Congenitally Corrected Transposition Workup

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

Laboratory Studies

Cyanotic conditions may be associated with elevations in red cell volume reflected in the hemoglobin and hematocrit. This elevation is a reactive process to the body's demand for oxygen and is not a primary polycythemia. CBC count, clotting profile, renal function, and ferritin and uric acid levels should be measured.

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Imaging Studies

  • Echocardiography either in utero or by transthoracic or transesophageal imaging generally confirms the diagnosis (see following images). Subcostal view of a 1-year-old child with L-transpSubcostal 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 discordancApical 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].
  • Chest radiography reveals parallel great vessels. The upper left heart border is formed by the aorta and appears straight, and the pulmonary artery knob is absent because of the rightward, posterior displacement of the artery.
  • Transesophageal echocardiography (TEE) may be needed to assess ventricular function, AV valve regurgitation, and pulmonary outflow tract if this information is not provided by transthoracic imaging, particularly in the patient who has undergone an operation.
  • Nuclear cardiology assessment of ventricular function may be indicated. Radionuclide angiography usually better reports right ventricular function compared with echocardiography.
  • Cardiac MRI evaluates ventricular volumes, ventricular function, and valvular or conduit function.
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Other Tests

  • Electrocardiography is affected by the associated cardiac anomalies but commonly shows AV block, atrial arrhythmias, and abnormal initial ventricular activation due to disordered anatomy of the conduction system. With ventricular inversion, the ventricular bundle branches are inverted and the initial activation is oriented from right-to-left. This results in reversal of the normal Q-wave pattern in the precordial leads such that Q waves are present in the right precordial leads but absent in the left precordial leads. The ECG in patients with congenitally corrected transposition may therefore be misinterpreted as inferior myocardial infarction.
  • A Holter monitor is used for assessment of AV block and atrial arrhythmia.
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Procedures

Cardiac catheterization carries a significant risk of inducing transient or complete heart block.

  • Use caution. Transient or permanent complete heart block may be induced in this condition because the conduction system lies just below the pulmonic valve.
  • Always use a balloon-tipped catheter to approach the pulmonic valve. Always have transvenous pacing capability available during this procedure.
  • Limit catheterization to assessment of pulmonic stenosis, shunt volume, pulmonary vascular resistance before and in response to therapy, and angiography in preparation for reparative surgery.
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Histologic Findings

Anderson et al[8, 9] identified both a normal-appearing AV node in the usual position without connections to a penetrating bundle and an accessory AV node located in the right atrium at the junction of the mitral valve and the left border of the right atrial appendage. This second AV node connects directly to an aberrantly located penetrating bundle. The bundle passes laterally onto the pulmonary outflow tract just below the pulmonic valve, then descends to the interventricular septum, remaining on the right side of the septum rather than the left side (ie, normal system). The course varies depending on the integrity of the ventricular septum.

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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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