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Heterotaxia Syndromes
Suhny Abbara, MD, FACR, FSCCT; Justin Stowell, MD; Christopher M. Walker, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Clinical Issues

          • Diagnostic Checklist

            TERMINOLOGY

            • Synonyms

              • Heterotaxy, visceral heterotaxy, situs ambiguous, right/left isomerism, cardiosplenic syndromes
            • Definitions

              • Abnormal arrangement of thoracoabdominal viscera across left-right body axis
                • Exclusive of mirror image thoracoabdominal arrangement (situs inversus totalis)
              • Isomerism: Dominance or duplication of normally right- or left-sided structures
                • Right isomerism (asplenia syndrome, bilateral right-sidedness)
                • Left isomerism (polysplenia syndrome, bilateral left-sidedness)

            IMAGING

            • General Features

              • Radiographic Findings

                • CT Findings

                  • MR Findings

                    • Echocardiographic 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. Yim D et al: Disharmonious patterns of heterotaxy and isomerism: how often are the classic patterns breached? Circ Cardiovasc Imaging. 11(2):e006917, 2018
                                          2. Franklin RCG et al: Nomenclature for congenital and paediatric cardiac disease: the International Paediatric and Congenital Cardiac Code (IPCCC) and the Eleventh Iteration of the International Classification of Diseases (ICD-11). Cardiol Young. 27(10):1872-938, 2017
                                          3. Abdullah NL et al: Clinics in diagnostic imaging (160). Levocardia with abdominal situs inversus. Singapore Med J. 56(4):198-201; quiz 202, 2015
                                          4. Lin AE et al: Laterality defects in the national birth defects prevention study (1998-2007): birth prevalence and descriptive epidemiology. Am J Med Genet A. 164A(10):2581-91, 2014
                                          5. 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
                                          6. Jacobs JP et al: Heterotaxy: lessons learned about patterns of practice and outcomes from the congenital heart surgery database of the society of thoracic surgeons. World J Pediatr Congenit Heart Surg. 2(2):278-86, 2011
                                          7. Kim SJ: Heterotaxy syndrome. Korean Circ J. 41(5):227-32, 2011
                                          8. Babar JL et al: Application of MR imaging in assessment and follow-up of congenital heart disease in adults. Radiographics. 30(4):1145, 2010
                                          9. Maldjian PD et al: Approach to dextrocardia in adults: review. AJR Am J Roentgenol. 188(6 Suppl):S39-49; quiz S35-8, 2007
                                          10. 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
                                          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

                                                      • Heterotaxy, visceral heterotaxy, situs ambiguous, right/left isomerism, cardiosplenic syndromes
                                                    • Definitions

                                                      • Abnormal arrangement of thoracoabdominal viscera across left-right body axis
                                                        • Exclusive of mirror image thoracoabdominal arrangement (situs inversus totalis)
                                                      • Isomerism: Dominance or duplication of normally right- or left-sided structures
                                                        • Right isomerism (asplenia syndrome, bilateral right-sidedness)
                                                        • Left isomerism (polysplenia syndrome, bilateral left-sidedness)

                                                    IMAGING

                                                    • General Features

                                                      • Radiographic Findings

                                                        • CT Findings

                                                          • MR Findings

                                                            • Echocardiographic 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. Yim D et al: Disharmonious patterns of heterotaxy and isomerism: how often are the classic patterns breached? Circ Cardiovasc Imaging. 11(2):e006917, 2018
                                                                                  2. Franklin RCG et al: Nomenclature for congenital and paediatric cardiac disease: the International Paediatric and Congenital Cardiac Code (IPCCC) and the Eleventh Iteration of the International Classification of Diseases (ICD-11). Cardiol Young. 27(10):1872-938, 2017
                                                                                  3. Abdullah NL et al: Clinics in diagnostic imaging (160). Levocardia with abdominal situs inversus. Singapore Med J. 56(4):198-201; quiz 202, 2015
                                                                                  4. Lin AE et al: Laterality defects in the national birth defects prevention study (1998-2007): birth prevalence and descriptive epidemiology. Am J Med Genet A. 164A(10):2581-91, 2014
                                                                                  5. 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
                                                                                  6. Jacobs JP et al: Heterotaxy: lessons learned about patterns of practice and outcomes from the congenital heart surgery database of the society of thoracic surgeons. World J Pediatr Congenit Heart Surg. 2(2):278-86, 2011
                                                                                  7. Kim SJ: Heterotaxy syndrome. Korean Circ J. 41(5):227-32, 2011
                                                                                  8. Babar JL et al: Application of MR imaging in assessment and follow-up of congenital heart disease in adults. Radiographics. 30(4):1145, 2010
                                                                                  9. Maldjian PD et al: Approach to dextrocardia in adults: review. AJR Am J Roentgenol. 188(6 Suppl):S39-49; quiz S35-8, 2007
                                                                                  10. 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