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Transposition of the Great Arteries

  • Author: John R Charpie, MD, PhD; Chief Editor: Howard S Weber, MD, FSCAI  more...
 
Updated: May 12, 2015
 

Background

Transposition of the great arteries (TGA) is the most common cyanotic congenital heart lesion that presents in neonates. The hallmark of transposition of the great arteries is ventriculoarterial discordance, in which the aorta arises from the morphologic right ventricle and the pulmonary artery arises from the morphologic left ventricle. See the images below.

This right ventricular angiogram shows a patient w This right ventricular angiogram shows a patient with transposition of the great arteries. The aorta arises directly from the right-sided anterior right ventricle (10° left anterior oblique [LAO]).
This right ventricular angiogram shows a patient w This right ventricular angiogram shows a patient with transposition of the great arteries. The aorta arises directly from the right-sided anterior right ventricle (70° left anterior oblique [LAO]).
This left ventricular angiogram shows a patient wi This left ventricular angiogram shows a patient with transposition of the great arteries. The pulmonary artery arises directly from the left-sided posterior left ventricle (30° right anterior oblique [RAO]).
This left ventricular angiogram shows a patient wi This left ventricular angiogram shows a patient with transposition of the great arteries. The pulmonary artery arises directly from the left-sided posterior left ventricle (20° cranial).

Although transposition of the great arteries was first described over 2 centuries ago, no treatment was available until the middle of the 20th century, with the development of surgical atrial septectomy in the 1950s and balloon atrial septostomy in the 1960s. These palliative therapies were followed by physiological procedures (atrial switch operation) and anatomic repair (arterial switch operation). Today, the survival rate for infants with transposition of the great arteries is greater than 90%.

The major anatomic classifications of transposition of the great arteries depend on the relationship of the great arteries to each other and/or the infundibular morphology. In approximately 60% of the patients, the aorta is anterior and to the right of the pulmonary artery (dextro-transposition of the great arteries [d-TGA]). However in a subset of patients, the aorta may be anterior and to the left of the pulmonary artery (levo-transposition of the great arteries [l-TGA]). In addition, most patients with transposition of the great arteries (regardless of the spacial orientation of the great arteries) have a subaortic infundibulum, an absence of subpulmonary infundibulum, and fibrous continuity between the mitral valve and the pulmonary valve. Despite these useful classifications, several exceptions are noted, and, hence, discordant ventriculoarterial connection is the only distinguishing characteristic that defines transposition of the great arteries.

From a practical standpoint, the presence or absence of associated cardiac anomalies defines the clinical presentation and surgical management of a patient with transposition of the great arteries. The primary anatomic subtypes are (1) transposition of the great arteries with intact ventricular septum, (2) transposition of the great arteries with ventricular septal defect, (3) transposition of the great arteries with ventricular septal defect and left ventricular outflow tract obstruction, and (4) transposition of the great arteries with ventricular septal defect and pulmonary vascular obstructive disease.

In approximately one third of patients with transposition of the great arteries, the coronary artery anatomy is abnormal, with a left circumflex coronary arising from the right coronary artery (22%), a single right coronary artery (9.5%), a single left coronary artery (3%), or inverted origin of the coronary arteries (3%) representing the most common variants.

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Pathophysiology

The pulmonary and systemic circulations function in parallel, rather than in series. Oxygenated pulmonary venous blood returns to the left atrium and left ventricle but is recirculated to the pulmonary vascular bed via the abnormal pulmonary arterial connection to the left ventricle. Deoxygenated systemic venous blood returns to the right atrium and right ventricle where it is subsequently pumped to the systemic circulation, effectively bypassing the lungs. This parallel circulatory arrangement results in a deficient oxygen supply to the tissues and an excessive right and left ventricular workload. It is incompatible with prolonged survival unless mixing of oxygenated and deoxygenated blood occurs at some anatomic level.

