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Scimitar Syndrome
Scimitar Syndrome
Randy R. Richardson, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Clinical Issues

        TERMINOLOGY

        • Synonyms

          • Hypogenetic lung syndrome, congenital pulmonary venolobar syndrome

        • Definitions

          • Scimitar syndrome triad: Right lung hypoplasia, anomalous right pulmonary venous connection to inferior vena cava (IVC), & anomalous systemic arterial supply to right lower lobe
            • Scimitar vein represents form of partial anomalous pulmonary venous return (PAPVR)
          • Category: Acyanotic, right-sided cardiac chamber enlargement, ↑ pulmonary vascularity
          • Hemodynamics: Venous flow from right lung returns to right atrium → volume overload of right heart [atrial septal defect (ASD)-type physiology]

        IMAGING

        • General Features

          • Radiographic Findings

            • Fluoroscopic Findings

              • CT Findings

                • MR Findings

                  • Echocardiographic Findings

                    • Angiographic Findings

                      • Imaging Recommendations

                        DIFFERENTIAL DIAGNOSIS

                          PATHOLOGY

                          • General Features

                            • Gross Pathologic & Surgical Features

                              • Microscopic Features

                                CLINICAL ISSUES

                                • Presentation

                                  • Natural History & Prognosis

                                    • Treatment

                                      DIAGNOSTIC CHECKLIST

                                      • Consider

                                        • Image Interpretation Pearls

                                          Selected References

                                          1. Diaz-Frias J et al: Scimitar syndrome. StatPearls, 2021
                                          2. Mounir R et al: Adults forms of scimitar syndrome. J Card Surg. 35(7):1697-9, 2020
                                          3. Trimech T et al: Scimitar syndrome with bicuspid aortic valve. A case report of cross-sectional non- invasive imaging allowing a complete anatomical and functional assessment. Ann Cardiol Angeiol (Paris). 69(5):317-22, 2020
                                          4. Han F et al: The case of the missing pulmonary vein: a focused update on anomalous pulmonary venous connection in congenital cardiovascular disease. Echocardiography. 36(10):1930-5, 2019
                                          5. Masrani A et al: Anatomical associations and radiological characteristics of scimitar syndrome on CT and MR. J Cardiovasc Comput Tomogr. 12(4):286-9, 2018
                                          6. O'Byrne ML et al: Asymptomatic atresia of the anomalous pulmonary vein in a patient with scimitar syndrome presenting in childhood. Cardiol Young. 28(2):329-33, 2018
                                          7. Althomali SA et al: Uncommon presentation of adult-form scimitar syndrome associated with single left pulmonary vein in a pregnant woman. BMJ Case Rep. 2017
                                          8. Bernal Garnes N et al: Magnetic resonance imaging in the assessment of anomalous pulmonary venous connections. Radiologia. 58(2):111-9, 2015
                                          9. Nawrocki P et al: Scimitar syndrome associated with abnormal hepatic venous drainage. World J Pediatr Congenit Heart Surg. 6(3):474-6, 2015
                                          10. Yamakawa H et al: Transcatheter embolization for hemoptysis associated with anomalous systemic artery in a patient with scimitar syndrome. Springerplus. 4:422, 2015
                                          11. Ciçek S et al: Scimitar syndrome: the curved Turkish sabre. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 17(1):56-61, 2014
                                          12. Dyer KT et al: Imaging in congenital pulmonary vein anomalies: the role of computed tomography. Pediatr Radiol. 44(9):1158-68; quiz 1155-7, 2014
                                          13. Ganesan S et al: Prenatal findings in total anomalous pulmonary venous return: a diagnostic road map starts with obstetric screening views. J Ultrasound Med. 33(7):1193-207, 2014
                                          14. Goudarzi M et al: Anomalous unilateral single pulmonary vein mimicking pulmonary nodules on HRCT of the lungs and scimitar syndrome on chest radiograph: multidetector CT findings. J Thorac Imaging. 24(2):142-6, 2009
