Pediatric Cardiology

, Volume 34, Issue 1, pp 70–74 | Cite as

Abnormal Mitral Valve Anatomy in d-Transposition of the Great Arteries: Anatomic Characterization and Surgical Outcomes

  • Joseph A. Camarda
  • Susan E. Harris
  • John Hambrook
  • Michele A. FrommeltEmail author
  • James S. Tweddell
  • Peter C. Frommelt
Original Article


Mitral valve anomalies can occur with S,D,D-transposition of the great arteries (d-TGA). Their influence on surgical technique and outcome after an arterial switch operation (ASO) has not been well described. Patients with d-TGA who underwent ASO from February 1990 to January 2011 were identified. Echocardiograms, operative reports, hospital course, and latest follow-up evaluation were reviewed. A total of 218 infants underwent ASO at a median age of 15.8 days. Survival was 95 % during a mean follow-up period of 60 months. Nine patients (4 %) were found to have similar mitral valve anomalies including anterior malalignment conoventricular septal defect, anterior displacement of the mitral valve toward the left ventricular outflow tract (LVOT), malpositioning of the posteromedial papillary muscle, unusual rotation of the mitral valve leaflets orienting the commissure toward the anterior ventricular septum, and redundant mitral valve tissue extending into the LVOT. Coarctation was more frequent in this subgroup (33 vs. 10 %; p = 0.05). Preoperative echocardiography consistently indicated suspicion of a cleft mitral valve with chordal attachments to the ventricular septum causing potential LVOT obstruction. Operative inspection did not identify a cleft or anomalous attachments in any patient, and no valvuloplasty or chordal manipulation was performed. The average hospital length of stay were similar (30.7 vs. 25.3 days; p = 0.54). One patient died late due to progressive LVOT obstruction, and one required heart transplantation. No patient had significant mitral valve regurgitation. We conclude that mitral valve anomalies associated with d-TGA are rare but present with consistent anatomic features and higher risk of coarctation. Unusual mitral valve apparatus positioning and chordal redundancy can suggest the need for valvuloplasty and chordal resection preoperatively, but this is rarely needed.