The following are 3 common anatomic sites for mixing of oxygenated and deoxygenated blood in transposition of the great arteries:

  • Atrial septal defect
  • Ventricular septal defect (See the images below.)
    This 2-dimensional echocardiogram (parasternal lon This 2-dimensional echocardiogram (parasternal long-axis view) shows a patient with transposition of the great arteries and ventricular septal defect. The pulmonary artery arises from the posterior (left) ventricular, dives posteriorly, and bifurcates immediately into left and right branch pulmonary arteries. A large ventricular septal defect is present in the outlet septum.
    This 2-dimensional echocardiogram (apical 4-chambe This 2-dimensional echocardiogram (apical 4-chamber view) shows a patient with transposition of the great arteries and ventricular septal defect. The anterior aorta arises from the right-sided right ventricle.
  • Patent ductus arteriosus

One or all of these lesions can be present in concert with dextro-transposition of the great arteries, and the degree of arterial hypoxemia depends on the degree of anatomic mixing.

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Prognosis

The prognosis depends on the specific anatomic substrate and type of surgical therapy used (arterial switch operation, atrial switch operation, or Rastelli procedure).

Overall, perioperative survival following arterial switch operation is greater than 90%. Long-term and arrhythmia-free survival is excellent (approximately 97% at 25 years), and late mortality is predominantly due to sudden death and myocardial infarction.[1]

The overall mortality rate following an atrial level switch is low; however, long-term morbidity associated with systemic (right) ventricular dilatation and failure, systemic atrioventricular (tricuspid) valve regurgitation, and atrial bradyarrhythmias and tachyarrhythmias is significant.

After arterial switch operation, sequelae may include chronotropic incompetence and neoaortic, pulmonary, and coronary artery complications. However, most patients maintain normal systolic function and exercise capacity.[1] A subset of patients may experience profound right ventricular failure, but they may do well with left ventricular retraining and late arterial switch.[2]

Progressive neoaortic root dilation is common and is a risk factor for neoaortic valve regurgitation following arterial switch operation. Continued surveillance of this population is required.[3]

Morbidity/mortality

The mortality rate in untreated patients is approximately 30% in the first week, 50% in the first month, and 90% by the end of the first year. With improved diagnostic, medical, and surgical techniques, the overall short-term and midterm survival rate exceeds 90%.

Long-term complications are secondary to prolonged cyanosis and include polycythemia and hyperviscosity syndrome. These patients may develop headache, decreased exercise tolerance, and stroke. Thrombocytopenia is common in patients with cyanotic congenital heart disease leading to bleeding complications.

Patients with a large ventricular septal defect, a patent ductus arteriosus, or both may have an early predilection for congestive heart failure, as pulmonary vascular resistance falls with increasing age. Heart failure may be mitigated in those patients with left ventricular outflow tract (pulmonary) stenosi.

Arterioplasty in patients with right ventricular outflow tract obstruction (RVOT) following arterial switch surgery may be an effective and durable management option in the immediate term.[4] In a retrospective study (2004-2013) comprising 223 patients who underwent arterial switch for transposition of the great arteries, 38 patients (16%) developed RVOT within 12.5 months. The surgical morbidity for RVOT management (eg, main pulmonary artery plasty) was 13%, without hospital or late mortality. At the 41.2 months last RVOT postsurgical follow-up, all the patients had NYHA grade 0 or 1 symptoms.[4]

A small percentage (approximately 5%) of patients with transposition of the great arteries (and often a ventricular septal defect) develop accelerated pulmonary vascular obstructive disease and progressive cyanosis despite surgical repair or palliation. Long-term survival in this subgroup is particularly poor.

