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 are dependent on associated anomalies
Segmental analysis & description of anatomic relationships are 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
Tous C et al: Ex vivo cardiovascular magnetic resonance diffusion weighted imaging in congenital heart disease, an insight into the microstructures of tetralogy of Fallot, biventricular and univentricular systemic right ventricle. J Cardiovasc Magn Reson. 22(1):69, 2020
Lozier MR et al: Levo-transposition of the great arteries in an adult patient: management considerations and treatment strategy. Cureus. 11(3):e4306, 2019
Al-Zahrani RS et al: Transposition of the great arteries: a laterality defect in the group of heterotaxy syndromes or an outflow tract malformation? Ann Pediatr Cardiol. 11(3):237-49, 2018
Mainwaring RD et al: An analysis of left ventricular retraining in patients with dextro- and levo-transposition of the great arteries. Ann Thorac Surg. 105(3):823-9, 2018
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
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
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
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
Gutberlet M et al: [Preoperative and postoperative imaging in patients with transposition of the great arteries.] Radiologe. 51(1):15-22, 2011
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 (VA) discordance
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 are dependent on associated anomalies
Segmental analysis & description of anatomic relationships are 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
Tous C et al: Ex vivo cardiovascular magnetic resonance diffusion weighted imaging in congenital heart disease, an insight into the microstructures of tetralogy of Fallot, biventricular and univentricular systemic right ventricle. J Cardiovasc Magn Reson. 22(1):69, 2020
Lozier MR et al: Levo-transposition of the great arteries in an adult patient: management considerations and treatment strategy. Cureus. 11(3):e4306, 2019
Al-Zahrani RS et al: Transposition of the great arteries: a laterality defect in the group of heterotaxy syndromes or an outflow tract malformation? Ann Pediatr Cardiol. 11(3):237-49, 2018
Mainwaring RD et al: An analysis of left ventricular retraining in patients with dextro- and levo-transposition of the great arteries. Ann Thorac Surg. 105(3):823-9, 2018
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
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
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
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
Gutberlet M et al: [Preoperative and postoperative imaging in patients with transposition of the great arteries.] Radiologe. 51(1):15-22, 2011
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
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