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Heterotaxy Syndromes
Randy R. Richardson, MD; Ryan A. Moore, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Pathology

        • Clinical Issues

          TERMINOLOGY

          • Synonyms

            • Situs ambiguous, right/left isomerism, cardiosplenic syndromes, Ivemark syndrome
          • Definitions

            • Disturbance of normal left-right asymmetry in position of thoracic & abdominal organs

          IMAGING

          • General Features

            • Radiographic Findings

              • Echocardiographic Findings

                • Ultrasonographic Findings

                  • CT Findings

                    • MR Findings

                      • Other Modality Findings

                        • Imaging Recommendations

                          DIFFERENTIAL DIAGNOSIS

                            PATHOLOGY

                            • General Features

                              • Staging, Grading, & Classification

                                CLINICAL ISSUES

                                • Presentation

                                  • Demographics

                                    • Natural History & Prognosis

                                      • Treatment

                                        DIAGNOSTIC CHECKLIST

                                        • Image Interpretation Pearls

                                          Selected References

                                          1. Murat SN et al: Diagnosis of heterotaxy syndrome in a patient with multiple congenital cardiac malformations using magnetic resonance imaging. Circ Cardiovasc Imaging. 13(9):e010307, 2020
                                          2. Broda CR et al: Outcomes in adults with congenital heart disease and heterotaxy syndrome: a single-center experience. Congenit Heart Dis. 14(6):885-94, 2019
                                          3. Buca DIP et al: Outcome of prenatally diagnosed fetal heterotaxy: systematic review and meta-analysis. Ultrasound Obstet Gynecol. 51(3):323-30, 2018
                                          4. Cupers S et al: Heterotaxy syndrome with intestinal malrotation, polysplenia and azygos continuity. Clin Pract. 8(1):1004, 2018
                                          5. Mahmood K et al: Heterotaxy syndrome. J Coll Physicians Surg Pak. 28(3):252-3, 2018
                                          6. Sanders SP et al: Classifying heterotaxy syndrome: time for a new approach. Circ Cardiovasc Imaging. 11(2):e007490, 2018
                                          7. Moradi B et al: Fetal echocardiographic evaluation in cases of heterotaxy syndrome. J Clin Ultrasound. 45(7):436-7, 2017
                                          8. Lambert TE et al: Abnormalities of fetal situs: an overview and literature review. Obstet Gynecol Surv. 71(1):33-8, 2016
                                          9. Teele SA et al: Heterotaxy syndrome: proceedings from the 10th international PCICS meeting. World J Pediatr Congenit Heart Surg. 6(4):616-29, 2015
                                          10. Hill SJ et al: Heterotaxy syndrome and malrotation: does isomerism influence risk and decision to treat. J Pediatr Surg. 49(6):934-7; discussion 937, 2014
                                          11. Kothari SS: Non-cardiac issues in patients with heterotaxy syndrome. Ann Pediatr Cardiol. 7(3):187-92, 2014
                                          12. Newman B et al: Heterotaxy syndromes and abnormal bowel rotation. Pediatr Radiol. 44(5):542-51, 2014
                                          13. Wolla CD et al: Cardiovascular manifestations of heterotaxy and related situs abnormalities assessed with CT angiography. J Cardiovasc Comput Tomogr. 7(6):408-16, 2013
                                          14. Balan A et al: Atrial isomerism: a pictorial review. J Cardiovasc Comput Tomogr. 6(2):127-36, 2012
                                          15. Danilov T et al: Bilateral left-sidedness heterotaxy syndrome. J Am Coll Cardiol. 58(1):87, 2011
                                          16. Valsangiacomo Buechel ER et al: Congenital cardiac defects and MR-guided planning of surgery. Magn Reson Imaging Clin N Am. 19(4):823-40; viii, 2011
                                          17. Evans WN: Thoracoabdominal situs: a practical approach accompanied by a short history of descriptive terms. Pediatr Cardiol. 31(7):1049-51, 2010
                                          18. Lapierre C et al: Segmental approach to imaging of congenital heart disease. Radiographics. 30(2):397-411, 2010
                                          19. Ghosh S et al: Anomalies of visceroatrial situs. AJR Am J Roentgenol. 193(4):1107-17, 2009
                                          20. Cohen MS et al: Controversies, genetics, diagnostic assessment, and outcomes relating to the heterotaxy syndrome. Cardiol Young. 17 Suppl 2:29-43, 2007
                                          21. Bartram U et al: Heterotaxy syndrome -- asplenia and polysplenia as indicators of visceral malposition and complex congenital heart disease. Biol Neonate. 88(4):278-90, 2005
                                          22. Hong YK et al: Efficacy of MRI in complicated congenital heart disease with visceral heterotaxy syndrome. J Comput Assist Tomogr. 24(5):671-82, 2000
                                          23. Applegate KE et al: Situs revisited: imaging of the heterotaxy syndrome. Radiographics. 19(4):837-52; discussion 853-4, 1999
                                          Related Anatomy
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                                          Related Differential Diagnoses
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                                          References
                                          Tables

