Congenitally Corrected Transposition 

  • Author: Arnold S Baas, MD, FACC, FACP; Chief Editor: Park W Willis IV, MD   more...
 
Updated: Jul 2, 2010
 

Background

Congenitally corrected transposition of the great vessels is a rare congenital heart defect associated with multiple cardiac morphological abnormalities and conduction defects.

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Pathophysiology

During embryological development, left-handed looping of the heart tube results in atrioventricular (AV) discordance, and the aortopulmonary septum fails to rotate 180°, resulting in ventriculoarterial discordance. Blood flows in an effective sequence, hence the name corrected; however, the right ventricle supports the systemic circulation in this disorder.

Venous blood returns from the body into the right atrium before passing through the mitral valve into a morphological left ventricle. Blood then enters the lungs via the pulmonic valve into the main pulmonary artery. Pulmonary venous blood returns to the left atrium and then passes through the tricuspid valve to the morphological right ventricle, exiting to the aorta via the aortic valve. The aorta is positioned anterior and to the left of the pulmonary artery. In effect, the ventricles are transposed.

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Epidemiology

Frequency

United States

Data from the Baltimore-Washington Infant Study supported the fact that congenitally corrected transposition is a rare disorder.[1] As many as 40 infants per 100,000 live births are affected by congenitally corrected transposition of the great vessels; this is fewer than 1% of all congenital heart defects.

International

This disorder is reported in 0.5% of patients with congenital heart disease, and the literature reports fewer than 1000 cases. Most pediatric cardiologists have seen multiple cases of congenitally corrected transposition of the great vessels; however, the true prevalence of the malformation is not known.

Mortality/Morbidity

  • Ten-year survival rate ranges from 64-83% from the time of diagnosis and is dependent on associated anomalies.
  • Freedom[2] reported an operative mortality rate of 6% and a 15-year actuarial survival rate of 48% in a cohort of patients with congenitally corrected transposition of the great vessels at the Hospital for Sick Children in Toronto.
  • A rare patient without associated cardiac anomalies may have a much more benign course, and literature documents many examples of these patients being diagnosed in the sixth and seventh decades of life.[3, 4, 5]
  • A median age at death of 40 years has been reported in both patients who have undergone operation and those who have not.
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Contributor Information and Disclosures
Author

Arnold S Baas, MD, FACC, FACP  Assistant Professor of Medicine, Division of Cardiology, University of California, Los Angeles School of Medicine; Attending Physician, UCLA Santa Monica Hospital and UCLA Westwood Hospital

Arnold S Baas, MD, FACC, FACP is a member of the following medical societies: American College of Cardiology, American College of Physicians, American Federation for Medical Research, and American Society of Echocardiography

Disclosure: Nothing to disclose.

Specialty Editor Board

Park W Willis IV, MD  Sarah Graham Distinguished Professor of Medicine and Pediatrics, University of North Carolina at Chapel Hill School of Medicine

Park W Willis IV, MD is a member of the following medical societies: American Society of Echocardiography

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Senior Pharmacy Editor, eMedicine

Disclosure: eMedicine Salary Employment

Marschall S Runge, MD, PhD  Charles and Anne Sanders Distinguished Professor of Medicine, Chairman, Department of Medicine, Vice Dean for Clinical Affairs, University of North Carolina at Chapel Hill School of Medicine

Marschall S Runge, MD, PhD is a member of the following medical societies: American Association for the Advancement of Science, American College of Cardiology, American College of Physicians-American Society of Internal Medicine, American Federation for Clinical Research, American Federation for Medical Research, American Heart Association, American Physiological Society, American Society for Clinical Investigation, American Society for Investigative Pathology, Association of American Physicians, Association of Professors of Cardiology, Association of Professors of Medicine, Southern Society for Clinical Investigation, and Texas Medical Association

Disclosure: Pfizer Honoraria Speaking and teaching; Merck Honoraria Speaking and teaching; Orthoclinica Diagnostica Consulting fee Consulting

Amer Suleman, MD  Consultant in Electrophysiology and Cardiovascular Medicine, Department of Internal Medicine, Division of Cardiology, Medical City Dallas Hospital

Amer Suleman, MD is a member of the following medical societies: American College of Physicians, American Heart Association, American Institute of Stress, American Society of Hypertension, Federation of American Societies for Experimental Biology, Royal Society of Medicine, and Society of Cardiac Angiography and Interventions

Disclosure: Nothing to disclose.

Chief Editor

Park W Willis IV, MD  Sarah Graham Distinguished Professor of Medicine and Pediatrics, University of North Carolina at Chapel Hill School of Medicine

Park W Willis IV, MD is a member of the following medical societies: American Society of Echocardiography

Disclosure: Nothing to disclose.

References

  1. Ferencz, Loffredo, Correa-Villasenor. Genetic and environmental risk factors of major cardiovascular malformations: The Baltimore-Washington Infant Study, 1981-1989. Perspectives in Pediatric Cardiology. 1989:Volume 5.

  2. Freedom RM. Congenitally corrected transposition of the great arteries: definitions and pathologic anatomy. Prog Ped Cardiol. 1999;10:3-16.

  3. Sloth AD, Jensen JK, Steffensen FH, Norgaard BL. [Congenital transposition of the great arteries newly diagnosed in a 76-year-old woman]. Ugeskr Laeger. Jan 26 2009;171(5):319-21. [Medline].

  4. Kwon SH, Oh JH, Hwang SJ, Kim SJ, Kim WS, Kim MG, et al. Congenitally corrected transposition of the great arteries in a 59-year-old man: ECG-gated 64-slice MDCT findings. Int J Cardiol. Jan 23 2009;[Medline].

