Pediatric and Adult Congenital Cardiology
Transposition of the Great Arteries
D-transposition requires rapid confirmation of anatomy, maintenance of effective intracardiac mixing, and neonatal anatomic repair; lifelong surveillance after arterial switch focuses on coronary obstruction, neo-aortic disease, ventricular function, and pulmonary arterial lesions.
Initial Decision
Establish the anatomic branch before choosing palliation or repair
Management diverges by ventriculoarterial anatomy, septal defects, outflow obstruction, and coronary pattern.
Use transthoracic echocardiography as the decisive initial study in suspected D-TGA to establish ventriculoarterial discordance, define ventricular septal and atrial communications, assess outflow anatomy, ventricular performance, and map coronary features relevant to arterial switch planning. Echocardiography also identifies risk factors that affect operative planning. jaccjaccD-Transposition of the Great Arteries: The Current Era of the Arterial Switch Operation
Treat D-TGA as a neonatal surgical condition rather than a lesion for delayed outpatient decision-making. In simple D-TGA, the arterial switch operation (ASO) is the established anatomic corrective procedure; it transects and switches the great arteries and transfers the coronary arteries to the neo-aortic root. jacc+1jaccD-Transposition of the Great Arteries: The Current Era of the Arterial Switch OperationNatureArterial Switch Operation in Congenital Heart Disease | Surgery | Clinical Sciences | Health sciences | Topics | Nature Index
Separate D-TGA from congenitally corrected TGA (CCTGA) because CCTGA commonly coexists with ventricular septal defect, pulmonary stenosis, or atrial septal defect and has a different longitudinal surgical and ventricular-risk framework. Oxford AcademicOxford AcademicCongenitally corrected transposition of the great arteries: one ...
Document whether the anatomy is simple D-TGA or includes ventricular septal defect, pulmonary outflow obstruction, or a Taussig-Bing-type physiology; associated anatomy changes the surgical strategy and follow-up targets. jacc+1jacc2025 ACC/AHA/HRS/ISACHD/SCAI Guideline for the ... - JACCScienceDirectTwenty-Three-Year Experience With the Arterial Switch Operation: Expectations and Long-Term Outcomes - ScienceDirect
Specifically report single and intramural coronary arteries to the congenital surgical team; both remain risk factors with ASO. jaccjaccD-Transposition of the Great Arteries: The Current Era of the Arterial Switch Operation
Avoid routine preoperative CT, cardiac MR, or catheterization when echocardiography adequately defines neonatal D-TGA anatomy; these modalities have a larger role after repair when echocardiographic windows are limited. ScienceDirectScienceDirectTransposition of the Great Vessels — The arterial switch operation, the atrial switch operation, the coronaries - ScienceDirect
Preoperative Care
Use prostaglandin and atrial septostomy selectively to restore effective mixing
The immediate objective is adequate systemic oxygen delivery while definitive repair is organized.
In an infant with D-TGA and inadequate physiologic mixing, use prostaglandin E1 and/or balloon atrial septostomy (BAS) to improve mixing while preparing for definitive repair. The need is physiologic and anatomic, not automatic: expert review concludes that routine use of either BAS or prostaglandin E1 is not indicated for every patient with simple D-TGA. jaccjaccD-Transposition of the Great Arteries: The Current Era of the Arterial Switch Operation
Escalate to BAS when the atrial communication is restrictive or when clinically inadequate mixing persists despite medical stabilization. BAS is a catheter-based procedure that enlarges an atrial septal communication; its purpose is preoperative stabilization, not definitive correction of ventriculoarterial discordance. NatureNatureArterial Switch Operation in Congenital Heart Disease | Surgery | Clinical Sciences | Health sciences | Topics | Nature Index
Once anatomy and coronary pattern are defined and the infant is stabilized, avoid unnecessary delay to definitive surgery. Early ASO improves outcomes and reduces costs in simple D-TGA, with low operative mortality in the contemporary surgical era. jaccjaccD-Transposition of the Great Arteries: The Current Era of the Arterial Switch Operation
Use serial echocardiography when clinical status changes to reassess atrial communication, ventricular function, outflow anatomy, and operative risk features. jaccjaccD-Transposition of the Great Arteries: The Current Era of the Arterial Switch Operation
Do not interpret BAS as eliminating the need for timely ASO; it is a bridge to anatomic repair. jacc+1jaccD-Transposition of the Great Arteries: The Current Era of the Arterial Switch OperationNatureArterial Switch Operation in Congenital Heart Disease | Surgery | Clinical Sciences | Health sciences | Topics | Nature Index
Transfer infants requiring BAS or ASO to a center with pediatric interventional cardiology, congenital cardiac surgery, and postoperative cardiac intensive care capability. jacc+1jaccD-Transposition of the Great Arteries: The Current Era of the Arterial Switch OperationNatureArterial Switch Operation in Congenital Heart Disease | Surgery | Clinical Sciences | Health sciences | Topics | Nature Index
Surgery
Choose arterial switch as the standard neonatal anatomic repair and plan around coronary risk
The ASO restores the morphologic left ventricle to the systemic circulation and transfers the coronaries.
