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Transposition of the Great Arteries Treatment & Management

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

Medical Care

Initial treatment consists of maintaining ductal patency with continuous intravenous (IV) prostaglandin E1 infusion to promote pulmonary blood flow, increase left atrial pressure, and promote left-to-right intercirculatory mixing at the atrial level. This is particularly important in patients with severe left ventricular outflow tract stenosis or atresia. Prostaglandin therapy may or may not benefit the patient with simple transposition of the great arteries (TGA) and an intact ventricular septum without left ventricular outflow tract obstruction.

Cardiac catheterization, depending on the degree of restriction at the atrial septum and the timing of operative repair, is indicated for a balloon atrial septostomy in severely hypoxemic patients with an inadequate atrial level communication and insufficient mixing. The balloon atrial septostomy is used to increase the atrial level shunt and to improve mixing.

For the ill neonate, metabolic acidosis should be corrected with fluid replacement and bicarbonate administration.

Mechanical ventilation may be necessary if pulmonary edema develops in concert with severe hypoxemia.

Ultimately, the patient requires surgical repair or palliation early in life.

Consultations

Consult with a pediatric cardiologist and a pediatric cardiothoracic surgeon.

Transfer

Transfer may be required for specialized diagnostic, therapeutic, and surgical interventions.

Diet and activity

Patients with transposition of the great arteries and a large ventricular septal defect who have not undergone repair may require increased caloric density during infancy (120-130 kcal/kg/d), particularly if they have significant congestive heart failure and poor weight gain.

Following definitive repair, most patients do not need a special diet.

No specific activity requirements are necessary.

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Surgical Care

Surgical approach depends on the age of the patient at presentation, the presence of associated congenital cardiac lesions, and the experience of the cardiothoracic surgeon with a given surgical technique. Most full-term neonates with uncomplicated transposition of the great arteries can undergo an arterial switch procedure in one operation, with minimal mortality.

Transposition of the great arteries with intact ventricular septum

The ideal operation is an arterial switch procedure. It represents an anatomic repair and establishes ventriculoarterial concordance. This procedure should be performed when the infant is younger than 4 weeks, as the left ventricle may not be able to handle systemic pressure postoperatively if left too long in the low-pressure, low-resistance pulmonary circuit. Rarely, however, depending on the particular coronary artery anatomy (eg, intramural coronary artery), coronary artery translocation may not be feasible, and an arterial switch is not recommended. In this subgroup, an atrial level switch (Senning or Mustard procedure) has lower surgical and short-term morbidity and mortality.

Transposition of the great arteries with ventricular septal defect

The preferred operation is an arterial switch procedure with ventricular septal defect closure. If the ventricular septal defect is large and nonrestrictive and coronary artery anatomy makes an arterial switch operation inadvisable, a Rastelli-type intracardiac repair may be feasible. With the Rastelli-type procedure, waiting until the infant is older and larger may be preferred because of the need for a right ventricle–pulmonary artery conduit in the Rastelli operation. If the infant has excessive congestive heart failure (with growth failure), it may be advisable to either proceed with reparative surgery or, if not feasible, band/ligate the main pulmonary artery and place an aortopulmonary shunt during the newborn period to restrict pulmonary blood flow.

Transposition of the great arteries with ventricular septal defect and left ventricular outflow tract obstruction

An arterial switch operation may not be feasible due to pulmonary (left ventricular outflow tract) stenosis or atresia. If the ventricular septal defect is nonrestrictive and not too remote from the aorta, a Rastelli intracardiac repair could be possible. Because the Rastelli procedure necessitates a conduit from the right ventricle to the pulmonary artery, delaying repair until the infant is older and larger may be preferable. In this case, placing an aortopulmonary shunt during the newborn period may be necessary to establish adequate pulmonary blood flow while waiting.

Transposition of the great arteries with ventricular septal defect and pulmonary vascular obstructive disease

These patients might not be appropriate surgical candidates because of the progressive increase in pulmonary vascular resistance. This is a small subgroup of patients whose conditions are not often diagnosed until after a palliative or reparative procedure is performed.

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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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