Complications

Complications include the following:

  • Congestive heart failure
  • Arrhythmia
  • Eisenmenger syndrome (irreversible and progressive pulmonary vascular obstructive disease)

Rare cases of supravalvular aortic stenosis as a late complication of transposition of the great arteries have been reported.[5]

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Epidemiology

Frequency

United States

Despite its overall low prevalence, transposition of the great arteries is the most common etiology for cyanotic congenital heart disease in the newborn.[6] This lesion presents in 5-7% of all patients with congenital heart disease. The overall annual incidence is 20-30 per 100,000 live births, and inheritance is multifactorial. Transposition of the great arteries is isolated in 90% of patients and is rarely associated with syndromes or extracardiac malformations. This congenital heart defect is more common in infants of diabetic mothers.

Race-, sex-, and age-related demographics

No racial predilection is known, but transposition of the great arteries has a 60-70% male predominance.

Patients with transposition of the great arteries usually present with cyanosis in the newborn period, but clinical manifestations and courses are influenced predominantly by the degree of intercirculatory mixing.

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Contributor Information and Disclosures
Author

John R Charpie, MD, PhD Professor and Director, Division of Pediatric Cardiology, Department of Pediatrics, University of Michigan Medical Center

John R Charpie, MD, PhD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Heart Association, Society for Pediatric Research

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: Sorin Group, USA.

Coauthor(s)

Kevin O Maher, MD Associate Professor of Pediatrics, Emory University School of Medicine; Pediatric Cardiac Intensivist, Sibley Heart Center, Children’s Healthcare of Atlanta

Kevin O Maher, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Heart Association

Disclosure: Nothing to disclose.

Specialty Editor Board

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Ameeta Martin, MD Clinical Associate Professor, Department of Pediatric Cardiology, University of Nebraska College of Medicine

Ameeta Martin, MD is a member of the following medical societies: American College of Cardiology

Disclosure: Nothing to disclose.

Chief Editor

Howard S Weber, MD, FSCAI Professor of Pediatrics, Section of Pediatric Cardiology, Pennsylvania State University College of Medicine; Director of Interventional Pediatric Cardiology, Penn State Hershey Children's Hospital

Howard S Weber, MD, FSCAI is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, Society for Cardiovascular Angiography and Interventions

Disclosure: Received income in an amount equal to or greater than $250 from: St. Jude Medical.

Additional Contributors

Charles I Berul, MD Professor of Pediatrics and Integrative Systems Biology, George Washington University School of Medicine; Chief, Division of Cardiology, Children's National Medical Center

Charles I Berul, MD is a member of the following medical societies: American Academy of Pediatrics, Heart Rhythm Society, Cardiac Electrophysiology Society, Pediatric and Congenital Electrophysiology Society, American College of Cardiology, American Heart Association, Society for Pediatric Research

Disclosure: Received grant/research funds from Medtronic for consulting.

References
  1. Khairy P, Clair M, Fernandes SM, Blume ED, Powell AJ, Newburger JW, et al. Cardiovascular outcomes after the arterial switch operation for d-transposition of the great arteries. Circulation. 2013 Jan 22. 127(3):331-9. [Medline].

  2. Watanabe N, Mainwaring RD, Carrillo SA, Lui GK, Reddy VM, Hanley FL. Left Ventricular Retraining and Late Arterial Switch for d-Transposition of the Great Arteries. Ann Thorac Surg. 2015 May. 99(5):1655-63. [Medline].

  3. Co-Vu JG, Ginde S, Bartz PJ, Frommelt PC, Tweddell JS, Earing MG. Long-Term Outcomes of the Neoaorta After Arterial Switch Operation for Transposition of the Great Arteries. Ann Thorac Surg. 2012 Dec 5. [Medline].

  4. Wiggins LM, Kumar SR, Starnes VA, Wells WJ. Arterioplasty for right ventricular outflow tract obstruction after arterial switch is a durable procedure. Ann Thorac Surg. 2015 Apr 25. [Medline].

  5. Maeda T, Koide M, Kunii Y, Watanabe K, Kanzaki T, Ohashi Y. Supravalvular aortic stenosis after arterial switch operation. Asian Cardiovasc Thorac Ann. 2015 May 8. [Medline].

