Skip to main content
SpringerLink
Log in
Book cover

Transesophageal Echocardiography for Congenital Heart Disease pp 307–340Cite as

Evaluation of Conotruncal Abnormalities

  • Chapter
  • First Online:

Abstract

Conotruncal malformations encompass a group of congenital heart defects with abnormal ventriculo-arterial connections. The conotruncal abnormalities are often seen in association with other cardiac defects, and the intracardiac anatomy can be quite complex. Cardiac surgery is almost always required to repair the anatomic abnormalities and restore normal physiology. As such, transesophageal echocardiography (TEE) is often required in the intraoperative setting in order to evaluate and confirm preoperatively the cardiac abnormalities, and to assess the cardiac repair postoperatively. Moreover, patients with a conotruncal malformation (operated and sometimes unoperated) will survive into adulthood, and in the ambulatory setting, TEE can provide important anatomic and physiologic information superior to that available by transthoracic echocardiography. This chapter discusses the TEE evaluation of the most common and important conotruncal defects, including tetralogy of Fallot, double outlet right ventricle, truncus arteriosus, transposition of the great arteries (also known as complete, or D-transposition of the great arteries), and congenitally corrected transposition of the great arteries.

This is a preview of subscription content, log in via an institution.

Chapter
USD   29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD   159.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD   209.00
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Learn about institutional subscriptions

References

  1. Snider AR, Ritter SB, Serwer GA. Abnormalities within the cardiac chambers, proximal vessels, and thorax. In: Echocardiography in pediatric heart disease. 2nd ed. St Louis: Mosby; 1997. p. 497–511.

    Google Scholar 

  2. Anderson RH, Weinberg PM. The clinical anatomy of tetralogy of Fallot. Cardiol Young. 2005;15 Suppl 1:38–47.

    Article  PubMed  Google Scholar 

  3. Michielon G, Marino B, Formigari R, et al. Genetic syndromes and outcome after surgical correction of tetralogy of Fallot. Ann Thorac Surg. 2006;81:968–75.

    Article  PubMed  Google Scholar 

  4. Kohli V, Azad S, Sachdev MS, et al. Balloon dilation of the pulmonary valve in premature infants with tetralogy of Fallot. Pediatr Cardiol. 2008;29:946–9.

    Article  PubMed  Google Scholar 

  5. Massoud I, Imam A, Mabrouk A, et al. Palliative balloon valvoplasty of the pulmonary valve in tetralogy of Fallot. Cardiol Young. 1999;9:24–36.

    CAS  PubMed  Google Scholar 

  6. Sluysmans T, Neven B, Rubay J, et al. Early balloon dilatation of the pulmonary valve in infants with tetralogy of Fallot. Risks and benefits. Circulation. 1995;91:1506–11.

    Article  CAS  PubMed  Google Scholar 

  7. Hirsch JC, Mosca RS, Bove EL. Complete repair of tetralogy of Fallot in the neonate: results in the modern era. Ann Surg. 2000;232:508–14.

    Article  CAS  PubMed  Google Scholar 

  8. Kolcz J, Pizarro C. Neonatal repair of tetralogy of Fallot results in improved pulmonary artery development without increased need for reintervention. Eur J Cardiothorac Surg. 2005;28:394–9.

    Article  PubMed  Google Scholar 

  9. Reddy VM, Liddicoat JR, McElhinney DB, Brook MM, Stanger P, Hanley FL. Routine primary repair of tetralogy of Fallot in neonates and infants less than three months of age. Ann Thorac Surg. 1995;60:S592–6.

    Article  CAS  PubMed  Google Scholar 

  10. Schneider M, Zartner P, Sidiropoulos A, Konertz W, Hausdorf G. Stent implantation of the arterial duct in newborns with duct-dependent circulation. Eur Heart J. 1998;19:1401–9.

    Article  CAS  PubMed  Google Scholar 

  11. Alwi M, Choo KK, Latiff HA, Kandavello G, Samion H, Mulyadi MD. Initial results and medium-term follow-up of stent implantation of patent ductus arteriosus in duct-dependent pulmonary circulation. J Am Coll Cardiol. 2004;44:438–45.

    Article  PubMed  Google Scholar 

  12. Alwi M. Stenting the ductus arteriosus: case selection, technique and possible complications. Ann Pediatr Cardiol. 2008;1:38–45.

    Article  PubMed Central  PubMed  Google Scholar 

  13. Walsh MA, Lee KJ, Chaturvedi R, Van Arsdell GS, Benson LN. Radiofrequency perforation of the right ventricular outflow tract as a palliative strategy for pulmonary atresia with ventricular septal defect. Catheter Cardiovasc Interv. 2007;69:1015–20.

