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Transposition of the Great Arteries Workup

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

Laboratory Studies

A hyperoxia test (for cyanotic congenital heart disease) may be indicated in patients with transposition of the great arteries (TGA).

In a patient with arterial hypoxemia, an ABG measurement is obtained on 100% oxygen for 10 minutes.

Pulmonary disease (not cyanotic congenital heart disease) is suspected if the partial pressure of oxygen increases to more than 150 mm Hg with oxygen.

Note the following:

  • Admit patients with transposition of the great arteries (TGA) for preoperative testing and surgical interventions.
  • Carefully monitor medication doses and side effects.
  • Monitor adequacy of repair and palliation with periodic physical examinations and possibly echocardiograms.
  • Periodic electrocardiograms and/or 24-hour Holter monitoring to monitor for atrial arrhythmias should be employed, particularly following atrial-level switch operation (ie, Senning or Mustard procedure).
  • Controversy surrounds whether patients should undergo cardiac catheterization every few years following arterial switch operation because of the concern for long-term patency and normal function of the coronary arteries following surgical translocation. No specific recommendations for routine cardiac catheterization will be possible until more information is available, and treatment of coronary artery stenosis is still a matter of debate.
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Procedures

Cardiac catheterization

Diagnostic cardiac catheterization is usually reserved for the subgroup of patients for whom echocardiography does not adequately delineate the anatomy. Suspected coronary artery abnormalities and additional ventricular septal defects may be confirmed or better delineated by cardiac catheterization with angiography. In addition, cardiac catheterization may be necessary to improve left-to-right shunting.

Postcatheterization precautions include hemorrhage, vascular disruption after balloon dilation, pain, nausea and vomiting, and arterial or venous obstruction from thrombosis or spasm.

Complications may include rupture of blood vessel, tachyarrhythmias, bradyarrhythmias, and vascular occlusion.

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Chest Radiography

The chest radiograph may appear normal in newborns with transposition of the great arteries and intact ventricular septum but may demonstrate the classic "egg on a string" appearance in approximately one third of patients.

With a ventricular septal defect, cardiomegaly usually occurs with increased pulmonary arterial vascular markings.

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Echocardiography

Echocardiographic images should be diagnostic of transposition of the great arteries by demonstrating the bifurcating pulmonary artery arising posteriorly from the left ventricle in the parasternal long-axis view.

The parasternal short-axis view shows the relationship of the great arteries to one another. The aorta is usually anterior and rightward of the pulmonary artery in cross-section.

Most associated anatomic lesions, including atrial septal defects, ventricular septal defects, and patent ductus arteriosus, are also diagnosed readily by echocardiography.

The coronary artery anatomy needs to be ascertained and may be abnormal in nearly one third of patients. The coronary artery origins and branching pattern are often delineated by echocardiography.

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Magnetic Resonance Imaging

Late gadolinium enhancement (LGE) cardiovascular magnetic resonance imaging (MRI) studies may have the potential to help clinicians stratify risk in patients with transposition of the great arteries following atrial switch procedure.[7] In a single-center prospective study of 55 patients with transposition of the great arteries who underwent LGE MRI, investigators found that the presence of systemic right ventricular fibrosis was strongly associated with adverse clinical outcomes, particularly new-onset sustained tachyarrhythmias.[7]

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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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