                                          15. Gavazzi E et al: Scimitar syndrome: comprehensive, noninvasive assessment with cardiovascular magnetic resonance imaging. Circulation. 118(3):e63-4, 2008
                                          16. Lee ML: Isolated and complex scimitar vein anomalies and their differentiation from the meandering right pulmonary vein. Yonsei Med J. 48(6):973-80, 2007
                                          17. Berrocal T et al: Congenital anomalies of the tracheobronchial tree, lung, and mediastinum: embryology, radiology, and pathology. Radiographics. 24(1):e17, 2004
                                          18. Sinha R et al: Scimitar syndrome: imaging by magnetic resonance angiography and Doppler echocardiography. Indian J Chest Dis Allied Sci. 46(4):283-6, 2004
                                          19. Konen E et al: Congenital pulmonary venolobar syndrome: spectrum of helical CT findings with emphasis on computerized reformatting. Radiographics. 23(5):1175-84, 2003
                                          20. Kramer U et al: Scimitar syndrome: morphological diagnosis and assessment of hemodynamic significance by magnetic resonance imaging. Eur Radiol. 13 Suppl 4:L147-50, 2003
                                          21. Marco de Lucas E et al: Scimitar syndrome: complete anatomical and functional diagnosis with gadolinium-enhanced and velocity-encoded cine MRI. Pediatr Radiol. 33(10):716-8, 2003
                                          22. Vanderheyden M et al: Partial anomalous pulmonary venous connection or scimitar syndrome. Heart. 89(7):761, 2003
                                          23. Reddy R et al: Scimitar syndrome: a rare cause of haemoptysis. Eur J Cardiothorac Surg. 22(5):821, 2002
                                          24. Vaes MF et al: Scimitar syndrome. JBR-BTR. 85(3):160-1, 2002
                                          25. Zylak CJ et al: Developmental lung anomalies in the adult: radiologic-pathologic correlation. Radiographics. 22 Spec No:S25-43, 2002
                                          26. Do KH et al: Systemic arterial supply to the lungs in adults: spiral CT findings. Radiographics. 21(2):387-402, 2001
                                          27. Gilkeson RC et al: Gadolinium-enhanced magnetic resonance angiography in scimitar syndrome: diagnosis and postoperative evaluation. Tex Heart Inst J. 27(3):309-11, 2000
                                          28. Huddleston CB et al: Scimitar syndrome presenting in infancy. Ann Thorac Surg. 67(1):154-9; discussion 160, 1999
                                          29. Henk CB et al: Scimitar syndrome: MR assessment of hemodynamic significance. J Comput Assist Tomogr. 21(4):628-30, 1997
                                          30. Baran R et al: Scimitar syndrome: confirmation of diagnosis by a noninvasive technique (MRI). Eur Radiol. 6(1):92-4, 1996
                                          31. Ramseyer L et al: The scimitar syndrome: demonstration with magnetic resonance imaging. J Okla State Med Assoc. 89(9):324-5, 1996
                                          32. Vrachliotis TG et al: Hypogenetic lung syndrome: functional and anatomic evaluation with magnetic resonance imaging and magnetic resonance angiography. J Magn Reson Imaging. 6(5):798-800, 1996
                                          33. Boothroyd AE et al: Shoe, scimitar or sequestration: a shifting spectrum. Pediatr Radiol. 25(8):652-3, 1995
                                          34. Woodring JH et al: Congenital pulmonary venolobar syndrome revisited. Radiographics. 14(2):349-69, 1994
                                          35. Figa FH et al: Horseshoe lung--a case report with unusual bronchial and pleural anomalies and a proposed new classification. Pediatr Radiol. 23(1):44-7, 1993
                                          36. Gao YA et al: Scimitar syndrome in infancy. J Am Coll Cardiol. 22(3):873-82, 1993
                                          37. Baxter R et al: Scimitar syndrome: cine magnetic resonance imaging demonstration of anomalous pulmonary venous drainage. Ann Thorac Surg. 50(1):121-3, 1990
                                          38. Partridge JB et al: Scimitar etcetera--the dysmorphic right lung. Clin Radiol. 39(1):11-9, 1988
                                          Related Anatomy
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                                          Related Differential Diagnoses
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                                          References
                                          Tables