                                          Tables

                                          KEY FACTS

                                          • Terminology

                                            • Imaging

                                              • Pathology

                                                • Clinical Issues

                                                  TERMINOLOGY

                                                  • Synonyms

                                                    • Situs ambiguous, right/left isomerism, cardiosplenic syndromes, Ivemark syndrome
                                                  • Definitions

                                                    • Disturbance of normal left-right asymmetry in position of thoracic & abdominal organs

                                                  IMAGING

                                                  • General Features

                                                    • Radiographic Findings

                                                      • Echocardiographic Findings

                                                        • Ultrasonographic Findings

                                                          • CT Findings

                                                            • MR Findings

                                                              • Other Modality Findings

                                                                • Imaging Recommendations

                                                                  DIFFERENTIAL DIAGNOSIS

                                                                    PATHOLOGY

                                                                    • General Features

                                                                      • Staging, Grading, & Classification

                                                                        CLINICAL ISSUES

                                                                        • Presentation

                                                                          • Demographics

                                                                            • Natural History & Prognosis

                                                                              • Treatment

                                                                                DIAGNOSTIC CHECKLIST

                                                                                • Image Interpretation Pearls

                                                                                  Selected References

                                                                                  1. Murat SN et al: Diagnosis of heterotaxy syndrome in a patient with multiple congenital cardiac malformations using magnetic resonance imaging. Circ Cardiovasc Imaging. 13(9):e010307, 2020
                                                                                  2. Broda CR et al: Outcomes in adults with congenital heart disease and heterotaxy syndrome: a single-center experience. Congenit Heart Dis. 14(6):885-94, 2019
                                                                                  3. Buca DIP et al: Outcome of prenatally diagnosed fetal heterotaxy: systematic review and meta-analysis. Ultrasound Obstet Gynecol. 51(3):323-30, 2018
                                                                                  4. Cupers S et al: Heterotaxy syndrome with intestinal malrotation, polysplenia and azygos continuity. Clin Pract. 8(1):1004, 2018
                                                                                  5. Mahmood K et al: Heterotaxy syndrome. J Coll Physicians Surg Pak. 28(3):252-3, 2018
                                                                                  6. Sanders SP et al: Classifying heterotaxy syndrome: time for a new approach. Circ Cardiovasc Imaging. 11(2):e007490, 2018
                                                                                  7. Moradi B et al: Fetal echocardiographic evaluation in cases of heterotaxy syndrome. J Clin Ultrasound. 45(7):436-7, 2017
                                                                                  8. Lambert TE et al: Abnormalities of fetal situs: an overview and literature review. Obstet Gynecol Surv. 71(1):33-8, 2016
                                                                                  9. Teele SA et al: Heterotaxy syndrome: proceedings from the 10th international PCICS meeting. World J Pediatr Congenit Heart Surg. 6(4):616-29, 2015
                                                                                  10. Hill SJ et al: Heterotaxy syndrome and malrotation: does isomerism influence risk and decision to treat. J Pediatr Surg. 49(6):934-7; discussion 937, 2014
                                                                                  11. Kothari SS: Non-cardiac issues in patients with heterotaxy syndrome. Ann Pediatr Cardiol. 7(3):187-92, 2014
                                                                                  12. Newman B et al: Heterotaxy syndromes and abnormal bowel rotation. Pediatr Radiol. 44(5):542-51, 2014
                                                                                  13. Wolla CD et al: Cardiovascular manifestations of heterotaxy and related situs abnormalities assessed with CT angiography. J Cardiovasc Comput Tomogr. 7(6):408-16, 2013
                                                                                  14. Balan A et al: Atrial isomerism: a pictorial review. J Cardiovasc Comput Tomogr. 6(2):127-36, 2012
                                                                                  15. Danilov T et al: Bilateral left-sidedness heterotaxy syndrome. J Am Coll Cardiol. 58(1):87, 2011
                                                                                  16. Valsangiacomo Buechel ER et al: Congenital cardiac defects and MR-guided planning of surgery. Magn Reson Imaging Clin N Am. 19(4):823-40; viii, 2011
                                                                                  17. Evans WN: Thoracoabdominal situs: a practical approach accompanied by a short history of descriptive terms. Pediatr Cardiol. 31(7):1049-51, 2010
                                                                                  18. Lapierre C et al: Segmental approach to imaging of congenital heart disease. Radiographics. 30(2):397-411, 2010
                                                                                  19. Ghosh S et al: Anomalies of visceroatrial situs. AJR Am J Roentgenol. 193(4):1107-17, 2009
                                                                                  20. Cohen MS et al: Controversies, genetics, diagnostic assessment, and outcomes relating to the heterotaxy syndrome. Cardiol Young. 17 Suppl 2:29-43, 2007
                                                                                  21. Bartram U et al: Heterotaxy syndrome -- asplenia and polysplenia as indicators of visceral malposition and complex congenital heart disease. Biol Neonate. 88(4):278-90, 2005
                                                                                  22. Hong YK et al: Efficacy of MRI in complicated congenital heart disease with visceral heterotaxy syndrome. J Comput Assist Tomogr. 24(5):671-82, 2000
                                                                                  23. Applegate KE et al: Situs revisited: imaging of the heterotaxy syndrome. Radiographics. 19(4):837-52; discussion 853-4, 1999