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Scimitar Syndrome
Jonathan Hero Chung, MD; Prabhakar Rajiah, MBBS, MD, FRCR, FSCMR
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Clinical Issues

          TERMINOLOGY

          • Synonyms

            • Hypogenetic lung/pulmonary venolobar syndrome
          • Definitions

            • Right lung hypoplasia with anomalous right pulmonary venous connection to inferior vena cava (IVC)
            • Often associated: Anomalous systemic arterial supply
            • Category: Acyanotic, right-sided cardiac chamber enlargement, increased pulmonary vascularity (partial anomalous pulmonary venous return)
            • Hemodynamics: Venous flow from right lung returns to systemic veins → L-to-R shunt

          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. 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
                                            2. Wang H et al: Scimitar syndrome in children and adults: natural history, outcomes, and risk analysis. Ann Thorac Surg. 105(2):592-98, 2018
                                            3. Nazarian J et al: Scimitar sign. J Thorac Imaging. 28(4):W61, 2013
                                            4. Wasilewska E et al: Unilateral hyperlucent lung in children. AJR Am J Roentgenol. 198(5):W400-14, 2012
                                            5. Korkmaz AA et al: Scimitar syndrome: a complex form of anomalous pulmonary venous return. J Card Surg. 26(5):529-34, 2011
                                            6. Biyyam DR et al: Congenital lung abnormalities: embryologic features, prenatal diagnosis, and postnatal radiologic-pathologic correlation. Radiographics. 30(6):1721-38, 2010
                                            7. Rajiah P et al: Computed tomography of pulmonary venous variants and anomalies. J Cardiovasc Comput Tomogr. 4(3):155-63, 2010
                                            8. Ho ML et al: MDCT of partial anomalous pulmonary venous return (PAPVR) in adults. J Thorac Imaging. 24(2):89-95, 2009
                                            9. Navas Lobato MA et al: Scimitar syndrome. Clin Cardiol. 32(7):E15-6, 2009
                                            10. Gavazzi E et al: Scimitar syndrome: comprehensive, noninvasive assessment with cardiovascular magnetic resonance imaging. Circulation. 118(3):e63-4, 2008
                                            11. Tjang YS et al: Scimitar syndrome presenting in adults. J Card Surg. 23(1):71-2, 2008
                                            12. Wang CC et al: Scimitar syndrome: incidence, treatment, and prognosis. Eur J Pediatr. 167(2):155-60, 2008
                                            13. Tsitouridis I et al: Scimitar syndrome versus meandering pulmonary vein: evaluation with three-dimensional computed tomography. Acta Radiol. 47(9):927-32, 2006
                                            14. Yoo SJ et al: The relationship between scimitar syndrome, so-called scimitar variant, meandering right pulmonary vein, horseshoe lung and pulmonary arterial sling. Cardiol Young. 16(3):300-4, 2006
                                            15. Khan MA et al: Usefulness of magnetic resonance angiography for diagnosis of scimitar syndrome in early infancy. Am J Cardiol. 96(9):1313-6, 2005
                                            16. Berrocal T et al: Congenital anomalies of the tracheobronchial tree, lung, and mediastinum: embryology, radiology, and pathology. Radiographics. 24(1):e17, 2004
                                            17. 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
                                            18. Konen E et al: Congenital pulmonary venolobar syndrome: spectrum of helical CT findings with emphasis on computerized reformatting. Radiographics. 23(5):1175-84, 2003
                                            19. 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
                                            20. 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
                                            21. Vanderheyden M et al: Partial anomalous pulmonary venous connection or scimitar syndrome. Heart. 89(7):761, 2003
                                            22. Reddy R et al: Scimitar syndrome: a rare cause of haemoptysis. Eur J Cardiothorac Surg. 22(5):821, 2002
                                            23. Vaes MF et al: Scimitar syndrome. JBR-BTR. 85(3):160-1, 2002
                                            24. Zylak CJ et al: Developmental lung anomalies in the adult: radiologic-pathologic correlation. Radiographics. 22 Spec No:S25-43, 2002
                                            25. Do KH et al: Systemic arterial supply to the lungs in adults: spiral CT findings. Radiographics. 21(2):387-402, 2001
                                            26. 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
                                            27. Huddleston CB et al: Scimitar syndrome presenting in infancy. Ann Thorac Surg. 67(1):154-9; discussion 160, 1999
                                            28. Henk CB et al: Scimitar syndrome: MR assessment of hemodynamic significance. J Comput Assist Tomogr. 21(4):628-30, 1997
                                            29. Baran R et al: Scimitar syndrome: confirmation of diagnosis by a noninvasive technique (MRI). Eur Radiol. 6(1):92-4, 1996
                                            30. 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
                                            31. Boothroyd AE et al: Shoe, scimitar or sequestration: a shifting spectrum. Pediatr Radiol. 25(8):652-3, 1995
                                            32. Woodring JH et al: Congenital pulmonary venolobar syndrome revisited. Radiographics. 14(2):349-69, 1994
                                            33. 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
                                            Related Anatomy
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                                            Related Differential Diagnoses
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                                            References
                                            Tables