Arterial switch Echocardiography Mitral valve Transposition 


  1. 1.
    Brown J, Park H, Turrentine M (2001) Arterial switch operation: factors impacting survival in the current era. Ann Thorac Surg 71:1978–1984PubMedCrossRefGoogle Scholar
  2. 2.
    Daebritz S, Nollert G, Sachweh J, Engelhardt W, von Bernuth G, Messmer B (2000) Anatomical risk factors for mortality and cardiac morbidity after arterial switch operation. Ann Thorac Surg 69:1880–1886PubMedCrossRefGoogle Scholar
  3. 3.
    Emani SM, Beroukhim R, Zurakowski D, Pigula FA, Mayer JE, del Nido PJ, Geva T, Bacha EA (2009) Outcomes after anatomic repair for d-transposition of the great arteries with left ventricular outflow tract obstruction. Circulation 120:S53–S58PubMedCrossRefGoogle Scholar
  4. 4.
    Gorler H, Ono M, this A, Lunkewitz E, Westhoff-Bleck M, Haverich A, Breymann T, Boethig D (2011) Long-term morbidity and quality of life after surgical repair of transposition of the great arteries: atrial versus arterial switch operation. Interact Cardiovasc Thorac Surg 12:569–574. doi: 10.1510/icvts.2010.253898 PubMedCrossRefGoogle Scholar
  5. 5.
    Gottlieb D, Schwartz ML, Bischoff K, Gauvreau K, Mayer JE (2008) Predictors of outcome of arterial switch operation for complex d-transposition. Ann Thorac Surg 85:1698–1703PubMedCrossRefGoogle Scholar
  6. 6.
    Harris PA, Taylor R, Thielke R, Payne J, Gonzalez N, Conde JG (2009) Research electronic data capture (REDCap): a metadata-driven methodology and workflow process for providing translational research information support. J Biomed Informatics 42:377–381CrossRefGoogle Scholar
  7. 7.
    Hazekamp M, Portela F, Bartelings M (2007) The optimal procedure for the great arteries and left ventricular outflow tract obstruction: an anatomical study. Eur J Cardiothorac Surg 31:879–887PubMedCrossRefGoogle Scholar
  8. 8.
    Lee JR, Lim HG, Kim YJ, Rho JR, Bae EJ, Noh CI, Yun YS, Ahn C (2004) Repair of transposition of the great arteries, ventricular septal defect and left ventricular outflow tract obstruction. Eur J Cardiothorac Surg 25:735–741PubMedCrossRefGoogle Scholar
  9. 9.
    Leobon B, Belli E, Ly M, Kortas C, Le Bret E, Sigal-Cinqualbre A, Roussin R, Serraf A (2008) Left ventricular outflow tract obstruction after arterial switch operation. Eur J Cardiothorac Surg 34:1046–1050PubMedCrossRefGoogle Scholar
  10. 10.
    Losay J, Touchot A, Serraf A, Litvinova A, Lambert V, Piot J, Lacour-Gayet F, Capderou A, Planche C (2001) Late outcomes after arterial switch operation for transposition of the great arteries. Circulation 104:I121–I126PubMedCrossRefGoogle Scholar
  11. 11.
    Martin EC, LaCorte MA, Steeg CN, Bowman FO (1981) Accessory mitral valve tissue causing left ventricular outflow tract obstruction in d-transposition of the great arteries. Cardiovasc Intervent Radiol 4:124–127PubMedCrossRefGoogle Scholar
  12. 12.
    Menahem S, Anderson RH (2004) Cleft mitral valve in transposition with intact ventricular septum. Cardiol Young 14:550–552PubMedCrossRefGoogle Scholar
  13. 13.
    Moene RJ, Oppenheimer-Dekker A (1982) Congenital mitral valve anomalies in transposition of the great arteries. Am J Cardiol 49:1972–1978PubMedCrossRefGoogle Scholar
  14. 14.
    Rosenquist GC, Stark J, Taylor JF (1975) Congenital mitral valve disease in transposition of the great arteries. Circulation 51:731–737PubMedCrossRefGoogle Scholar
  15. 15.
    Sohn YS, Brizard CPR, Cochrane AD, Wilkinson JL, Mas C, Karl TR (1998) Arterial switch in hearts with left ventricular outflow and pulmonary valve abnormalities. Ann Thorac Surg 66:842–848PubMedCrossRefGoogle Scholar
  16. 16.
    Tobler D, Williams WG, Jegatheeswaran A, Van Arsdell G, McCrindle BW, Greutmann M, Oechslin EN, Silversides CK (2010) Cardiac outcomes in young adult survivors of the arterial switch operation for transposition of the great arteries. J Am Coll Cardiol 56:58–64PubMedCrossRefGoogle Scholar
  17. 17.
    Vargo P, Mavroudis C, Stewart R, Backer C (2010) Late complications following the arterial switch operation. World J Pediatr Congenit Heart Surg 2:37–42. doi: 10.1177/2150135110386976 CrossRefGoogle Scholar
  18. 18.
    Wernovsky G (2008) Transposition of the great arteries. In: Allen HD, Driscoll DJ, Shaddy RE, Feltes TF (eds) Moss and Adams’ heart disease in infants, children, and adolescents including the fetus and young adult, 7th edn. Lippincott Williams and Wilkins, Philadelphia, pp 1038–1086Google Scholar
  19. 19.
    Wernovsky G, Jonas R, Colan S, Sanders S, Wessel D, Castaneda A, Mayer J (1990) Results of the arterial switch operation in patients with transposition of the great arteries and abnormalities of the mitral valve or left ventricular outflow tract. J Am Coll Cardiol 16:1446–1454PubMedCrossRefGoogle Scholar
  20. 20.
    Yoshimura N, Yamaguchi M, Oshima Y, Oka S, Ootaki Y, Tei T, Ogawa K (2000) Clinical and pathological features of accessory valve tissue. Ann Thorac Surg 69:1205–1208PubMedCrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media, LLC 2012

Authors and Affiliations

  • Joseph A. Camarda
    • 1
  • Susan E. Harris
    • 1
  • John Hambrook
    • 1
  • Michele A. Frommelt
    • 1
    Email author
  • James S. Tweddell
    • 2
  • Peter C. Frommelt
    • 1
  1. 1.Department of Pediatrics, The Children’s Hospital of WisconsinMedical College of WisconsinMilwaukeeUSA
  2. 2.Department of Cardiothoracic Surgery, The Children’s Hospital of WisconsinMedical College of WisconsinMilwaukeeUSA

Personalised recommendations