link
Bookmarks
Heterotaxy, Cardiosplenic Syndromes
Michael D. Puchalski, MD; Thomas A. Miller, DO
To access 4,300 diagnoses written by the world's leading experts in radiology, please log in or subscribe.Log inSubscribe
0
4
2
1

KEY FACTS

  • Terminology

    • Imaging

      • Pathology

        • Clinical Issues

          TERMINOLOGY

          • Synonyms

            • Heterotaxy syndrome
            • Visceral heterotaxy
            • Situs ambiguous
            • Terminology is confusing with multiple terms for similar anatomic combinations
              • Listed below are terms used for certain combinations of findings (not necessarily synonymous)
            • Right atrial appendage (RA) isomerism
              • Asplenia
              • Bilateral right sidedness
            • Left atrial appendage (LA) isomerism
              • Polysplenia
              • Bilateral left sidedness
          • Definitions

            • Abnormality where internal thoraco-abdominal organs demonstrate abnormal arrangement across left-right axis of body
              • Implies that laterality of internal organs is neither situs solitus (normal) nor situs inversus (mirror image of situs solitus)

          IMAGING

          • General Features

            • Ultrasonographic Findings

              • Echocardiographic Findings

                • Imaging Recommendations

                  DIFFERENTIAL DIAGNOSIS

                    PATHOLOGY

                    • General Features

                      CLINICAL ISSUES

                      • Presentation

                        • Demographics

                          • Natural History & Prognosis

                            • Treatment

                              DIAGNOSTIC CHECKLIST

                              • Consider

                                • Image Interpretation Pearls

                                  Selected References

                                  1. Gottschalk I et al: Extracardiac anomalies in prenatally diagnosed heterotaxy syndromes. Ultrasound Obstet Gynecol. ePub, 2015
                                  2. Khan MS et al: Contemporary outcomes of surgical repair of total anomalous pulmonary venous connection in patients with heterotaxy syndrome. Ann Thorac Surg. ePub, 2015
                                  3. Kothari SS: Non-cardiac issues in patients with heterotaxy syndrome. Ann Pediatr Cardiol. 7(3):187-92, 2014
                                  4. Anagnostopoulos PV et al: Improved current era outcomes in patients with heterotaxy syndromes. Eur J Cardiothorac Surg. 35(5):871-7; discussion 877-8, 2009
                                  5. Cohen MS et al: Controversies, genetics, diagnostic assessment, and outcomes relating to the heterotaxy syndrome. Cardiol Young. 17 Suppl 2:29-43, 2007
                                  Related Anatomy
                                  Loading...
                                  Related Differential Diagnoses
                                  Loading...
                                  References
                                  Tables

                                  Tables

                                  KEY FACTS

                                  • Terminology

                                    • Imaging

                                      • Pathology

                                        • Clinical Issues

                                          TERMINOLOGY

                                          • Synonyms

                                            • Heterotaxy syndrome
                                            • Visceral heterotaxy
                                            • Situs ambiguous
                                            • Terminology is confusing with multiple terms for similar anatomic combinations
                                              • Listed below are terms used for certain combinations of findings (not necessarily synonymous)
                                            • Right atrial appendage (RA) isomerism
                                              • Asplenia
                                              • Bilateral right sidedness
                                            • Left atrial appendage (LA) isomerism
                                              • Polysplenia
                                              • Bilateral left sidedness
                                          • Definitions

                                            • Abnormality where internal thoraco-abdominal organs demonstrate abnormal arrangement across left-right axis of body
                                              • Implies that laterality of internal organs is neither situs solitus (normal) nor situs inversus (mirror image of situs solitus)

                                          IMAGING

                                          • General Features

                                            • Ultrasonographic Findings

                                              • Echocardiographic Findings

                                                • Imaging Recommendations

                                                  DIFFERENTIAL DIAGNOSIS

                                                    PATHOLOGY

                                                    • General Features

                                                      CLINICAL ISSUES

                                                      • Presentation

                                                        • Demographics

                                                          • Natural History & Prognosis

                                                            • Treatment

                                                              DIAGNOSTIC CHECKLIST

                                                              • Consider

                                                                • Image Interpretation Pearls

                                                                  Selected References

                                                                  1. Gottschalk I et al: Extracardiac anomalies in prenatally diagnosed heterotaxy syndromes. Ultrasound Obstet Gynecol. ePub, 2015
                                                                  2. Khan MS et al: Contemporary outcomes of surgical repair of total anomalous pulmonary venous connection in patients with heterotaxy syndrome. Ann Thorac Surg. ePub, 2015
                                                                  3. Kothari SS: Non-cardiac issues in patients with heterotaxy syndrome. Ann Pediatr Cardiol. 7(3):187-92, 2014
                                                                  4. Anagnostopoulos PV et al: Improved current era outcomes in patients with heterotaxy syndromes. Eur J Cardiothorac Surg. 35(5):871-7; discussion 877-8, 2009
                                                                  5. Cohen MS et al: Controversies, genetics, diagnostic assessment, and outcomes relating to the heterotaxy syndrome. Cardiol Young. 17 Suppl 2:29-43, 2007