                                          Tables

                                          KEY FACTS

                                          • Terminology

                                            • Imaging

                                              • Clinical Issues

                                                TERMINOLOGY

                                                • Synonyms

                                                  • Hypogenetic lung syndrome, congenital pulmonary venolobar syndrome

                                                • Definitions

                                                  • Scimitar syndrome triad: Right lung hypoplasia, anomalous right pulmonary venous connection to inferior vena cava (IVC), & anomalous systemic arterial supply to right lower lobe
                                                    • Scimitar vein represents form of partial anomalous pulmonary venous return (PAPVR)
                                                  • Category: Acyanotic, right-sided cardiac chamber enlargement, ↑ pulmonary vascularity
                                                  • Hemodynamics: Venous flow from right lung returns to right atrium → volume overload of right heart [atrial septal defect (ASD)-type physiology]

                                                IMAGING

                                                • General Features

                                                  • Radiographic Findings

                                                    • Fluoroscopic Findings

                                                      • CT Findings

                                                        • MR Findings

                                                          • Echocardiographic Findings

                                                            • Angiographic Findings

                                                              • Imaging Recommendations

                                                                DIFFERENTIAL DIAGNOSIS

                                                                  PATHOLOGY

                                                                  • General Features

                                                                    • Gross Pathologic & Surgical Features

                                                                      • Microscopic Features

                                                                        CLINICAL ISSUES

                                                                        • Presentation

                                                                          • Natural History & Prognosis

                                                                            • Treatment

                                                                              DIAGNOSTIC CHECKLIST

                                                                              • Consider

                                                                                • Image Interpretation Pearls

                                                                                  Selected References

                                                                                  1. Diaz-Frias J et al: Scimitar syndrome. StatPearls, 2021
                                                                                  2. Mounir R et al: Adults forms of scimitar syndrome. J Card Surg. 35(7):1697-9, 2020
                                                                                  3. Trimech T et al: Scimitar syndrome with bicuspid aortic valve. A case report of cross-sectional non- invasive imaging allowing a complete anatomical and functional assessment. Ann Cardiol Angeiol (Paris). 69(5):317-22, 2020
                                                                                  4. Han F et al: The case of the missing pulmonary vein: a focused update on anomalous pulmonary venous connection in congenital cardiovascular disease. Echocardiography. 36(10):1930-5, 2019
                                                                                  5. Masrani A et al: Anatomical associations and radiological characteristics of scimitar syndrome on CT and MR. J Cardiovasc Comput Tomogr. 12(4):286-9, 2018
                                                                                  6. O'Byrne ML et al: Asymptomatic atresia of the anomalous pulmonary vein in a patient with scimitar syndrome presenting in childhood. Cardiol Young. 28(2):329-33, 2018
                                                                                  7. Althomali SA et al: Uncommon presentation of adult-form scimitar syndrome associated with single left pulmonary vein in a pregnant woman. BMJ Case Rep. 2017
                                                                                  8. Bernal Garnes N et al: Magnetic resonance imaging in the assessment of anomalous pulmonary venous connections. Radiologia. 58(2):111-9, 2015
                                                                                  9. Nawrocki P et al: Scimitar syndrome associated with abnormal hepatic venous drainage. World J Pediatr Congenit Heart Surg. 6(3):474-6, 2015
                                                                                  10. Yamakawa H et al: Transcatheter embolization for hemoptysis associated with anomalous systemic artery in a patient with scimitar syndrome. Springerplus. 4:422, 2015
                                                                                  11. Ciçek S et al: Scimitar syndrome: the curved Turkish sabre. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 17(1):56-61, 2014
                                                                                  12. Dyer KT et al: Imaging in congenital pulmonary vein anomalies: the role of computed tomography. Pediatr Radiol. 44(9):1158-68; quiz 1155-7, 2014