    Article  PubMed  Google Scholar 

  14. Jureidini SB, Appleton RS, Nouri S. Detection of coronary artery abnormalities in tetralogy of Fallot by two-dimensional echocardiography. J Am Coll Cardiol. 1989;14:960–7.

    Article  CAS  PubMed  Google Scholar 

  15. Need LR, Powell AJ, del Nido P, Geva T. Coronary echocardiography in tetralogy of fallot: diagnostic accuracy, resource utilization and surgical implications over 13 years. J Am Coll Cardiol. 2000;36:1371–7.

    Article  CAS  PubMed  Google Scholar 

  16. Suzuki A, Ho SY, Anderson RH, Deanfield JE. Further morphologic studies on tetralogy of Fallot, with particular emphasis on the prevalence and structure of the membranous flap. J Thorac Cardiovasc Surg. 1990;99:528–35.

    CAS  PubMed  Google Scholar 

  17. Postema PG, Rammeloo LAJ, van Litsenburg R, Rothuis EGM, Hruda J. Left superior vena cava in pediatric cardiology associated with extra-cardiac anomalies. Int J Cardiol. 2008;123:302–6.

    Article  PubMed  Google Scholar 

  18. Knight L, Edwards JE. Right aortic arch. Types and associated cardiac anomalies. Circulation. 1974;50:1047–51.

    Article  CAS  PubMed  Google Scholar 

  19. Padalino MA, Vida VL, Stellin G. Transatrial-transpulmonary repair of tetralogy of Fallot. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2009;12(1):48–53.

    Google Scholar 

  20. Joyce JJ, Hwang EY, Wiles HB, Kline CH, Bradley SM, Crawford FA. Reliability of intraoperative transesophageal echocardiography during tetralogy of Fallot repair. Echocardiography. 2000;17:319–27.

    Article  CAS  PubMed  Google Scholar 

  21. Yang SG, Novello R, Nicolson S, et al. Evaluation of ventricular septal defect repair using intraoperative transesophageal echocardiography: frequency and significance of residual defects in infants and children. Echocardiography. 2000;17:681–4.

    Article  CAS  PubMed  Google Scholar 

  22. Belli E, Houyel L, Serraf A, Lacour-Gayet F, Petit J, Planché C. Transaortic closure of residual intramural ventricular septal defect. Ann Thorac Surg. 2000;69:1496–8.

    Article  CAS  PubMed  Google Scholar 

  23. Preminger TJ, Sanders SP, van der Velde ME, Castañeda AR, Lock JE. “Intramural” residual interventricular defects after repair of conotruncal malformations. Circulation. 1994;89:236–42.

    Article  CAS  PubMed  Google Scholar 

  24. Motta P, Miller-Hance WC. Transesophageal echocardiography in tetralogy of Fallot. Semin Cardiothorac Vasc Anesth. 2012;16(2):70–87.

    Google Scholar 

  25. Kaushal SK, Radhakrishanan S, Dagar KS, et al. Significant intraoperative right ventricular outflow gradients after repair for tetralogy of Fallot: to revise or not to revise? Ann Thorac Surg. 1999;68:1705–12; discussion 1712–3.

    Article  CAS  PubMed  Google Scholar 

  26. Zoghbi WA, Enriquez-Sarano M, Foster E, et al. Recommendations for evaluation of the severity of native valvular regurgitation with two-dimensional and Doppler echocardiography. J Am Soc Echocardiogr. 2003;16:777–802.

    Article  PubMed  Google Scholar 

  27. Bradley TJ, Karamlou T, Kulik A, et al. Determinants of repair type, reintervention, and mortality in 393 children with double-outlet right ventricle. J Thorac Cardiovasc Surg. 2007;134:967–973.e6.

    Article  PubMed  Google Scholar 

  28. Keane JF, Fyler DC. Double-outlet right ventricle. In: Keane JF, Lock JE, Fyler DC, Nadas AS, editors. Nadas’ pediatric cardiology. 2nd ed. Philadelphia: Saunders; 2006. p. 735–41.

    Google Scholar 

  29. Ikemoto Y, Nogi S, Teraguchi M, Imamura H, Kobayashi Y. Double-outlet right ventricle with intact ventricular septum. Acta Paediatr Jpn. 1997;39:233–6.

    Article  CAS  PubMed  Google Scholar 

  30. Vairo U, Tagliente MR, Fasano ML, Adurno G, Serino W. Double-outlet right ventricle with intact ventricular septum. Ital Heart J. 2001;2:397–400.

    CAS  PubMed  Google Scholar 

  31. Geva T, Van Praagh S, Sanders SP, Mayer JE, Van Praagh R. Straddling mitral valve with hypoplastic right ventricle, crisscross atrioventricular relations, double outlet right ventricle and dextrocardia: morphologic, diagnostic and surgical considerations. J Am Coll Cardiol. 1991;17:1603–12.

