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Cardiac
Diagnosis
Congenital Heart Disease
L-Transposition of Great Arteries
L-Transposition of Great Arteries
Jonathan Hero Chung, MD; Prabhakar Rajiah, MBBS, MD, FRCR, FSCMR
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KEY FACTS

  • Terminology

    • Imaging

      • Pathology

        • Clinical Issues

          TERMINOLOGY

          • Abbreviations

            • Levo transposition of great arteries (L-TGA)
            • L = levo = left

          • Synonyms

            • Congenitally corrected TGA (misnomer)
              • Most patients have concomitant congenital abnormalities
            • Discordant transposition
            • Ventricular inversion

          • Definitions

            • Inversion of ventricles and great arteries: Atrioventricular (AV) and ventriculoarterial discordance
            • Ebstein anomaly: Displacement of tricuspid valve into right ventricle (RV)
            • Segmental approach to congenital heart disease (3 steps)
              • Visceroatrial situs: Atria relative to nearby anatomy
                • Situs solitus (S): Normal
                • Situs inversus (I): Inverted
                • Situs ambiguus (A): Ambiguous
              • Ventricular topology: Orientation of ventricular loop
                • Dextro-loop (D): Rightward
                • Levo-loop (L): Leftward
              • Great arterial position
                • Solitus (S): Normal
                • Inversus (I): Inverted
                • L-transposition (L)
                • D-transposition (D)