For neonatal D-TGA, ASO is the current surgical treatment of choice and the established corrective operation for ventriculoarterial discordance. The procedure includes transection of the aorta and pulmonary artery, coronary reimplantation into the neo-aortic root, and anatomic realignment of the great vessels. Nature+1NatureArterial Switch Operation in Congenital Heart Disease | Surgery | Clinical Sciences | Health sciences | Topics | Nature IndexAHA JournalsNormal Coronary Flow Reserve After Arterial Switch Operation for ...
The dominant technical and prognostic discriminator is coronary anatomy. Single or intramural coronary arteries remain ASO risk factors, so operative planning and subsequent ischemia surveillance should not treat coronary transfer as a completed issue once the neonatal operation ends. jaccjaccD-Transposition of the Great Arteries: The Current Era of the Arterial Switch Operation
Counsel families that contemporary early mortality in experienced centers is reported below 5%, but survival does not eliminate the need for reintervention surveillance. Late targets include pulmonary or neo-aortic valve stenosis, neo-aortic root dilation, coronary complications, and reoperation. Nature+2NatureArterial Switch Operation in Congenital Heart Disease | Surgery | Clinical Sciences | Health sciences | Topics | Nature IndexScienceDirectTwenty-Three-Year Experience With the Arterial Switch Operation: Expectations and Long-Term Outcomes - ScienceDirectOxford AcademicLong-term outcomes of the arterial switch operation for transposition ...
Use ASO follow-up imaging to interrogate both reconstructed great arteries and ventricular function; branch pulmonary artery obstruction and neo-aortic disease are distinct late pathways. jacc+2jaccACC/AHA 2008 Guidelines for the Management of Adults With Congenital Heart Disease: Executive Summary: A Report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Develop Guidelines for the Management of Adults With Congenital Heart Disease) Developed in Collaboration With the American Society of Echocardiography, Heart Rhythm Society, International Society for Adult Congenital Heart Disease, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeonsjacc2025 ACC/AHA/HRS/ISACHD/SCAI Guideline for the ... - JACCOxford AcademicLong-term outcomes of the arterial switch operation for transposition ...
When postoperative ventricular dysfunction or arrhythmia develops, prioritize coronary insufficiency in the differential rather than attributing symptoms only to nonspecific postoperative status. jaccjaccD-Transposition of the Great Arteries: The Current Era of the Arterial Switch Operation
Patients repaired with older atrial-switch procedures require a different surveillance framework from ASO survivors because the systemic ventricle is morphologic right ventricle rather than left ventricle. ScienceDirect+1ScienceDirectTransposition of the Great Vessels — The arterial switch operation, the atrial switch operation, the coronaries - ScienceDirectScienceDirectReoperations in Adolescents and Adults After Prior Arterial Switch Operation: The Society of Thoracic Surgeons Congenital Heart Surgery Database Analysis - ScienceDirect
Long-Term Care
Surveil arterial-switch survivors for coronary, neo-aortic, pulmonary arterial, ventricular, and rhythm complications
Follow-up must continue through adulthood even when childhood repair was uncomplicated.
