Transposition of the Great Arteries Medication

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

Medication Summary

Transposition of the great arteries (TGA) has no specific or recommended drug therapies. Newborn infants with transposition of the great arteries (particularly those with severe left ventricular outflow tract obstruction) may derive some initial benefit from alprostadil (ie, prostaglandin E1) therapy. Patients with transposition of the great arteries and ventricular septal defect who have not undergone surgical repair, and some patients following complete repair, might potentially benefit from digoxin and diuretic therapy to improve systemic ventricular function and avoid fluid retention. All patients require antibiotic prophylaxis prior to dental and indicated surgical procedures in order to reduce the risk of subacute bacterial endocarditis. For more information, see Antibiotic Prophylactic Regimens for Endocarditis.

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Inotropic agents

Class Summary

These drugs increase the contractility of cardiac muscle in a dose-dependent manner (ie, positive inotropic effect).

Digoxin (Lanoxin)

 

Frequently used cardiac glycoside that inhibits the sarcolemmal sodium-potassium adenosine triphosphatase, which leads to an increase in intracellular calcium concentration and increased myocardial contractility.

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Loop diuretics

Class Summary

These drugs inhibit electrolyte reabsorption in the thick ascending limb of the loop of Henle, thus promoting diuresis.

Furosemide (Lasix)

 

This is a commonly used loop diuretic with moderate diuretic potency. Increases excretion of water by interfering with chloride-binding co-transport system which in turn inhibits sodium and chloride reabsorption in ascending loop of Henle and distal renal tubule.

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Prostaglandins

Class Summary

Temporary maintenance of patency of ductus arteriosus in neonates with ductal-dependent congenital heart disease.

Alprostadil IV (Prostin VR)

 

Identical to the naturally occurring prostaglandin E1 (PGE1) and possesses various pharmacologic effects, including vasodilation and inhibition of platelet aggregation. Temporary maintenance of patency of ductus arteriosus in neonates with ductal-dependent congenital heart disease. Relaxes smooth muscle of the ductus arteriosus. Beneficial in infants with congenital defects that restrict pulmonary or systemic blood flow and who in order to get adequate oxygenation and lower body perfusion, depend on a patent ductus arteriosus.

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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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