    Article  CAS  PubMed  Google Scholar 

  32. Hagler DJ. Double-outlet right ventricle and double outlet left ventricle. In: Allen HD, Dricoll DJ, Shaddy RE, Feltes TF, editors. Moss and Adams’ heart disease in infants, children, and adolescents: including the fetus and young adult. 7th ed. Philadelphia: Lippincott Williams & Wilkins; 2008. p. 1100–27.

    Google Scholar 

  33. Anderson RH, Becker AE, Wilcox BR, Macartney FJ, Wilkinson JL. Surgical anatomy of double-outlet right ventricle—a reappraisal. Am J Cardiol. 1983;52:555–9.

    Article  CAS  PubMed  Google Scholar 

  34. Belli E, Serraf A, Lacour-Gayet F, et al. Surgical treatment of subaortic stenosis after biventricular repair of double-outlet right ventricle. J Thorac Cardiovasc Surg. 1996;112:1570–8.

    Article  CAS  PubMed  Google Scholar 

  35. Bartram U, Molin DG, Wisse LJ, et al. Double-outlet right ventricle and overriding tricuspid valve reflect disturbances of looping, myocardialization, endocardial cushion differentiation, and apoptosis in TGF-beta(2)-knockout mice. Circulation. 2001;103:2745–52.

    Article  CAS  PubMed  Google Scholar 

  36. Kleinert S, Sano T, Weintraub RG, Mee RB, Karl TR, Wilkinson JL. Anatomic features and surgical strategies in double-outlet right ventricle. Circulation. 1997;96:1233–9.

    Article  CAS  PubMed  Google Scholar 

  37. Castaneda AR, Jonas RA, Mayer JE, Hanley FL. Double-outlet right ventricle. In: Cardiac surgery of the neonate and infant. Philadelphia: W.B. Saunders; 1994. p. 445–59.

    Google Scholar 

  38. Artrip JH, Sauer H, Campbell DN, et al. Biventricular repair in double outlet right ventricle: surgical results based on the STS-EACTS International Nomenclature classification. Eur J Cardiothorac Surg. 2006;29:545–50.

    Article  PubMed  Google Scholar 

  39. Morell VO, Jacobs JP, Quintessenza JA. Aortic translocation in the management of transposition of the great arteries with ventricular septal defect and pulmonary stenosis: results and follow-up. Ann Thorac Surg. 2005;79:2089–92; discussion 2092–3.

    Article  PubMed  Google Scholar 

  40. Yeh T, Ramaciotti C, Leonard SR, Roy L, Nikaidoh H. The aortic translocation (Nikaidoh) procedure: midterm results superior to the Rastelli procedure. J Thorac Cardiovasc Surg. 2007;133:461–9.

    Article  PubMed  Google Scholar 

  41. Penny DJ, Anderson RH. Common arterial trunk. In: Anderson RH, Baker EJ, Redington A, Rigby ML, Penny D, Wernovsky G, editors. Paediatric cardiology. 3rd ed. Philadelphia: Churchill Livingstone/Elsevier; 2009. p. 859–74.

    Google Scholar 

  42. Van Praagh R, Van Praagh S. The anatomy of common aorticopulmonary trunk (truncus arteriosus communis) and its embryologic implications. A study of 57 necropsy cases. Am J Cardiol. 1965;16:406–25.

    Article  PubMed  Google Scholar 

  43. Cabalka AK, Edwards WD, Dearani JA. Truncus arteriosus. In: Allen HD, Driscoll DJ, Shaddy RE, Feltes TF, editors. Moss and Adams’ heart disease in infants, children, and adolescents: including the fetus and young adult. 7th ed. Philadelphia: Lippincott Williams & Wilkins; 2008. p. 911–22.

    Google Scholar 

  44. Collett RW, Edwards JE. Persistent truncus arteriosus; a classification according to anatomic types. Surg Clin North Am. 1949;29:1245–70.

    CAS  PubMed  Google Scholar 

  45. Van Praagh R. Truncus arteriosus: what is it really and how should it be classified? Eur J Cardiothorac Surg. 1987;1:65.

    Article  PubMed  Google Scholar 

  46. Jacobs ML. Congenital heart surgery nomenclature and database project: truncus arteriosus. Ann Thorac Surg. 2000;69:S50–5.

    Article  CAS  PubMed  Google Scholar 

  47. McElhinney DB, Driscoll DA, Emanuel BS, Goldmuntz E. Chromosome 22q11 deletion in patients with truncus arteriosus. Pediatr Cardiol. 2003;24:569–73.

    Article  CAS  PubMed  Google Scholar 

  48. Silengo M, Rulli I, Delmonaco AG, Ferrero GB, Pucci A, Sanna R. Truncus arteriosus and isochromosome 8q. Am J Med Genet A. 2005;133A:223–4.