Adults after ASO need longitudinal assessment in adult congenital heart disease care. Major late concerns are coronary insufficiency and myocardial ischemia, ventricular dysfunction, arrhythmias, stenosis at great-artery anastomoses, and aortic or pulmonary regurgitation. AHA Journals+1AHA Journals2018 AHA/ACC Guideline for the Management of Adults With ...jaccACC/AHA 2008 Guidelines for the Management of Adults With Congenital Heart Disease: Executive Summary: A Report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Develop Guidelines for the Management of Adults With Congenital Heart Disease) Developed in Collaboration With the American Society of Echocardiography, Heart Rhythm Society, International Society for Adult Congenital Heart Disease, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons
Use echocardiography as the routine structural and functional surveillance tool, then add cardiac CT or MR when echocardiographic acoustic windows are poor or when postoperative great-vessel or coronary anatomy requires more complete definition. CT and MR have an increasing role in the late postoperative period. ScienceDirectScienceDirectTransposition of the Great Vessels — The arterial switch operation, the atrial switch operation, the coronaries - ScienceDirect
Treat new exertional symptoms, arrhythmia, ventricular dysfunction, or unexplained clinical deterioration as a coronary-warning phenotype in a patient with prior ASO. Coronary insufficiency and arrhythmias are uncommon but associated with sudden death; the appropriate response is coronary-focused evaluation rather than reassurance from a remote neonatal repair. jaccjaccD-Transposition of the Great Arteries: The Current Era of the Arterial Switch Operation
Before pregnancy, obtain comprehensive anatomic and functional assessment, including coronary anatomy. Pregnancy is generally well tolerated after ASO when important cardiovascular residua are absent, but this assessment determines whether that assumption applies to the individual patient. jaccjaccACC/AHA 2008 Guidelines for the Management of Adults With Congenital Heart Disease: Executive Summary: A Report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Develop Guidelines for the Management of Adults With Congenital Heart Disease) Developed in Collaboration With the American Society of Echocardiography, Heart Rhythm Society, International Society for Adult Congenital Heart Disease, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons
At each longitudinal review, document ventricular function, rhythm history, neo-aortic root and valve status, and evidence of pulmonary arterial or anastomotic obstruction. AHA Journals+1AHA Journals2018 AHA/ACC Guideline for the Management of Adults With ...jaccACC/AHA 2008 Guidelines for the Management of Adults With Congenital Heart Disease: Executive Summary: A Report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Develop Guidelines for the Management of Adults With Congenital Heart Disease) Developed in Collaboration With the American Society of Echocardiography, Heart Rhythm Society, International Society for Adult Congenital Heart Disease, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons
Increase surveillance intensity when prior imaging shows neo-aortic dilation or regurgitation, branch pulmonary artery stenosis, ventricular dysfunction, or concerning coronary anatomy. jacc+2jaccD-Transposition of the Great Arteries: The Current Era of the Arterial Switch OperationjaccACC/AHA 2008 Guidelines for the Management of Adults With Congenital Heart Disease: Executive Summary: A Report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Develop Guidelines for the Management of Adults With Congenital Heart Disease) Developed in Collaboration With the American Society of Echocardiography, Heart Rhythm Society, International Society for Adult Congenital Heart Disease, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeonsjacc2025 ACC/AHA/HRS/ISACHD/SCAI Guideline for the ... - JACC
Refer patients with suspected coronary insufficiency, significant neo-aortic valve/root disease, great-vessel obstruction, or sustained arrhythmia to an adult congenital heart disease center for integrated imaging and reintervention planning. AHA Journals+2AHA Journals2018 AHA/ACC Guideline for the Management of Adults With ...jaccACC/AHA 2008 Guidelines for the Management of Adults With Congenital Heart Disease: Executive Summary: A Report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Develop Guidelines for the Management of Adults With Congenital Heart Disease) Developed in Collaboration With the American Society of Echocardiography, Heart Rhythm Society, International Society for Adult Congenital Heart Disease, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic SurgeonsScienceDirectTransposition of the Great Vessels — The arterial switch operation, the atrial switch operation, the coronaries - ScienceDirect
Pregnancy planning after arterial switch
Do not base pregnancy counseling solely on absence of symptoms. Perform comprehensive preconception anatomic and functional assessment, explicitly including coronary anatomy; in the absence of important cardiovascular residua, pregnancy is generally well tolerated after ASO. jaccjaccACC/AHA 2008 Guidelines for the Management of Adults With Congenital Heart Disease: Executive Summary: A Report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Develop Guidelines for the Management of Adults With Congenital Heart Disease) Developed in Collaboration With the American Society of Echocardiography, Heart Rhythm Society, International Society for Adult Congenital Heart Disease, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons
Identify neo-aortic regurgitation or root dilation, ventricular dysfunction, arrhythmia, coronary concerns, and great-vessel obstruction before conception because each represents a residual lesion requiring individualized risk assessment. AHA Journals+1AHA Journals2018 AHA/ACC Guideline for the Management of Adults With ...jaccACC/AHA 2008 Guidelines for the Management of Adults With Congenital Heart Disease: Executive Summary: A Report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Develop Guidelines for the Management of Adults With Congenital Heart Disease) Developed in Collaboration With the American Society of Echocardiography, Heart Rhythm Society, International Society for Adult Congenital Heart Disease, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons
Adult Assessment
Identify the prior repair before interpreting adult TGA physiology
The operative history determines the systemic ventricle, failure mode, and imaging priorities.