          IMAGING

          • General Features

            • Radiographic Findings

              • CT Findings

                • MR Findings

                  • Echocardiographic Findings

                    • Imaging Recommendations

                      DIFFERENTIAL DIAGNOSIS

                        PATHOLOGY

                        • General Features

                          • Gross Pathologic & Surgical Features

                            CLINICAL ISSUES

                            • Presentation

                              • Demographics

                                • Natural History & Prognosis

                                  • Treatment

                                    Selected References

                                    1. Ranganath P et al: Computed tomography in adult congenital heart disease. Radiol Clin North Am. 57(1):85-111, 2019
                                    2. Rajiah P et al: Role of CT in congenital heart disease. Curr Treat Options Cardiovasc Med. 19(1):6, 2017
                                    3. Rajiah P et al: Update on the role of cardiac magnetic resonance imaging in congenital heart disease. Curr Treat Options Cardiovasc Med. 19(1):2, 2017
                                    4. Han BK et al: Computed tomography imaging in patients with congenital heart disease part i: rationale and utility. an expert consensus document of the Society of Cardiovascular Computed Tomography (SCCT): endorsed by the Society of Pediatric Radiology (SPR) and the North American Society of Cardiac Imaging (NASCI). J Cardiovasc Comput Tomogr. 9(6):475-92, 2015
                                    5. Han BK et al: Computed tomography imaging in patients with congenital heart disease, part 2: technical recommendations. an expert consensus document of the Society of Cardiovascular Computed Tomography (SCCT): endorsed by the Society of Pediatric Radiology (SPR) and the North American Society of Cardiac Imaging (NASCI). J Cardiovasc Comput Tomogr. 9(6):493-513, 2015
                                    6. Ghoshhajra BB et al: Radiation dose reduction in pediatric cardiac computed tomography: experience from a tertiary medical center. Pediatr Cardiol. 35(1):171-9, 2014
                                    7. Ghoshhajra BB et al: Adult congenital heart disease imaging with second-generation dual-source computed tomography: initial experiences and findings. Congenit Heart Dis. 7(6):516-25, 2012
                                    8. Wallis GA et al: Congenitally corrected transposition. Orphanet J Rare Dis. 6:22, 2011
                                    9. Cohen MD et al: MRI of surgical repair of transposition of the great vessels. AJR Am J Roentgenol. 194(1):250-60, 2010
                                    10. Hornung TS et al: Congenitally corrected transposition of the great arteries. Heart. 96(14):1154-61, 2010
                                    11. Lapierre C et al: Segmental approach to imaging of congenital heart disease. Radiographics. 30(2):397-411, 2010
                                    12. Martins P et al: Transposition of the great arteries. Orphanet J Rare Dis. 3:27, 2008
                                    13. Skinner J et al: Transposition of the great arteries: from fetus to adult. Heart. 94(9):1227-35, 2008
                                    14. Chang DS et al: Congenitally corrected transposition of the great arteries: imaging with 16-MDCT. AJR Am J Roentgenol. 188(5):W428-30, 2007
                                    15. Dorfman AL et al: Magnetic resonance imaging evaluation of congenital heart disease: conotruncal anomalies. J Cardiovasc Magn Reson. 8(4):645-59, 2006
                                    16. Kantarci M et al: Congenitally corrected transposition of the great arteries: MDCT angiography findings and interpretation of complex coronary anatomy. Int J Cardiovasc Imaging. 2006
                                    17. Mohrs OK et al: Time-resolved contrast-enhanced MR angiography of the thorax in adults with congenital heart disease. AJR Am J Roentgenol. 187(4):1107-14, 2006
                                    18. Warnes CA: Transposition of the great arteries. Circulation. 114(24):2699-709, 2006
                                    19. Sorensen TS et al: Operator-independent isotropic three-dimensional magnetic resonance imaging for morphology in congenital heart disease: a validation study. Circulation. 110(2):163-9, 2004
                                    20. Tulevski II et al: Regional and global right ventricular dysfunction in asymptomatic or minimally symptomatic patients with congenitally corrected transposition. Cardiol Young. 14(2):168-73, 2004
                                    21. Karl TR et al: Congenitally corrected transposition of the great arteries. In Mavroudis C et al: Pediatric Cardiac Surgery. 3rd ed. Philadelphia: Mosby. 476-95, 2003
                                    22. Dodge-Khatami A et al: Comparable systemic ventricular function in healthy adults and patients with unoperated congenitally corrected transposition using MRI dobutamine stress testing. Ann Thorac Surg. 73(6):1759-64, 2002
                                    23. Tulevski II et al: Usefulness of magnetic resonance imaging dobutamine stress in asymptomatic and minimally symptomatic patients with decreased cardiac reserve from congenital heart disease (complete and corrected transposition of the great arteries and subpulmonic obstruction). Am J Cardiol. 89(9):1077-81, 2002
                                    24. Schmidt M et al: Tc-99m MIBI SPECT correlated with magnetic resonance imaging for cardiac evaluation of a patient with congenitally corrected transposition of the great arteries (L-TGA). Clin Nucl Med. 26(8):714-5, 2001
                                    25. Freedom RM. Congenitally corrected transposition of the great arteries. In Moller et al. Pediatric Cardiovascular Medicine. 1st ed. Philadelphia: Churchill Livingstone. 391-408, 2000
                                    26. Schmidt M et al: Congenitally corrected transposition of the great arteries (L-TGA) with situs inversus totalis in adulthood: findings with magnetic resonance imaging. Magn Reson Imaging. 18(4):417-22, 2000
                                    27. Reddy GP et al: Case 15: Congenitally corrected transposition of the great arteries. Radiology. 213:102-6, 1999
                                    28. Chen SJ et al: Three-dimensional reconstruction of abnormal ventriculoarterial relationship by electron beam CT. J Comput Assist Tomogr. 22(4):560-8, 1998
                                    29. Formanek AG: MR imaging of congenitally corrected transposition of the great vessels in adults. AJR Am J Roentgenol. 154(4):898-9, 1990
                                    30. Park JH et al: MR imaging of congenitally corrected transposition of the great vessels in adults. AJR Am J Roentgenol. 153(3):491-4, 1989
                                    Related Anatomy
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                                    Related Differential Diagnoses
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                                    References
                                    Tables