                                            Tables

                                            KEY FACTS

                                            • Terminology

                                              • Imaging

                                                • Top Differential Diagnoses

                                                  • Clinical Issues

                                                    TERMINOLOGY

                                                    • Synonyms

                                                      • Hypogenetic lung/pulmonary venolobar syndrome
                                                    • Definitions

                                                      • Right lung hypoplasia with anomalous right pulmonary venous connection to inferior vena cava (IVC)
                                                      • Often associated: Anomalous systemic arterial supply
                                                      • Category: Acyanotic, right-sided cardiac chamber enlargement, increased pulmonary vascularity (partial anomalous pulmonary venous return)
                                                      • Hemodynamics: Venous flow from right lung returns to systemic veins → L-to-R shunt

                                                    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. 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
                                                                                      2. Wang H et al: Scimitar syndrome in children and adults: natural history, outcomes, and risk analysis. Ann Thorac Surg. 105(2):592-98, 2018
                                                                                      3. Nazarian J et al: Scimitar sign. J Thorac Imaging. 28(4):W61, 2013
                                                                                      4. Wasilewska E et al: Unilateral hyperlucent lung in children. AJR Am J Roentgenol. 198(5):W400-14, 2012
                                                                                      5. Korkmaz AA et al: Scimitar syndrome: a complex form of anomalous pulmonary venous return. J Card Surg. 26(5):529-34, 2011
                                                                                      6. Biyyam DR et al: Congenital lung abnormalities: embryologic features, prenatal diagnosis, and postnatal radiologic-pathologic correlation. Radiographics. 30(6):1721-38, 2010
                                                                                      7. Rajiah P et al: Computed tomography of pulmonary venous variants and anomalies. J Cardiovasc Comput Tomogr. 4(3):155-63, 2010
                                                                                      8. Ho ML et al: MDCT of partial anomalous pulmonary venous return (PAPVR) in adults. J Thorac Imaging. 24(2):89-95, 2009
                                                                                      9. Navas Lobato MA et al: Scimitar syndrome. Clin Cardiol. 32(7):E15-6, 2009
                                                                                      10. Gavazzi E et al: Scimitar syndrome: comprehensive, noninvasive assessment with cardiovascular magnetic resonance imaging. Circulation. 118(3):e63-4, 2008
                                                                                      11. Tjang YS et al: Scimitar syndrome presenting in adults. J Card Surg. 23(1):71-2, 2008
                                                                                      12. Wang CC et al: Scimitar syndrome: incidence, treatment, and prognosis. Eur J Pediatr. 167(2):155-60, 2008
                                                                                      13. Tsitouridis I et al: Scimitar syndrome versus meandering pulmonary vein: evaluation with three-dimensional computed tomography. Acta Radiol. 47(9):927-32, 2006
                                                                                      14. Yoo SJ et al: The relationship between scimitar syndrome, so-called scimitar variant, meandering right pulmonary vein, horseshoe lung and pulmonary arterial sling. Cardiol Young. 16(3):300-4, 2006
                                                                                      15. Khan MA et al: Usefulness of magnetic resonance angiography for diagnosis of scimitar syndrome in early infancy. Am J Cardiol. 96(9):1313-6, 2005
                                                                                      16. Berrocal T et al: Congenital anomalies of the tracheobronchial tree, lung, and mediastinum: embryology, radiology, and pathology. Radiographics. 24(1):e17, 2004
                                                                                      17. 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
                                                                                      18. Konen E et al: Congenital pulmonary venolobar syndrome: spectrum of helical CT findings with emphasis on computerized reformatting. Radiographics. 23(5):1175-84, 2003
                                                                                      19. 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
                                                                                      20. 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
                                                                                      21. Vanderheyden M et al: Partial anomalous pulmonary venous connection or scimitar syndrome. Heart. 89(7):761, 2003
                                                                                      22. Reddy R et al: Scimitar syndrome: a rare cause of haemoptysis. Eur J Cardiothorac Surg. 22(5):821, 2002
                                                                                      23. Vaes MF et al: Scimitar syndrome. JBR-BTR. 85(3):160-1, 2002
                                                                                      24. Zylak CJ et al: Developmental lung anomalies in the adult: radiologic-pathologic correlation. Radiographics. 22 Spec No:S25-43, 2002
                                                                                      25. Do KH et al: Systemic arterial supply to the lungs in adults: spiral CT findings. Radiographics. 21(2):387-402, 2001
                                                                                      26. 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
                                                                                      27. Huddleston CB et al: Scimitar syndrome presenting in infancy. Ann Thorac Surg. 67(1):154-9; discussion 160, 1999
                                                                                      28. Henk CB et al: Scimitar syndrome: MR assessment of hemodynamic significance. J Comput Assist Tomogr. 21(4):628-30, 1997
                                                                                      29. Baran R et al: Scimitar syndrome: confirmation of diagnosis by a noninvasive technique (MRI). Eur Radiol. 6(1):92-4, 1996
                                                                                      30. 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
                                                                                      31. Boothroyd AE et al: Shoe, scimitar or sequestration: a shifting spectrum. Pediatr Radiol. 25(8):652-3, 1995
                                                                                      32. Woodring JH et al: Congenital pulmonary venolobar syndrome revisited. Radiographics. 14(2):349-69, 1994
                                                                                      33. 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