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Diagnosis
Developmental Abnormalities
Pulmonary Circulation
Scimitar Syndrome
Scimitar Syndrome
Santiago Martínez-Jiménez, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            • Diagnostic Checklist

              TERMINOLOGY

              • Synonyms

                • Hypogenetic lung syndrome
                • Pulmonary venolobar syndrome

              • Definitions

                • Partial or total anomalous pulmonary venous return of right lung to the inferior vena cava above or below the diaphragm
                • Infantile and pediatric/adult variants
                • Associated abnormalities in descending frequency
                  • Abnormal right lung lobation and right lung hypoplasia (~ 100%)
                  • Dextroposition of heart; right pulmonary artery hypoplasia (60%)
                  • Systemic arterialization of right lower lung (60%)
                  • Secundum atrial septal defect (40% overall, 80-90% in infantile variant)
                  • Right diaphragmatic hernia (15%)
                  • Horseshoe lung
                  • Infantile: Ventricular septal defect, patent ductus, hypoplastic aortic arch, coarctation, tetralogy of Fallot, anomalous origin of left coronary artery, truncus arteriosus

              IMAGING

              • General Features

                • Radiographic Findings

                  • CT Findings

                    • MR Findings

                      • Echocardiographic Findings

                        • Angiographic Findings

                          • Imaging Recommendations

                            DIFFERENTIAL DIAGNOSIS

                              PATHOLOGY

                              • General Features

                                • Gross Pathologic & Surgical Features

                                  CLINICAL ISSUES

                                  • Presentation

                                    • Demographics

                                      • Natural History & Prognosis

                                        • Treatment

                                          DIAGNOSTIC CHECKLIST

                                          • Image Interpretation Pearls

                                            Selected References

                                            1. Ali YZ et al: Scimitar syndrome. Eur Heart J Cardiovasc Imaging. 21(8):939, 2020
                                            2. Diaz-Frias J et al: Scimitar Syndrome 2020
                                            3. Manso PH et al: Scimitar sign in the absence of anomalous pulmonary venous drainage: a case report. Eur Heart J Case Rep. 3(2), 2019
                                            4. Wang H et al: Scimitar syndrome in children and adults: natural history, outcomes, and risk analysis. Ann Thorac Surg. 105(2):592-98, 2018
                                            5. Bo I et al: Variants of the scimitar syndrome. Cardiol Young. 26(5):941-7, 2016
                                            6. Korkmaz AA et al: Scimitar syndrome: a complex form of anomalous pulmonary venous return. J Card Surg. 26(5):529-34, 2011
                                            7. Midyat L et al: Eponym. Scimitar syndrome. Eur J Pediatr. 169(10):1171-7, 2010
                                            8. Gudjonsson U et al: Scimitar syndrome. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 9(1):56-62, 2006
                                            9. Agarwal PP et al: MDCT of anomalous unilateral single pulmonary vein. AJR Am J Roentgenol. 183(5):1241-3, 2004
                                            10. Berrocal T et al: Congenital anomalies of the tracheobronchial tree, lung, and mediastinum: embryology, radiology, and pathology. Radiographics. 24(1):e17, 2004
                                            11. Konen E et al: Congenital pulmonary venolobar syndrome: spectrum of helical CT findings with emphasis on computerized reformatting. Radiographics. 23(5):1175-84, 2003
                                            12. Kamijoh M et al: Horseshoe lung with bilateral vascular anomalies: a rare variant of hypogenetic lung syndrome (scimitar syndrome). Pediatr Int. 44(4):443-5, 2002
                                            13. Zylak CJ et al: Developmental lung anomalies in the adult: radiologic-pathologic correlation. Radiographics. 22 Spec No(S25-43, 2002
                                            14. 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
                                            15. Cairns RA et al: Pediatric case of the day. Hypogenetic lung syndrome (scimitar syndrome) with right-sided congenital diaphragmatic hernia. Radiographics. 15(2):496-9, 1995
                                            16. Woodring JH et al: Congenital pulmonary venolobar syndrome revisited. Radiographics. 14(2):349-69, 1994
                                            17. Dupuis C et al: "Infantile" form of the scimitar syndrome with pulmonary hypertension. Am J Cardiol. 71(15):1326-30, 1993
                                            18. Pomerantz SM et al: False pulmonary artery catheter measurements due to the scimitar (hypogenetic lung) syndrome. Potential for iatrogenic pulmonary edema. Chest. 103(6):1895-7, 1993
                                            19. Dupuis C et al: The "adult" form of the scimitar syndrome. Am J Cardiol. 70(4):502-7, 1992
                                            20. Heron CW et al: Anomalous systemic venous drainage occurring in association with the hypogenetic lung syndrome. Clin Radiol. 39(4):446-9, 1988
                                            21. Gilman MJ et al: Hypoplastic right pulmonary artery in the hypogenetic lung syndrome. J Comput Assist Tomogr. 6(5):1015-8, 1982
                                            22. Goodman LR et al: Meandering right pulmonary vein simulating the Scimitar syndrome. Chest. 62(4):510-2, 1972
                                            23. Roehm JO Jr et al: Radiographic features of the scimitar syndrome. Radiology. 86(5):856-9, 1966
                                            Related Anatomy
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                                            Related Differential Diagnoses
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                                            References
                                            Tables