                                    Tables

                                    KEY FACTS

                                    • Terminology

                                      • Imaging

                                        • Pathology

                                          • Clinical Issues

                                            TERMINOLOGY

                                            • Abbreviations

                                              • Levo transposition of great arteries (L-TGA)
                                              • L = levo = left

                                            • Synonyms

                                              • Congenitally corrected TGA (misnomer)
                                                • Most patients have concomitant congenital abnormalities
                                              • Discordant transposition
                                              • Ventricular inversion

                                            • Definitions

                                              • Inversion of ventricles and great arteries: Atrioventricular (AV) and ventriculoarterial discordance
                                              • Ebstein anomaly: Displacement of tricuspid valve into right ventricle (RV)
                                              • Segmental approach to congenital heart disease (3 steps)
                                                • Visceroatrial situs: Atria relative to nearby anatomy
                                                  • Situs solitus (S): Normal
                                                  • Situs inversus (I): Inverted
                                                  • Situs ambiguus (A): Ambiguous
                                                • Ventricular topology: Orientation of ventricular loop
                                                  • Dextro-loop (D): Rightward
                                                  • Levo-loop (L): Leftward
                                                • Great arterial position
                                                  • Solitus (S): Normal
                                                  • Inversus (I): Inverted
                                                  • L-transposition (L)
                                                  • D-transposition (D)

