Transposition of the Great Arteries Follow-up

  • Author: John R Charpie, MD, PhD; Chief Editor: Stuart Berger, MD   more...
 
Updated: Aug 10, 2011
 

Further Inpatient Care

  • 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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Further Outpatient Care

  • Evidence from the Boston Circulatory Arrest Trial suggests that neurodevelopmental outcomes for children with dextro-transposition of the great arteries (d-TGA) who undergo arterial switch operation (and other complex neonatal operations) may not be normal and may require further investigation and follow-up.[2]
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Inpatient & Outpatient Medications

  • Many patients do not require any specific medications. Possible discharge medications might include digoxin, furosemide, or both.
  • All patients require preoperative and postoperative antibiotic prophylaxis for dental procedures that involve manipulation of gingival tissue or the periapical region of teeth or perforation of the oral mucosa. Antibiotic prophylaxis is also recommended for invasive respiratory tract procedures that involve incision or biopsy of the respiratory mucosa (eg, tonsillectomy, adenoidectomy). Antibiotic prophylaxis is not recommended for bronchoscopy unless the procedure involves incision of the respiratory tract mucosa. For invasive respiratory tract procedures to treat an established infection (eg, drainage of abscess, empyema), administer an antibiotic active against Streptococcus viridans. See the endocarditis prophylaxis guidelines that were revised by the American Heart Association (AHA) in the Medication section.[3]
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Transfer

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

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

  • Prognosis depends on the specific anatomic substrate and type of surgical therapy used (arterial switch operation, atrial switch operation, or Rastelli procedure).
  • The overall survival rate following arterial switch operation is 90%.
  • 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.
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Patient Education

  • Family members should learn cardiopulmonary resuscitation (CPR).
  • Educate family members about congenital heart disease.
  • Obtain genetics counseling for future pregnancy, despite the relatively low risk of recurrence.
  • For excellent patient education resources, visit eMedicine's Heart Center. Also, see eMedicine's patient education article Tetralogy of Fallot.
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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, and Society for Pediatric Research

Disclosure: Nothing to disclose.

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, and American Heart Association

Disclosure: Nothing to disclose.

Specialty Editor Board

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, American College of Cardiology, American Heart Association, Cardiac Electrophysiology Society, Heart Rhythm Society, Pediatric and Congenital Electrophysiology Society, and Society for Pediatric Research

Disclosure: Johnson & Johnson Consulting fee Consulting

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.

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.

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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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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