                                            Tables

                                            KEY FACTS

                                            • Terminology

                                              • Imaging

                                                • Top Differential Diagnoses

                                                  • Pathology

                                                    • Clinical Issues

                                                      • Diagnostic Checklist

                                                        TERMINOLOGY

                                                        • Synonyms

                                                          • Hypogenetic lung syndrome
                                                          • Pulmonary venolobar syndrome

                                                        • Definitions

                                                          • Partial or total anomalous pulmonary venous return of right lung to the inferior vena cava above or below the diaphragm
                                                          • Infantile and pediatric/adult variants
                                                          • Associated abnormalities in descending frequency
                                                            • Abnormal right lung lobation and right lung hypoplasia (~ 100%)
                                                            • Dextroposition of heart; right pulmonary artery hypoplasia (60%)
                                                            • Systemic arterialization of right lower lung (60%)
                                                            • Secundum atrial septal defect (40% overall, 80-90% in infantile variant)
                                                            • Right diaphragmatic hernia (15%)
                                                            • Horseshoe lung
                                                            • Infantile: Ventricular septal defect, patent ductus, hypoplastic aortic arch, coarctation, tetralogy of Fallot, anomalous origin of left coronary artery, truncus arteriosus

                                                        IMAGING

                                                        • General Features

                                                          • Radiographic Findings

                                                            • CT Findings

                                                              • MR Findings

                                                                • Echocardiographic Findings

                                                                  • Angiographic Findings

                                                                    • Imaging Recommendations

                                                                      DIFFERENTIAL DIAGNOSIS

                                                                        PATHOLOGY

                                                                        • General Features

                                                                          • Gross Pathologic & Surgical Features

                                                                            CLINICAL ISSUES

                                                                            • Presentation

                                                                              • Demographics

                                                                                • Natural History & Prognosis

                                                                                  • Treatment

                                                                                    DIAGNOSTIC CHECKLIST

                                                                                    • Image Interpretation Pearls

                                                                                      Selected References

                                                                                      1. Ali YZ et al: Scimitar syndrome. Eur Heart J Cardiovasc Imaging. 21(8):939, 2020
                                                                                      2. Diaz-Frias J et al: Scimitar Syndrome 2020
                                                                                      3. Manso PH et al: Scimitar sign in the absence of anomalous pulmonary venous drainage: a case report. Eur Heart J Case Rep. 3(2), 2019
                                                                                      4. Wang H et al: Scimitar syndrome in children and adults: natural history, outcomes, and risk analysis. Ann Thorac Surg. 105(2):592-98, 2018
                                                                                      5. Bo I et al: Variants of the scimitar syndrome. Cardiol Young. 26(5):941-7, 2016
                                                                                      6. Korkmaz AA et al: Scimitar syndrome: a complex form of anomalous pulmonary venous return. J Card Surg. 26(5):529-34, 2011
                                                                                      7. Midyat L et al: Eponym. Scimitar syndrome. Eur J Pediatr. 169(10):1171-7, 2010
                                                                                      8. Gudjonsson U et al: Scimitar syndrome. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 9(1):56-62, 2006
                                                                                      9. Agarwal PP et al: MDCT of anomalous unilateral single pulmonary vein. AJR Am J Roentgenol. 183(5):1241-3, 2004
                                                                                      10. Berrocal T et al: Congenital anomalies of the tracheobronchial tree, lung, and mediastinum: embryology, radiology, and pathology. Radiographics. 24(1):e17, 2004
                                                                                      11. Konen E et al: Congenital pulmonary venolobar syndrome: spectrum of helical CT findings with emphasis on computerized reformatting. Radiographics. 23(5):1175-84, 2003
                                                                                      12. Kamijoh M et al: Horseshoe lung with bilateral vascular anomalies: a rare variant of hypogenetic lung syndrome (scimitar syndrome). Pediatr Int. 44(4):443-5, 2002
                                                                                      13. Zylak CJ et al: Developmental lung anomalies in the adult: radiologic-pathologic correlation. Radiographics. 22 Spec No(S25-43, 2002
                                                                                      14. 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
                                                                                      15. Cairns RA et al: Pediatric case of the day. Hypogenetic lung syndrome (scimitar syndrome) with right-sided congenital diaphragmatic hernia. Radiographics. 15(2):496-9, 1995
                                                                                      16. Woodring JH et al: Congenital pulmonary venolobar syndrome revisited. Radiographics. 14(2):349-69, 1994
                                                                                      17. Dupuis C et al: "Infantile" form of the scimitar syndrome with pulmonary hypertension. Am J Cardiol. 71(15):1326-30, 1993
                                                                                      18. Pomerantz SM et al: False pulmonary artery catheter measurements due to the scimitar (hypogenetic lung) syndrome. Potential for iatrogenic pulmonary edema. Chest. 103(6):1895-7, 1993
                                                                                      19. Dupuis C et al: The "adult" form of the scimitar syndrome. Am J Cardiol. 70(4):502-7, 1992
                                                                                      20. Heron CW et al: Anomalous systemic venous drainage occurring in association with the hypogenetic lung syndrome. Clin Radiol. 39(4):446-9, 1988
                                                                                      21. Gilman MJ et al: Hypoplastic right pulmonary artery in the hypogenetic lung syndrome. J Comput Assist Tomogr. 6(5):1015-8, 1982
                                                                                      22. Goodman LR et al: Meandering right pulmonary vein simulating the Scimitar syndrome. Chest. 62(4):510-2, 1972
                                                                                      23. Roehm JO Jr et al: Radiographic features of the scimitar syndrome. Radiology. 86(5):856-9, 1966