eMedicine Specialties > Pediatrics: Cardiac Disease and Critical Care Medicine > Cardiology

Transposition of the Great Arteries: Differential Diagnoses & Workup

Author: John R Charpie, MD, PhD, Associate Professor, Department of Pediatrics, University of Michigan Medical Center
Coauthor(s): Kevin O Maher, MD, Assistant Professor of Pediatrics, Emory University School of Medicine; Consulting Staff, Department of Pediatrics, Pediatric Cardiovascular Intensive Care Unit, Sibley Heart Center
Contributor Information and Disclosures

Updated: Jun 11, 2009

Differential Diagnoses

Pulmonary Atresia With Intact Ventricular Septum
Tetralogy of Fallot With Absent Pulmonary Valve
Tetralogy of Fallot With Pulmonary Atresia
Total Anomalous Pulmonary Venous Connection
Tricuspid Atresia
Truncus Arteriosus

Other Problems to Be Considered

Double-outlet right ventricle with malposed great arteries

Workup

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.

Imaging Studies

  • 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.
  • 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.

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.

More on Transposition of the Great Arteries

Overview: Transposition of the Great Arteries
Differential Diagnoses & Workup: Transposition of the Great Arteries
Treatment & Medication: Transposition of the Great Arteries
Follow-up: Transposition of the Great Arteries
Multimedia: Transposition of the Great Arteries
References
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References

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

  2. 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. Nov 2003;126(5):1397-403. [Medline].

  3. [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. Jun 2007;138(6):739-45, 747-60. [Medline]. [Full Text].

  4. Aseervatham R, Pohlner P. A clinical comparison of arterial and atrial repairs for transposition of the great arteries: early and midterm survival and functional results. Aust N Z J Surg. Mar 1998;68(3):206-8. [Medline].

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

  6. 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. Nov 2005;80(5):1641-6. [Medline].

  7. Kirjavainen M, Happonen JM, Louhimo I. Late results of Senning operation. J Thorac Cardiovasc Surg. Mar 1999;117(3):488-95. [Medline].

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

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

  10. 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. Jun 21 2005;45(12):2061-8. [Medline].

  11. Planche C, Lacour-Gayet F, Serraf A. Arterial switch. Pediatr Cardiol. Jul-Aug 1998;19(4):297-307. [Medline].

  12. Puley G, Siu S, Connelly M, et al. Arrhythmia and survival in patients >18 years of age after the mustard procedure for complete transposition of the great arteries. Am J Cardiol. Apr 1 1999;83(7):1080-4. [Medline].

  13. Soongswang J, Adatia I, Newman C, et al. Mortality in potential arterial switch candidates with transposition of the great arteries. J Am Coll Cardiol. Sep 1998;32(3):753-7. [Medline].

  14. 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. Aug 17 2005;[Medline].

  15. Wren C, Birrell G, Hawthorne G. Cardiovascular malformations in infants of diabetic mothers. Heart. Oct 2003;89(10):1217-20. [Medline].

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Further Reading

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Keywords

transposition of the great arteries, TGA, complete transposition of the great arteries, d-TGA, simple transposition, ventriculoarterial discordance, heart lesion in neonate, cyanotic congenital heart lesion, intact ventricular septum, ventricular septal defect, left ventricular outflow tract obstruction, pulmonary vascular obstructive disease, atrial septal defect, patent ductus arteriosus, thrombocytopenia, congestive heart failure, cyanosis, tachypnea, tachycardia, diaphoresis, tetralogy of Fallot, hepatomegaly, dextro-transposition of the great arteries, levo-transposition of the great arteries, L-TGA, pulmonary vascular obstructive disease, treatment, diagnosis

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Contributor Information and Disclosures

Author

John R Charpie, MD, PhD, Associate Professor, 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, and Society for Pediatric Research
Disclosure: Nothing to disclose.

Coauthor(s)

Kevin O Maher, MD, Assistant Professor of Pediatrics, Emory University School of Medicine; Consulting Staff, Department of Pediatrics, Pediatric Cardiovascular Intensive Care Unit, Sibley Heart Center
Kevin O Maher, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, and American Medical Association
Disclosure: Nothing to disclose.

Medical Editor

Charles I Berul, MD, Associate Professor of Pediatrics, Harvard Medical School; Senior Associate, Department of Cardiology, Children's Hospital of Boston
Charles I Berul, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Heart Association, and Society for Pediatric Research
Disclosure: Nothing to disclose.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from financial planner; Avanir Pharma Stock Investment from financial planner ; WebMD Salary and stock Employment and investment from financial planner

Managing Editor

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.

CME Editor

Gilbert Z Herzberg, MD, Assistant Professor, Department of Pediatrics, Section of Pediatric Cardiology, New York Medical College; Consulting Staff, Department of Pediatrics, Sound Shore Medical Center
Gilbert Z Herzberg, MD is a member of the following medical societies: American Academy of Pediatrics
Disclosure: Nothing to disclose.

Chief Editor

Stuart Berger, MD, Professor of Pediatrics, Division of Cardiology, Medical College of Wisconsin; Chief of Pediatric Cardiology, Medical Director of Pediatric Heart Transplant Program, Medical Director of The Heart Center, Children's Hospital of Wisconsin
Stuart Berger, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American College of Chest Physicians, American Heart Association, and Society for Cardiac Angiography and Interventions
Disclosure: Nothing to disclose.

 
 
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