                                            IMAGING

                                            • General Features

                                              • Radiographic Findings

                                                • CT Findings

                                                  • MR Findings

                                                    • Echocardiographic Findings

                                                      • Imaging Recommendations

                                                        DIFFERENTIAL DIAGNOSIS

                                                          PATHOLOGY

                                                          • General Features

                                                            • Gross Pathologic & Surgical Features

                                                              CLINICAL ISSUES

                                                              • Presentation

                                                                • Demographics

                                                                  • Natural History & Prognosis

                                                                    • Treatment

                                                                      Selected References

                                                                      1. Ranganath P et al: Computed tomography in adult congenital heart disease. Radiol Clin North Am. 57(1):85-111, 2019
                                                                      2. Rajiah P et al: Role of CT in congenital heart disease. Curr Treat Options Cardiovasc Med. 19(1):6, 2017
                                                                      3. Rajiah P et al: Update on the role of cardiac magnetic resonance imaging in congenital heart disease. Curr Treat Options Cardiovasc Med. 19(1):2, 2017
                                                                      4. Han BK et al: Computed tomography imaging in patients with congenital heart disease part i: rationale and utility. an expert consensus document of the Society of Cardiovascular Computed Tomography (SCCT): endorsed by the Society of Pediatric Radiology (SPR) and the North American Society of Cardiac Imaging (NASCI). J Cardiovasc Comput Tomogr. 9(6):475-92, 2015
                                                                      5. Han BK et al: Computed tomography imaging in patients with congenital heart disease, part 2: technical recommendations. an expert consensus document of the Society of Cardiovascular Computed Tomography (SCCT): endorsed by the Society of Pediatric Radiology (SPR) and the North American Society of Cardiac Imaging (NASCI). J Cardiovasc Comput Tomogr. 9(6):493-513, 2015
                                                                      6. Ghoshhajra BB et al: Radiation dose reduction in pediatric cardiac computed tomography: experience from a tertiary medical center. Pediatr Cardiol. 35(1):171-9, 2014
                                                                      7. Ghoshhajra BB et al: Adult congenital heart disease imaging with second-generation dual-source computed tomography: initial experiences and findings. Congenit Heart Dis. 7(6):516-25, 2012
                                                                      8. Wallis GA et al: Congenitally corrected transposition. Orphanet J Rare Dis. 6:22, 2011
                                                                      9. Cohen MD et al: MRI of surgical repair of transposition of the great vessels. AJR Am J Roentgenol. 194(1):250-60, 2010
                                                                      10. Hornung TS et al: Congenitally corrected transposition of the great arteries. Heart. 96(14):1154-61, 2010
                                                                      11. Lapierre C et al: Segmental approach to imaging of congenital heart disease. Radiographics. 30(2):397-411, 2010
                                                                      12. Martins P et al: Transposition of the great arteries. Orphanet J Rare Dis. 3:27, 2008
                                                                      13. Skinner J et al: Transposition of the great arteries: from fetus to adult. Heart. 94(9):1227-35, 2008
                                                                      14. Chang DS et al: Congenitally corrected transposition of the great arteries: imaging with 16-MDCT. AJR Am J Roentgenol. 188(5):W428-30, 2007
                                                                      15. Dorfman AL et al: Magnetic resonance imaging evaluation of congenital heart disease: conotruncal anomalies. J Cardiovasc Magn Reson. 8(4):645-59, 2006
                                                                      16. Kantarci M et al: Congenitally corrected transposition of the great arteries: MDCT angiography findings and interpretation of complex coronary anatomy. Int J Cardiovasc Imaging. 2006
                                                                      17. Mohrs OK et al: Time-resolved contrast-enhanced MR angiography of the thorax in adults with congenital heart disease. AJR Am J Roentgenol. 187(4):1107-14, 2006
                                                                      18. Warnes CA: Transposition of the great arteries. Circulation. 114(24):2699-709, 2006
                                                                      19. Sorensen TS et al: Operator-independent isotropic three-dimensional magnetic resonance imaging for morphology in congenital heart disease: a validation study. Circulation. 110(2):163-9, 2004
                                                                      20. Tulevski II et al: Regional and global right ventricular dysfunction in asymptomatic or minimally symptomatic patients with congenitally corrected transposition. Cardiol Young. 14(2):168-73, 2004
                                                                      21. Karl TR et al: Congenitally corrected transposition of the great arteries. In Mavroudis C et al: Pediatric Cardiac Surgery. 3rd ed. Philadelphia: Mosby. 476-95, 2003
                                                                      22. Dodge-Khatami A et al: Comparable systemic ventricular function in healthy adults and patients with unoperated congenitally corrected transposition using MRI dobutamine stress testing. Ann Thorac Surg. 73(6):1759-64, 2002
                                                                      23. Tulevski II et al: Usefulness of magnetic resonance imaging dobutamine stress in asymptomatic and minimally symptomatic patients with decreased cardiac reserve from congenital heart disease (complete and corrected transposition of the great arteries and subpulmonic obstruction). Am J Cardiol. 89(9):1077-81, 2002
                                                                      24. Schmidt M et al: Tc-99m MIBI SPECT correlated with magnetic resonance imaging for cardiac evaluation of a patient with congenitally corrected transposition of the great arteries (L-TGA). Clin Nucl Med. 26(8):714-5, 2001
                                                                      25. Freedom RM. Congenitally corrected transposition of the great arteries. In Moller et al. Pediatric Cardiovascular Medicine. 1st ed. Philadelphia: Churchill Livingstone. 391-408, 2000
                                                                      26. Schmidt M et al: Congenitally corrected transposition of the great arteries (L-TGA) with situs inversus totalis in adulthood: findings with magnetic resonance imaging. Magn Reson Imaging. 18(4):417-22, 2000
                                                                      27. Reddy GP et al: Case 15: Congenitally corrected transposition of the great arteries. Radiology. 213:102-6, 1999
                                                                      28. Chen SJ et al: Three-dimensional reconstruction of abnormal ventriculoarterial relationship by electron beam CT. J Comput Assist Tomogr. 22(4):560-8, 1998
                                                                      29. Formanek AG: MR imaging of congenitally corrected transposition of the great vessels in adults. AJR Am J Roentgenol. 154(4):898-9, 1990
                                                                      30. Park JH et al: MR imaging of congenitally corrected transposition of the great vessels in adults. AJR Am J Roentgenol. 153(3):491-4, 1989