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L-Transposition of Great Arteries
Randy R. Richardson, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Pathology

        • Clinical Issues

          TERMINOLOGY

          • Synonyms

            • "Congenitally corrected transposition" (misnomer)
            • Discordant transposition
          • Definitions

            • Inversion of ventricles & great arteries: Atrioventricular (AV) discordance & ventriculoarterial discordance
            • Category: Dependent on associated anomalies
              • Ventricular septal defect (VSD) (60-80%): Acyanotic, ↑ pulmonary vascularity
              • Left ventricular outflow tract (subpulmonary) obstruction (30-50%): Cyanotic
              • Dysplasia (Ebstein anomaly) with regurgitation of left-sided AV valve: Pulmonary venous congestion/hypertension
              • Conduction abnormalities, heart block
              • Only 1% have no associated anomalies: True congenitally corrected transposition
            • Hemodynamics
              • Right atrium connects via mitral valve to right-sided morphologic left ventricle (LV), which connects to pulmonary circulation
              • Left atrium connects via tricuspid valve to left-sided morphologic right ventricle (RV), which connects to systemic circulation
              • Hemodynamic sequelae dependent on associated anomalies
            • Segmental analysis & description of anatomic relationships required for complete evaluation of this complex disorder

          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

                                      Selected References

                                      1. Bilal MS et al: Double switch procedure and surgical alternatives for the treatment of congenitally corrected transposition of the great arteries. J Card Surg. 31(4):231-6, 2016
                                      2. Han BK et al: Multi-institutional evaluation of the indications and radiation dose of functional cardiovascular computed tomography (CCT) imaging in congenital heart disease. Int J Cardiovasc Imaging. 32(2):339-46, 2016
                                      3. Rickers C et al: Is the Lecompte technique the last word on transposition of the great arteries repair for all patients? A magnetic resonance imaging study including a spiral technique two decades postoperatively. Interact Cardiovasc Thorac Surg. 2016 Jun;22(6):817-25, 2016
                                      4. Sinning C et al: Long-term course of a patient with congenital corrected transposition of the great arteries and dextrocardia - the Fisher King. Int J Cardiol. 203:436-7, 2016
                                      5. Gutberlet M et al: [Preoperative and postoperative imaging in patients with transposition of the great arteries.] Radiologe. 51(1):15-22, 2011
                                      6. Huang SC et al: Coronary artery anatomy in anatomically corrected malposition of the great arteries and their surgical implications. Eur J Cardiothorac Surg. 39(5):705-10., 2010
                                      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

                                                • "Congenitally corrected transposition" (misnomer)
                                                • Discordant transposition
                                              • Definitions

                                                • Inversion of ventricles & great arteries: Atrioventricular (AV) discordance & ventriculoarterial discordance
                                                • Category: Dependent on associated anomalies
                                                  • Ventricular septal defect (VSD) (60-80%): Acyanotic, ↑ pulmonary vascularity
                                                  • Left ventricular outflow tract (subpulmonary) obstruction (30-50%): Cyanotic
                                                  • Dysplasia (Ebstein anomaly) with regurgitation of left-sided AV valve: Pulmonary venous congestion/hypertension
                                                  • Conduction abnormalities, heart block
                                                  • Only 1% have no associated anomalies: True congenitally corrected transposition
                                                • Hemodynamics
                                                  • Right atrium connects via mitral valve to right-sided morphologic left ventricle (LV), which connects to pulmonary circulation
                                                  • Left atrium connects via tricuspid valve to left-sided morphologic right ventricle (RV), which connects to systemic circulation
                                                  • Hemodynamic sequelae dependent on associated anomalies
                                                • Segmental analysis & description of anatomic relationships required for complete evaluation of this complex disorder

                                              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

                                                                          Selected References

                                                                          1. Bilal MS et al: Double switch procedure and surgical alternatives for the treatment of congenitally corrected transposition of the great arteries. J Card Surg. 31(4):231-6, 2016
                                                                          2. Han BK et al: Multi-institutional evaluation of the indications and radiation dose of functional cardiovascular computed tomography (CCT) imaging in congenital heart disease. Int J Cardiovasc Imaging. 32(2):339-46, 2016
                                                                          3. Rickers C et al: Is the Lecompte technique the last word on transposition of the great arteries repair for all patients? A magnetic resonance imaging study including a spiral technique two decades postoperatively. Interact Cardiovasc Thorac Surg. 2016 Jun;22(6):817-25, 2016
                                                                          4. Sinning C et al: Long-term course of a patient with congenital corrected transposition of the great arteries and dextrocardia - the Fisher King. Int J Cardiol. 203:436-7, 2016
                                                                          5. Gutberlet M et al: [Preoperative and postoperative imaging in patients with transposition of the great arteries.] Radiologe. 51(1):15-22, 2011
                                                                          6. Huang SC et al: Coronary artery anatomy in anatomically corrected malposition of the great arteries and their surgical implications. Eur J Cardiothorac Surg. 39(5):705-10., 2010