the inferior vena cava. The syndrome is commonly associated with hypoplasia of the right lung, pulmonary sequestration, persisting left superior vena cava, and dextroposition of the heart. The pathogenesis of the syndrome is unclear, but it seems to originate from a basic developmental disorder of the entire lung bud early in embryogenesis. Two main forms of scimitar syndrome have been described. Signs and symptoms can start during infancy (infantile form) or beyond (childhood/adult form). The infantile form generally presents within the first 2 months of life with tachypnea, recurrent pneumonia, failure to thrive, and signs of heart failure. The diagnosis of scimitar syndrome is usually made based on the characteristic chest X-ray films and can be confirmed by angiography; however, it is now done mostly by transthoracic or transesophageal echocardiography, noninvasive computed tomography, or magnetic resonance angiography. Fetal echocardiography using three-dimensional power Doppler imaging permits prenatal diagnosis. Most frequently, patients are asymptomatic in the absence of associated abnormalities and can be followed conservatively. For patients with congestive heart failure, repeated pneumonia, or pulmonary-to-systemic blood flow ratios greater than 1.5 and pulmonary hypertension, it is important to reroute the anomalous right pulmonary veins and repair the associated cardiac defects in order to avoid progression to right ventricular failure. The triad of respiratory distress, right lung hypoplasia, and dextroposition of the heart should alert the clinician to think of scimitar syndrome.


Scimitar syndrome Congenital abnormality Pulmonary Cardiovascular 


Conflict of interest

The authors received no extra funding for preparation of this manuscript and believe that there is no relationship that can lead to any conflict of interests relevant to the content of this article.