  5. Kozelj M, Prokselj K, Berden P, Jan M, Osredkar J, Bunc M, et al. The syndrome of cardiac failure in adults with congenitally corrected transposition. Cardiol Young. Dec 2008;18(6):599-607. [Medline].

  6. Dabizzi RP, Barletta GA, Caprioli G, et al. Coronary artery anatomy in corrected transposition of the great arteries. J Am Coll Cardiol. Aug 1988;12(2):486-91. [Medline].

  7. Goldmuntz E, Clark BJ, Mitchell LE, et al. Frequency of 22q11 deletions in patients with conotruncal defects. J Am Coll Cardiol. Aug 1998;32(2):492-8. [Medline].

  8. Anderson RH, Becker AE, Arnold R, Wilkinson JL. The conducting tissues in congenitally corrected transposition. Circulation. Nov 1974;50(5):911-23. [Medline].

  9. Anderson RH, Arnold R, Wilkinson JL. The conducting system in congenitally corrected transposition. Lancet. Jun 9 1973;1(7815):1286-8. [Medline].

  10. Connelly MS, Liu PP, Williams WG, et al. Congenitally corrected transposition of the great arteries in the adult: functional status and complications. J Am Coll Cardiol. Apr 1996;27(5):1238-43. [Medline].

  11. Termignon JL, Leca F, Vouhe PR, et al. "Classic" repair of congenitally corrected transposition and ventricular septal defect. Ann Thorac Surg. Jul 1996;62(1):199-206. [Medline].

  12. Ly M, Belli E, Leobon B, Kortas C, Grollmüss OE, Piot D, et al. Results of the double switch operation for congenitally corrected transposition of the great arteries. Eur J Cardiothorac Surg. May 2009;35(5):879-83; discussion 883-4. [Medline].

  13. Warnes CA. Transposition of the Great Arteries. Circulation. 2006;114:2699-2709.

  14. Cheitlin MD, Armstrong WF, Aurigemma GP, Beller GA, Bierman FZ, Davis JL, et al. ACC/AHA/ASE 2003 guideline update for the clinical application of echocardiography--summary article: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (ACC/AHA/ASE Committee to Update the 1997 Guidelines for the Clinical Application of Echocardiography). J Am Coll Cardiol. Sep 3 2003;42(5):954-70. [Medline]. [Full Text].

  15. Hörer J, Schreiber C, Krane S, Prodan Z, Cleuziou J, Vogt M, et al. Outcome after surgical repair/palliation of congenitally corrected transposition of the great arteries. Thorac Cardiovasc Surg. Oct 2008;56(7):391-7. [Medline].

  16. Graham TP Jr, Bernard YD, Mellen BG, et al. Long-term outcome in congenitally corrected transposition of the great arteries: a multi-institutional study. J Am Coll Cardiol. 2000;36:255-261.

  17. Arendt KW, Connolly HM, Warnes CA, Watson WJ, Hebl JR, Craigo PA. Anesthetic management of parturients with congenitally corrected transposition of the great arteries: three cases and a review of the literature. Anesth Analg. Dec 2008;107(6):1973-7. [Medline].

  18. Therrien J, Barnes I, Somerville J. Outcome of pregnancy in patients with congenitally corrected transposition of the great arteries. Am J Cardiol. Oct 1 1999;84(7):820-4. [Medline].

  19. Genoni M, Jenni R, Hoerstrup SP, Vogt P, Turina M. Pregnancy after atrial repair for transposition of the great arteries. Heart. Mar 1999;81(3):276-7. [Medline]. [Full Text].

  20. Connolly HM, Grogan M, Warnes CA. Pregnancy among women with congenitally corrected transposition of the great arteries. J Am Coll Cardiol. 1999;33:1692-1695.

  21. Bove EL. Congenitally corrected transposition of the great arteries: surgical options for biventricular repair. Prog Ped Cardiol. 1999;10:45-49.

  22. Hui L, Chau AK, Leung MP, Chiu CS, Cheung YF. Assessment of left ventricular function long term after arterial switch operation for transposition of the great arteries by dobutamine stress echocardiography. Heart. Jan 2005;91(1):68-72. [Medline]. [Full Text].

  23. McKay R, Anderson RH, Smith A. The coronary arteries in hearts with discordant atrioventricular connections. J Thorac Cardiovasc Surg. May 1996;111(5):988-97. [Medline].

  24. Okamura K, Konno S. Two types of ventricular septal defect in corrected transposition of the great arteries: reference to surgical approaches. Am Heart J. Apr 1973;85(4):483-90. [Medline].

  25. Webb CL. Congenitally corrected transposition of the great arteries: clinical features, diagnosis, and prognosis. Prog Ped Cardiol. 1999;10:17-30.

 
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Subcostal view of a 1-year-old child with L-transposition of the great arteries, valvular and subvalvular pulmonic stenosis, and a moderate outlet ventriculoseptal defect (VSD). Note the ventriculoarterial discordance. Note the posterior, rightward position of the pulmonary artery. [PA = pulmonary artery, LV = left ventricle, RV = right ventricle].
Apical image revealing atrioventricular discordance. Note the pulmonary venous return into the left atrium, with sequential flow through the tricuspid valve to the right ventricle. The right ventricle is systemic. [LA = left atrium, RA = right atrium, LV = left ventricle, RV = right ventricle].
Post-Rastelli repair with left ventricle to aortic baffle through a ventriculoseptal defect (VSD) complicated by subaortic stenosis.
This image demonstrates a calcified pulmonary homograft anterior and adjacent to the chest wall (right ventricle to pulmonary artery bifurcation) with significant homograft stenosis and prior pulmonary valvular endocarditis (same patient as in Image 3).
 
 
 
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