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Heterotaxy Syndrome
Tyler H. Ternes, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Clinical Issues

          • Diagnostic Checklist

            TERMINOLOGY

            • Synonyms

              • Situs ambiguous
            • Definitions

              • Situs
                • Position of cardiac atria & viscera relative to midline
                • Independent of cardiac apex position
              • Situs solitus
                • Normal position of cardiac atria & viscera
              • Situs inversus
                • Mirror image of normal position
              • Situs ambiguous (heterotaxy)
                • Abnormal arrangement of thoraco-abdominal organs across left-right axis
              • Isomerism
                • Equal parts (bilateral left- or right-sidedness)
              • Cardiac position
                • Refers to global location of heart in chest
                • Left (levoposition), midline (mesoposition), right (dextroposition)
              • Levocardia & dextrocardia refer only to location of cardiac apex
                • Levoversion: Situs inversus with levocardia
                • Dextroversion: Situs solitus with dextrocardia

            IMAGING

            • General Features

              • Radiographic Findings

                • CT Findings

                  • MR Findings

                    • Fluoroscopic Findings

                      • Echocardiographic Findings

                        • Imaging Recommendations

                          DIFFERENTIAL DIAGNOSIS

                            PATHOLOGY

                            • General Features

                              • Staging, Grading, & Classification

                                CLINICAL ISSUES

                                • Presentation

                                  • Demographics

                                    • Natural History & Prognosis

                                      • Treatment

                                        DIAGNOSTIC CHECKLIST

                                        • Consider

                                          • Image Interpretation Pearls

                                            • Reporting Tips

                                              Selected References

                                              1. Kim SJ: Heterotaxy syndrome. Korean Circ J. 41(5):227-32, 2011
                                              2. Lapierre C et al: Segmental approach to imaging of congenital heart disease. Radiographics. 30(2):397-411, 2010
                                              3. Maldjian PD et al: Approach to dextrocardia in adults: review. AJR Am J Roentgenol. 188(6 Suppl):S39-49; quiz S35-8, 2007
                                              4. Garg N et al: Dextrocardia: an analysis of cardiac structures in 125 patients. Int J Cardiol. 88(2-3):143-55; discussion 55-6, 2003
                                              5. Fulcher AS et al: Abdominal manifestations of situs anomalies in adults. Radiographics. 22(6):1439-56, 2002
                                              6. Applegate KE et al: Situs revisited: imaging of the heterotaxy syndrome. Radiographics. 19(4):837-52; discussion 53-4, 1999
                                              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

                                                      • Diagnostic Checklist

                                                        TERMINOLOGY

                                                        • Synonyms

                                                          • Situs ambiguous
                                                        • Definitions

                                                          • Situs
                                                            • Position of cardiac atria & viscera relative to midline
                                                            • Independent of cardiac apex position
                                                          • Situs solitus
                                                            • Normal position of cardiac atria & viscera
                                                          • Situs inversus
                                                            • Mirror image of normal position
                                                          • Situs ambiguous (heterotaxy)
                                                            • Abnormal arrangement of thoraco-abdominal organs across left-right axis
                                                          • Isomerism
                                                            • Equal parts (bilateral left- or right-sidedness)
                                                          • Cardiac position
                                                            • Refers to global location of heart in chest
                                                            • Left (levoposition), midline (mesoposition), right (dextroposition)
                                                          • Levocardia & dextrocardia refer only to location of cardiac apex
                                                            • Levoversion: Situs inversus with levocardia
                                                            • Dextroversion: Situs solitus with dextrocardia

                                                        IMAGING

                                                        • General Features

                                                          • Radiographic Findings

                                                            • CT Findings

                                                              • MR Findings

                                                                • Fluoroscopic Findings

                                                                  • Echocardiographic Findings

                                                                    • Imaging Recommendations

                                                                      DIFFERENTIAL DIAGNOSIS

                                                                        PATHOLOGY

                                                                        • General Features

                                                                          • Staging, Grading, & Classification

                                                                            CLINICAL ISSUES

                                                                            • Presentation

                                                                              • Demographics

                                                                                • Natural History & Prognosis

                                                                                  • Treatment

                                                                                    DIAGNOSTIC CHECKLIST

                                                                                    • Consider

                                                                                      • Image Interpretation Pearls

                                                                                        • Reporting Tips

                                                                                          Selected References

                                                                                          1. Kim SJ: Heterotaxy syndrome. Korean Circ J. 41(5):227-32, 2011
                                                                                          2. Lapierre C et al: Segmental approach to imaging of congenital heart disease. Radiographics. 30(2):397-411, 2010
                                                                                          3. Maldjian PD et al: Approach to dextrocardia in adults: review. AJR Am J Roentgenol. 188(6 Suppl):S39-49; quiz S35-8, 2007
                                                                                          4. Garg N et al: Dextrocardia: an analysis of cardiac structures in 125 patients. Int J Cardiol. 88(2-3):143-55; discussion 55-6, 2003
                                                                                          5. Fulcher AS et al: Abdominal manifestations of situs anomalies in adults. Radiographics. 22(6):1439-56, 2002
                                                                                          6. Applegate KE et al: Situs revisited: imaging of the heterotaxy syndrome. Radiographics. 19(4):837-52; discussion 53-4, 1999