  6. Rao PS. Diagnosis and management of cyanotic congenital heart disease: part I. Indian J Pediatr. 2009 Jan. 76(1):57-70. [Medline].

  7. Rydman R, Gatzoulis MA, Ho SY, et al. Systemic right ventricular fibrosis detected by cardiovascular magnetic resonance is associated with clinical outcome, mainly new-onset atrial arrhythmia, in patients after atrial redirection surgery for transposition of the great arteries. Circ Cardiovasc Imaging. 2015 May. 8(5):[Medline].

  8. Wypij D, Newburger JW, Rappaport LA, et al. The effect of duration of deep hypothermic circulatory arrest in infant heart surgery on late neurodevelopment: the Boston Circulatory Arrest Trial. J Thorac Cardiovasc Surg. 2003 Nov. 126(5):1397-403. [Medline].

  9. [Guideline] Wilson W, Taubert KA, Gewitz M, et al. Prevention of infective endocarditis: guidelines from the American Heart Association: a guideline from the American Heart Association Rheumatic Fever, Endocarditis and Kawasaki Disease Committee, Council on Cardiovascular Disease in the Young, and the Council on Clinical Cardiology, Council on Cardiovascular Surgery and Anesthesia, and the Quality of Care and Outcomes Research Interdisciplinary Working Group. J Am Dent Assoc. 2007 Jun. 138(6):739-45, 747-60. [Medline]. [Full Text].

  10. Horer J, Schreiber C, Dworak E, et al. Long-term results after the Rastelli repair for transposition of the great arteries. Ann Thorac Surg. 2007 Jun. 83(6):2169-75. [Medline].

  11. Kampmann C, Kuroczynski W, Trubel H, et al. Late results after PTCA for coronary stenosis after the arterial switch procedure for transposition of the great arteries. Ann Thorac Surg. 2005 Nov. 80(5):1641-6. [Medline].

  12. Neches WH, Park SC, Ettedgui, JA. Transposition of the great arteries. The Science and Practice of Pediatric Cardiology. 1998. Vol 1: 1463-1503.

  13. Paul MH, Wernovsky G. Transposition of the great arteries. Moss and Adams Heart Disease in Infants, Children, and Adolescents. 1995. Vol 2: 1154-1224.

  14. Pedra SR, Pedra CA, Abizaid AA, et al. Intracoronary ultrasound assessment late after the arterial switch operation for transposition of the great arteries. J Am Coll Cardiol. 2005 Jun 21. 45(12):2061-8. [Medline].

  15. Takeuchi D, Nakanishi T, Tomimatsu H, Nakazawa M. Evaluation of Right Ventricular Performance Long After the Atrial Switch Operation for Transposition of the Great Arteries Using the Doppler Tei Index. Pediatr Cardiol. 2005 Aug 17. [Medline].

 
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This 2-dimensional echocardiogram (parasternal long-axis view) shows a patient with transposition of the great arteries and ventricular septal defect. The pulmonary artery arises from the posterior (left) ventricular, dives posteriorly, and bifurcates immediately into left and right branch pulmonary arteries. A large ventricular septal defect is present in the outlet septum.
This 2-dimensional echocardiogram (apical 4-chamber view) shows a patient with transposition of the great arteries and ventricular septal defect. The anterior aorta arises from the right-sided right ventricle.
This right ventricular angiogram shows a patient with transposition of the great arteries. The aorta arises directly from the right-sided anterior right ventricle (10° left anterior oblique [LAO]).
This right ventricular angiogram shows a patient with transposition of the great arteries. The aorta arises directly from the right-sided anterior right ventricle (70° left anterior oblique [LAO]).
This left ventricular angiogram shows a patient with transposition of the great arteries. The pulmonary artery arises directly from the left-sided posterior left ventricle (30° right anterior oblique [RAO]).
This left ventricular angiogram shows a patient with transposition of the great arteries. The pulmonary artery arises directly from the left-sided posterior left ventricle (20° cranial).
 
 
 
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