                                                                                  13. Ganesan S et al: Prenatal findings in total anomalous pulmonary venous return: a diagnostic road map starts with obstetric screening views. J Ultrasound Med. 33(7):1193-207, 2014
                                                                                  14. Goudarzi M et al: Anomalous unilateral single pulmonary vein mimicking pulmonary nodules on HRCT of the lungs and scimitar syndrome on chest radiograph: multidetector CT findings. J Thorac Imaging. 24(2):142-6, 2009
                                                                                  15. Gavazzi E et al: Scimitar syndrome: comprehensive, noninvasive assessment with cardiovascular magnetic resonance imaging. Circulation. 118(3):e63-4, 2008
                                                                                  16. Lee ML: Isolated and complex scimitar vein anomalies and their differentiation from the meandering right pulmonary vein. Yonsei Med J. 48(6):973-80, 2007
                                                                                  17. Berrocal T et al: Congenital anomalies of the tracheobronchial tree, lung, and mediastinum: embryology, radiology, and pathology. Radiographics. 24(1):e17, 2004
                                                                                  18. Sinha R et al: Scimitar syndrome: imaging by magnetic resonance angiography and Doppler echocardiography. Indian J Chest Dis Allied Sci. 46(4):283-6, 2004
                                                                                  19. Konen E et al: Congenital pulmonary venolobar syndrome: spectrum of helical CT findings with emphasis on computerized reformatting. Radiographics. 23(5):1175-84, 2003
                                                                                  20. Kramer U et al: Scimitar syndrome: morphological diagnosis and assessment of hemodynamic significance by magnetic resonance imaging. Eur Radiol. 13 Suppl 4:L147-50, 2003
                                                                                  21. Marco de Lucas E et al: Scimitar syndrome: complete anatomical and functional diagnosis with gadolinium-enhanced and velocity-encoded cine MRI. Pediatr Radiol. 33(10):716-8, 2003
                                                                                  22. Vanderheyden M et al: Partial anomalous pulmonary venous connection or scimitar syndrome. Heart. 89(7):761, 2003
                                                                                  23. Reddy R et al: Scimitar syndrome: a rare cause of haemoptysis. Eur J Cardiothorac Surg. 22(5):821, 2002
                                                                                  24. Vaes MF et al: Scimitar syndrome. JBR-BTR. 85(3):160-1, 2002
                                                                                  25. Zylak CJ et al: Developmental lung anomalies in the adult: radiologic-pathologic correlation. Radiographics. 22 Spec No:S25-43, 2002
                                                                                  26. Do KH et al: Systemic arterial supply to the lungs in adults: spiral CT findings. Radiographics. 21(2):387-402, 2001
                                                                                  27. Gilkeson RC et al: Gadolinium-enhanced magnetic resonance angiography in scimitar syndrome: diagnosis and postoperative evaluation. Tex Heart Inst J. 27(3):309-11, 2000
                                                                                  28. Huddleston CB et al: Scimitar syndrome presenting in infancy. Ann Thorac Surg. 67(1):154-9; discussion 160, 1999
                                                                                  29. Henk CB et al: Scimitar syndrome: MR assessment of hemodynamic significance. J Comput Assist Tomogr. 21(4):628-30, 1997
                                                                                  30. Baran R et al: Scimitar syndrome: confirmation of diagnosis by a noninvasive technique (MRI). Eur Radiol. 6(1):92-4, 1996
                                                                                  31. Ramseyer L et al: The scimitar syndrome: demonstration with magnetic resonance imaging. J Okla State Med Assoc. 89(9):324-5, 1996
                                                                                  32. Vrachliotis TG et al: Hypogenetic lung syndrome: functional and anatomic evaluation with magnetic resonance imaging and magnetic resonance angiography. J Magn Reson Imaging. 6(5):798-800, 1996
                                                                                  33. Boothroyd AE et al: Shoe, scimitar or sequestration: a shifting spectrum. Pediatr Radiol. 25(8):652-3, 1995
                                                                                  34. Woodring JH et al: Congenital pulmonary venolobar syndrome revisited. Radiographics. 14(2):349-69, 1994
                                                                                  35. Figa FH et al: Horseshoe lung--a case report with unusual bronchial and pleural anomalies and a proposed new classification. Pediatr Radiol. 23(1):44-7, 1993
                                                                                  36. Gao YA et al: Scimitar syndrome in infancy. J Am Coll Cardiol. 22(3):873-82, 1993
                                                                                  37. Baxter R et al: Scimitar syndrome: cine magnetic resonance imaging demonstration of anomalous pulmonary venous drainage. Ann Thorac Surg. 50(1):121-3, 1990
                                                                                  38. Partridge JB et al: Scimitar etcetera--the dysmorphic right lung. Clin Radiol. 39(1):11-9, 1988