Confirm whether the patient underwent ASO, atrial switch (Mustard or Senning), Rastelli-type repair, or another anatomy-specific operation before applying an adult surveillance plan. CT and MR reviews distinguish the differing postoperative anatomy and clinical role of imaging after arterial versus atrial switch procedures. ScienceDirectScienceDirectTransposition of the Great Vessels — The arterial switch operation, the atrial switch operation, the coronaries - ScienceDirect
An ASO survivor has coronary-transfer and neo-aortic/great-vessel surveillance priorities. By contrast, atrial-switch survivors have a systemic morphologic right ventricle, and publications of adult reoperations and long-term outcome analyze these populations separately. ScienceDirect+1ScienceDirectTransposition of the Great Vessels — The arterial switch operation, the atrial switch operation, the coronaries - ScienceDirectScienceDirectReoperations in Adolescents and Adults After Prior Arterial Switch Operation: The Society of Thoracic Surgeons Congenital Heart Surgery Database Analysis - ScienceDirect
For D-TGA with ventricular septal defect and pulmonary stenosis, historical and contemporary Rastelli pathways have distinct conduit and outflow considerations; avoid labeling all repaired TGA as an ASO result. jaccjacc2025 ACC/AHA/HRS/ISACHD/SCAI Guideline for the ... - JACC
Obtain operative reports whenever possible; the phrase “TGA repair” is insufficient to determine coronary anatomy, systemic ventricular morphology, or reintervention risk. ScienceDirect+1ScienceDirectTransposition of the Great Vessels — The arterial switch operation, the atrial switch operation, the coronaries - ScienceDirectScienceDirectReoperations in Adolescents and Adults After Prior Arterial Switch Operation: The Society of Thoracic Surgeons Congenital Heart Surgery Database Analysis - ScienceDirect
Use cross-sectional imaging selectively to clarify postoperative anatomy when prior records are unavailable or echocardiography is nondiagnostic. ScienceDirectScienceDirectTransposition of the Great Vessels — The arterial switch operation, the atrial switch operation, the coronaries - ScienceDirect
References
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- Arterial Switch Operation in Congenital Heart Disease | Surgery | Clinical Sciences | Health sciences | Topics | Nature Index — www.nature.com · www.nature.com
- ACC/AHA 2008 Guidelines for the Management of Adults With ... — www.ahajournals.org · www.ahajournals.org
- Normal Coronary Flow Reserve After Arterial Switch Operation for ... — www.ahajournals.org · www.ahajournals.org
- 2018 AHA/ACC Guideline for the Management of Adults With ... — www.ahajournals.org · www.ahajournals.org
- Association Between Variation in Preoperative Care Before Arterial ... — www.ahajournals.org · www.ahajournals.org
- ACC/AHA 2008 Guidelines for the Management of Adults With Congenital Heart Disease: Executive Summary: A Report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Develop Guidelines for the Management of Adults With Congenital Heart Disease) Developed in Collaboration With the American Society of Echocardiography, Heart Rhythm Society, International Society for Adult Congenital Heart Disease, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons — www.jacc.org · www.jacc.org
- 2025 ACC/AHA/HRS/ISACHD/SCAI Guideline for the ... - JACC — www.jacc.org · www.jacc.org
- Long-term outcome in congenitally corrected transposition of ... - JACC — www.jacc.org · www.jacc.org
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- Outcome in adult patients after arterial switch operation for transposition of the great arteries — www.sciencedirect.com · www.sciencedirect.com
- Twenty-Three-Year Experience With the Arterial Switch Operation: Expectations and Long-Term Outcomes - ScienceDirect — www.sciencedirect.com · www.sciencedirect.com
- Reoperations in Adolescents and Adults After Prior Arterial Switch Operation: The Society of Thoracic Surgeons Congenital Heart Surgery Database Analysis - ScienceDirect — www.sciencedirect.com · www.sciencedirect.com
- Coronary Artery Anatomy in Congenital Heart Disease — onlinelibrary.wiley.com · onlinelibrary.wiley.com
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