  1. 1.
    Baxter R, McFadden PM, Gradman M, Wright A (1990) Scimitar syndrome: cine magnetic resonance imaging demonstration of anomalous pulmonary venous drainage. Ann Thorac Surg 50:121–123CrossRefPubMedGoogle Scholar
  2. 2.
    Casha AR, Sulaiman M, Cale AJR (2003) Repair of adult scimitar syndrome with an intra-atrial conduit. Interactive Cardiovasc Thorac Surg 2:128–130CrossRefGoogle Scholar
  3. 3.
    Cirillo RL Jr (1998) The scimitar sign. Radiology 206(3):623–624PubMedGoogle Scholar
  4. 4.
    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
  5. 5.
    D’Cruz IA, Arcilla RA (1964) Anomalous venous drainage of the left lung into the inferior vena cava: a case report. Am Heart J 67:539–544CrossRefGoogle Scholar
  6. 6.
    Drake EH, Lynch JP (1950) Bronchiectasis associated with anomaly of the right pulmonary vein and right diaphragm; report of a case. J Thorac Surg 19(3):433–437PubMedGoogle Scholar
  7. 7.
    Dupuis C, Charaf LA, Breviere GM, Abou P (1993) “Infantile” form of the scimitar syndrome with pulmonary hypertension. Am J Cardiol 71:1326–1330CrossRefPubMedGoogle Scholar
  8. 8.
    Dupuis C, Charaf LA, Breviere GM et al (1992) The “adult” form of the scimitar syndrome. Am J Cardiol 70:502–507CrossRefPubMedGoogle Scholar
  9. 9.
    Felson B (1973) Chest roentgenology. Saunders, Philadelphia, pp 87–92Google Scholar
  10. 10.
    Gao YA, Burrows PE, Benson LN et al (1993) Scimitar syndrome in infancy. J Am Coll Cardiol 22:873–882CrossRefPubMedGoogle Scholar
  11. 11.
    Gupta M, Vaughan DJ, Zimmerman J, Grifka RG. Partial anomalous pulmonary venous connection.
  12. 12.
    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
  13. 13.
    Henk CB, Prokesch R, Grampp S et al (1997) Scimitar syndrome: MR assessment of hemodynamic significance. J Comput Assist Tomogr 21:628–630CrossRefPubMedGoogle Scholar
  14. 14.
    Holt PD, Berdon WE, Marans Z et al (2004) Scimitar vein draining to the left atrium and a historical review of the scimitar syndrome. Pediatr Radiol 34(5):409–413CrossRefPubMedGoogle Scholar
  15. 15.
    Honey M (1977) Anomalous pulmonary venous drainage of right lung to inferior vena cava (“scimitar syndrome”): clinical spectrum in older patients and role of surgery. Q J Med 46:463–483PubMedGoogle Scholar
  16. 16.
    Huddleston CB, Exil V, Canter CE, Mendeloff EN (1999) Scimitar syndrome presenting in infancy. Ann Thorac Surg 67:154–159CrossRefPubMedGoogle Scholar
  17. 17.
    Inoue T, Ichihara M, Uchida T et al (2002) Three dimensional computed tomography showing partial anomalous pulmonary venous connection complicated by the scimitar syndrome. Circulation 105:663CrossRefPubMedGoogle Scholar
  18. 18.
    Kabbani M, Haider N, Abu-Sulaiman R (2004) Bilateral scimitar syndrome. Cardiol Young 14:447–449CrossRefPubMedGoogle Scholar
  19. 19.
    Kamler M, Kerkhoff G, Budde T, Jakob H (2003) Scimitar syndrome in an adult: diagnosis and surgical treatment. Interact Cardiovasc Thorac Surg 2(3):350–351CrossRefPubMedGoogle Scholar
  20. 20.
    Khalilzadeh S, Hassanzad M, Khodayari AA (2009) Scimitar syndrome. Arch Iran Med 12(1):79–81PubMedGoogle Scholar
  21. 21.
    Khan MA, Torres AJ, Printz BF, Prakash A (2005) Usefulness of magnetic resonance angiography for diagnosis of scimitar syndrome in early infancy. Am J Cardiol 96:1313–1316CrossRefPubMedGoogle Scholar
  22. 22.
    Kiely BFJ, Stone S, Doyle EF (1967) Syndrome of anomalous 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 20:102–116CrossRefGoogle Scholar
  23. 23.
    Kirklin JW, Ellis FH Jr, Wood EH (1956) Treatment of anomalous pulmonary venous connections in association with interatrial communications. Surgery 39(3):389–398PubMedGoogle Scholar
  24. 24.
    Kramer U, Dörnberger V, Fenchel M et al (2003) Scimitar syndrome: morphological diagnosis and assessment of hemodynamic significance by magnetic resonance imaging. Eur Radiol 13(Suppl 4):L147–L150CrossRefPubMedGoogle Scholar
  25. 25.
    Mardini MK, Sakati NA, Lewall DB et al (1982) Scimitar syndrome. Clin Pediatr 21:350–354CrossRefGoogle Scholar
  26. 26.
    Mathey J, Galey JJ, Logeais Y et al (1968) Anomalous pulmonary venous return into inferior vena cava and associated bronchovascular anomalies (the scimitar syndrome): report of three cases and review of the literature. Thorax 23:398–407CrossRefPubMedGoogle Scholar
  27. 27.
