Congenitally Corrected Transposition Follow-up

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

Deterrence/Prevention

Serial echocardiograms to monitor right ventricular (ie, systemic ventricular) size and function, and tricuspid (systemic AV) valve regurgitation can help to time operative repair and assess effects of medical intervention. Data are emerging using right ventricular radionuclide angiography and magnetic resonance angiography for both perfusion and function assessments. Multiple gated acquisition (MUGA) scans can also accurately describe right ventricular function and dimension.

  • Guidelines for the Clinical Application of Echocardiography by an ACC/AHA Task Force suggest that class I indications for follow-up echocardiograms in patients with known congenital heart disease include any change in clinical findings, any uncertainty of the original diagnosis or of the structural abnormalities or hemodynamics, or periodic monitoring for those whose ventricular function and AV valve regurgitation must be followed.[14]
  • The timing of periodic monitoring is not specified. Most centers monitor patients with serial echocardiography; more frequent examinations are warranted for any change in clinical status.
  • Dobutamine stress echocardiography may also be helpful. In asymptomatic children after arterial switch surgery, baseline left ventricular function is often mildly impaired with reversible areas of ischemia revealed, despite normal coronary perfusion.
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Complications

  • Major postoperative residual complications include contractile dysfunction of the systemic right ventricle, progressive tricuspid (systemic AV) regurgitation, complete heart block, atrial or ventricular arrhythmias, and infective endocarditis. Patients may develop conduit or homograph dysfunction postoperatively.
  • Systemic AV valve regurgitation is well described after surgery even when the valve has not been directly manipulated.
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Prognosis

  • Prognosis depends on AV conduction, arrhythmias, structural abnormalities, and degree of hemodynamic disturbance.[15]
  • Sudden death may be related to the onset of complete heart block or atrial or ventricular arrhythmias.
  • Right ventricular failure can develop over time. This may be related to coronary perfusion mismatch as the right ventricle is supplied by a single coronary artery. In addition, differences in right and left ventricular fiber orientation, geometry, and microscopic structural features may play a role in early failure of the right ventricle when functioning as the systemic ventricle. Poor prognostic indicators include cyanosis, polycythemia, pulmonary vascular obstructive disease, tricuspid regurgitation, younger age at surgery, larger preoperative shunt size, and lower right ventricular ejection fraction. A multicenter series of 182 patients with congenitally corrected transposition of the great arteries demonstrated that 25% of patients without associated cardiac lesions and 67% of patients with other cardiac abnormalities developed congestive heart failure by age 45.[16]
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Patient Education

  • Pregnancy counseling[17]
  • Infective endocarditis prophylaxis
  • Moderate and not heavy exercise routines
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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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