European Journal of Pediatrics

, Volume 167, Issue 2, pp 155–160 | Cite as

Scimitar syndrome: incidence, treatment, and prognosis

  • Ching-Chia Wang
  • En-Ting Wu
  • Shyh-Jye Chen
  • Frank Lu
  • Shu-Chien Huang
  • Jou-Kou Wang
  • Chung-I Chang
  • Mei-Hwan Wu
Original Paper


This study is based on a database of 16 years; we sought to define the incidence and outcome of scimitar syndrome. Of 8,771 patients, 5 (0.057%) with scimitar syndrome were identified and constituted the study population. Follow-up ranged from 1 to 16 years (median: 10 years). Diagnosis was assured by computed tomography in four patients and by cardiac catheterization in one. Two patients presented with respiratory distress soon after birth and required early pneumonectomy in one case and coil embolization of the abnormal feeding arteries to the right lower lung followed by surgical rerouting of the abnormal pulmonary vein and repair of the atrial septal defect in the other case. The former was supported by ventilator therapy for 3 years after pneumonectomy, but was finally weaned from the ventilator. Among the other three, two had repeated pneumonia that resolved after rerouting of the abnormal right pulmonary vein and cardiac repair. The asymptomatic child did not receive any intervention. In spite of the abnormal orientation of the airways, none of the four patients with detailed computed tomography imaging showed any significant compression of the airways. All five patients were doing well as of the last follow-up. In conclusion, scimitar syndrome is a very rare disease in this Asian country and the varied symptoms, such as tachypnea and repeated infection, could be improved after interventions.


Scimitar syndrome Congenital heart disease Airway anomaly Pulmonary veins 


  1. 1.
    Brown JW, Ruzmetov M, Minnich DJ, Vijay P, Edwards CA, Uhlig PN, Fiore AC, Turrentine MW (2003) Surgical management of scimitar syndrome: an alternative approach. J Thorac Cardiovasc Surg 125:238–245PubMedCrossRefGoogle Scholar
  2. 2.
    Canter CE, Martin TC, Spray TL, Weldon CS, Strauss AW (1986) Scimitar syndrome in childhood. Am J Cardiol 58:652–654PubMedCrossRefGoogle Scholar
  3. 3.
    Chen CA, Chiu SN, Wu ET, Lin MT, Wang JK, Chang CI, Chiu IS, Wu MH (2006) Surgical outcome of aortopulmonary window repair in early infancy. J Formos Med Assoc 105:813–820PubMedCrossRefGoogle Scholar
  4. 4.
    Dupuis C, Charaf LA, Breviere GM, Abou P (1993) “Infantile” form of the scimitar syndrome with pulmonary hypertension. Am J Cardiol 71:1326–1330PubMedCrossRefGoogle Scholar
  5. 5.
    Dupuis C, Charaf LA, Breviere GM, Abou P, Remy-Jardin M, Helmius G (1992) The “adult” form of the scimitar syndrome. Am J Cardiol 70:502–507PubMedCrossRefGoogle Scholar
  6. 6.
    Dupuis C, Remy J, Remy-Jardin M, Coulomb M, Breviere GM, Ben Laden S (1994) The “horseshoe” lung: six new cases. Pediatr Pulmonol 17:124–130PubMedCrossRefGoogle Scholar
  7. 7.
    Farnsworth AE, Ankeney JL (1974) The spectrum of the scimitar syndrome. J Thorac Cardiovasc Surg 68:37–42PubMedGoogle Scholar
  8. 8.
    Frank JL, Poole CA, Rosas G (1986) Horseshoe lung: clinical, pathologic, and radiologic features and a new plain film finding. AJR Am J Roentgenol 146:217–226PubMedGoogle Scholar
  9. 9.
    Freedom RM, Burrows PE, Moes CA (1986) “Horseshoe” lung: report of five new cases. AJR Am J Roentgenol 146:211–215PubMedGoogle Scholar
  10. 10.
    Gao YA, Burrows PE, Benson LN, Rabinovitch M, Freedom RM (1993) Scimitar syndrome in infancy. J Am Coll Cardiol 22:873–882PubMedCrossRefGoogle Scholar
  11. 11.
    Gudjonsson U, Brown JW (2006) Scimitar syndrome. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 56–62Google Scholar
  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.
    Huddleston CB, Exil V, Canter CE, Mendeloff EN (1999) Scimitar syndrome presenting in infancy. Ann Thorac Surg 67:154–159PubMedCrossRefGoogle Scholar
  14. 14.
    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 96:1313–1316PubMedCrossRefGoogle Scholar
  15. 15.
    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
  16. 16.
    Kuiper-Oosterwal CH, Moulaert A (1973) The scimitar syndrome in infancy and childhood. Eur J Cardiol 1:55–61PubMedGoogle Scholar
  17. 17.
    Levine MM, Nudel DB, Gootman N, Wolpowitz A, Wisoff BG (1982) Pulmonary sequestration causing congestive heart failure in infancy: a report of two cases and review of the literature. Ann Thorac Surg 34:581–585PubMedCrossRefGoogle Scholar
  18. 18.
    Mulligan ME (1999) History of scimitar syndrome. Radiology 210:288–290PubMedGoogle Scholar
  19. 19.
    Najm HK, Williams WG, Coles JG, Rebeyka IM, Freedom RM (1996) Scimitar syndrome: twenty years’ experience and results of repair. J Thorac Cardiovasc Surg 112:1161–1168PubMedCrossRefGoogle Scholar
  20. 20.
    Ruggieri M, Abbate M, Parano E, Distefano A, Guarnera S, Pavone L (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 116:170–175PubMedCrossRefGoogle Scholar
  21. 21.
    Sener RN, Tugran C, Savas R, Alper H (1993) CT findings in scimitar syndrome. AJR Am J Roentgenol 160:1361PubMedGoogle Scholar
  22. 22.
    Sun LC, Wang JK, Lin MT, Wu ET, Lu FL, Lue HC, Chang CI, Chen YS, Chiu IS, Wu MH (2005) Persistent truncus arteriosus: twenty years experience in a tertiary care center in Taiwan. Acta Paediatr Taiwan 46:6–10PubMedGoogle Scholar
  23. 23.
    Thibault C, Perrault LP, Delisle G, Cartier PC, Cloutier A, Houde C, Deslauriers J (1995) Lobectomy in the treatment of the scimitar syndrome. Ann Thorac Surg 59:220–221PubMedCrossRefGoogle Scholar
  24. 24.
    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–e541PubMedCrossRefGoogle Scholar

Copyright information

© Springer-Verlag 2007

Authors and Affiliations

  • Ching-Chia Wang
    • 1
  • En-Ting Wu
    • 1
  • Shyh-Jye Chen
    • 2
  • Frank Lu
    • 1
  • Shu-Chien Huang
    • 3
  • Jou-Kou Wang
    • 1
  • Chung-I Chang
    • 3
  • Mei-Hwan Wu
    • 1
  1. 1.Department of PediatricsNational Taiwan University HospitalTaipeiTaiwan
  2. 2.Department of RadiologyNational Taiwan University HospitalTaipeiTaiwan
  3. 3.Department of TraumatologyNational Taiwan University HospitalTaipeiTaiwan

Personalised recommendations