    Michailidis GD, Simpson JM, Tulloh RM, Economides DL (2001) Retrospective prenatal diagnosis of scimitar syndrome aided by three-dimensional power Doppler imaging. Ultrasound Obstet Gynecol 17(5):449–452CrossRefPubMedGoogle Scholar
  28. 28.
    Misra M, Sadiq A, Rema Manohar KS, Neelakandhan KS (2005) Scimitar syndrome with anomalous connection of left superior pulmonary vein to left innominate vein. Interact Cardiovasc Thorac Surg 4(6):606–608CrossRefPubMedGoogle Scholar
  29. 29.
    Mordue BC (2003) A case series of five infants with scimitar syndrome. ADV Neonatal Care 3:121–132CrossRefPubMedGoogle Scholar
  30. 30.
    Najm HK, Williams WG, Coles JG et al (1996) Scimitar syndrome: twenty years’ experience and results of repair. J Thorac Cardiovasc Surg 112:1161–1168CrossRefPubMedGoogle Scholar
  31. 31.
    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 107:1–21PubMedGoogle Scholar
  32. 32.
    Oshima Y, Hashimoto I, Shimazu C, Ichida F (2003) Atypical infantile form of scimitar syndrome with bronchomalacia. Interact Cardiovasc Thorac Surg 2(3):298–300CrossRefPubMedGoogle Scholar
  33. 33.
    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
  34. 34.
    Puig-Massana M, Revuelta JM (1972) A new technique in the correction of partial anomalous pulmonary venous drainage. J Thoracic Surg 64:108–113Google Scholar
  35. 35.
    Rajaii-Khorasani A, Kahrom M, Mottaghi H, Kahrom H (2009) Scimitar syndrome: report of a case and its surgical management. Ann Saudi Med 29(1):50–52CrossRefPubMedGoogle Scholar
  36. 36.
    Ruggieri M, Abbate M, Parano E et al (2003) Scimitar vein anomaly with multiple cardiac malformations, craniofacial, and central nervous system abnormalities in a brother and sister: familial scimitar anomaly or new syndrome? Am J Med Genet A 116:170–175CrossRefGoogle Scholar
  37. 37.
    Rutledge JM, Hiatt PW, Vick GW III, Grifka RG (2001) A sword for the left hand: an unusual case of left-sided scimitar syndrome. Pediatr Cardiol 22:350–352PubMedGoogle Scholar
  38. 38.
    Schramel FMNH, Westermann CJJ, Kanaepen PJ, Van den Bosch JMM (1995) The scimitar syndrome: clinical spectrum and surgical treatment. Eur Respir J 8:196–201CrossRefPubMedGoogle Scholar
  39. 39.
    Sehgal A, Loughran-Fowlds A (2005) Scimitar syndrome. Indian J Pediatr 72(3):249–251CrossRefPubMedGoogle Scholar
  40. 40.
    Shumacker HB, Judd D (1964) Partial anomalous pulmonary venous return with reference to drainage into the inferior vena cava and to an intact atrial septum. J Cardiovasc Surg (Torino) 5:271–278Google Scholar
  41. 41.
    Sridhar AV, Tofeig M (2003) A 4 yr-old presenting with chronic cough and asymmetrical chest. Eur Respir J 22:862–864CrossRefPubMedGoogle Scholar
  42. 42.
    Suri D, Sodhi KS, Muralidharan J et al (2008) Scimitar syndrome: an uncommon cause of wheezing. Pediatr Emerg Care 24(3):164–166CrossRefPubMedGoogle Scholar
  43. 43.
    Thibault C, Perrault LP, Delisle G et al (1995) Lobectomy in the treatment of the scimitar syndrome. Ann Thorac Surg 59:220–221CrossRefPubMedGoogle Scholar
  44. 44.
    Tsuchida K, Anzai N, Hashimoto A et al (1987) Intraatrial conduit repair in scimitar syndrome. Chest 92:1120–1122CrossRefPubMedGoogle Scholar
  45. 45.
    Wang CC, Wu ET, Lu F et al (2008) Scimitar syndrome: incidence, treatment and prognosis. Eur J Pediatr 167:155–160CrossRefPubMedGoogle Scholar
  46. 46.
    Wu MT, Lai RS, Huang YL, Hsiao SH (2004) Images in cardiovascular medicine. Scimitar syndrome with esophageal varices: magnetic resonance angiography detects anomalous pulmonary venous return. Circulation 110:e540–e541CrossRefPubMedGoogle Scholar
  47. 47.
    Zubiate P, Kay JH (1962) Surgical correction of anomalous pulmonary venous connection. Ann Surg 156:234–250CrossRefPubMedGoogle Scholar

Copyright information

© Springer-Verlag 2010

Authors and Affiliations

  • Levent Midyat
    • 1
    • 4
    Email author
  • Esen Demir
    • 1
  • Memnune Aşkın
    • 1
  • Figen Gülen
    • 1
  • Zülal Ülger
    • 2
  • Remziye Tanaç
    • 1
  • Selen Bayraktaroğlu
    • 3
  1. 1.Division of Pulmonology–Allergy, Department of Pediatrics, Faculty of MedicineEge UniversityIzmirTurkey
  2. 2.Division of Cardiology, Department of Pediatrics, Faculty of MedicineEge UniversityIzmirTurkey
  3. 3.Department of Radiology, Faculty of MedicineEge UniversityIzmirTurkey
  4. 4.Cocuk Sagligi ve Hastaliklari ADEge UniversitesiIzmirTurkey

Personalised recommendations