    Article  PubMed  Google Scholar 

  49. Calder L, Van Praagh R, Van Praagh S, et al. Truncus arteriosus communis. Clinical, angiocardiographic, and pathologic findings in 100 patients. Am Heart J. 1976;92:23–38.

    Article  CAS  PubMed  Google Scholar 

  50. Kalavrouziotis G, Purohit M, Ciotti G, Corno AF, Pozzi M. Truncus arteriosus communis: early and midterm results of early primary repair. Ann Thorac Surg. 2006;82:2200–6.

    Article  PubMed  Google Scholar 

  51. Pasquini L, Sanders SP, Parness IA, et al. Conal anatomy in 119 patients with d-loop transposition of the great arteries and ventricular septal defect: an echocardiographic and pathologic study. J Am Coll Cardiol. 1993;21:1712–21.

    Article  CAS  PubMed  Google Scholar 

  52. Norwood WI, Dobell AR, Freed MD, Kirklin JW, Blackstone EH. Intermediate results of the arterial switch repair. A 20-institution study. J Thorac Cardiovasc Surg. 1988;96:854–63.

    CAS  PubMed  Google Scholar 

  53. Van Praagh R, Weinberg PM, Smith SD, Foran RB, Van Praagh S. Malpositions of the heart. In: Adams FH, Emmanouilides GC, Riemenschneider TA, editors. Moss’ heart disease in infants, children and adolescents. 4th ed. Baltimore: Williams and Wilkins; 1989. p. 530–80.

    Google Scholar 

  54. Van Praagh R. Segmental approach to diagnosis. In: Keane JF, Lock JE, Fyler DC, editors. Nadas’ pediatric cardiology. 2nd ed. Philadelphia: Saunders Elsevier; 2006. p. 39–46.

    Google Scholar 

  55. Kouchoukos NT, Blackstone EH, Hanley FL, Doty DB, Karp RB. Complete transposition of the great arteries. In: Kirklin/Barratt-Boyes cardiac surgery. 3rd ed. Philadelphia: Churchill Livingstone; 2003. p. 1438–507.

    Google Scholar 

  56. Wernovsky G. Transposition of the great arteries. In: Allen HD, Dricoll DJ, Shaddy RE, Feltes TF, editors. Moss and Adams’ heart disease in infants, children, and adolescents: including the fetus and young adult. 7th ed. Philadelphia: Lippincott Williams & Wilkins; 2008. p. 1038–87.

    Google Scholar 

  57. Pasquini L, Sanders SP, Parness IA, et al. Coronary echocardiography in 406 patients with d-loop transposition of the great arteries. J Am Coll Cardiol. 1994;24:763–8.

    Article  CAS  PubMed  Google Scholar 

  58. Frohn-Mulder IM, Wesby Swaay E, Bouwhuis C, et al. Chromosome 22q11 deletions in patients with selected outflow tract malformations. Genet Couns. 1999;10:35–41.

    CAS  PubMed  Google Scholar 

  59. Kirjavainen M, Happonen JM, Louhimo I. Late results of Senning operation. J Thorac Cardiovasc Surg. 1999;117:488–95.

    Article  CAS  PubMed  Google Scholar 

  60. Lecompte Y, Neveux JY, Leca F, et al. Reconstruction of the pulmonary outflow tract without prosthetic conduit. J Thorac Cardiovasc Surg. 1982;84:727–33.

    CAS  PubMed  Google Scholar 

  61. Castaneda AR, Jonas RA, Mayer JE, Hanley FL. D-Transposition of the great arteries. In: Cardiac surgery of the neonate and infant. Philadelphia: W.B. Saunders; 1994. p. 409–38.

    Google Scholar 

  62. Marino BS, Wernovsky G, McElhinney DB, et al. Neo-aortic valvar function after the arterial switch. Cardiol Young. 2006;16:481–9.

    Article  PubMed  Google Scholar 

  63. Vouhé PR, Tamisier D, Leca F, Ouaknine R, Vernant F, Neveux JY. Transposition of the great arteries, ventricular septal defect, and pulmonary outflow tract obstruction. Rastelli or Lecompte procedure? J Thorac Cardiovasc Surg. 1992;103:428–36.

    PubMed  Google Scholar 

  64. Kaulitz R, Stümper OF, Geuskens R, et al. Comparative values of the precordial and transesophageal approaches in the echocardiographic evaluation of atrial baffle function after an atrial correction procedure. J Am Coll Cardiol. 1990;16:686–94.

    Article  CAS  PubMed  Google Scholar 

  65. Warnes CA. Transposition of the great arteries. Circulation. 2006;114:2699–709.

    Article  PubMed  Google Scholar 

  66. Allwork SP, Bentall HH, Becker AE, et al. Congenitally corrected transposition of the great arteries: morphologic study of 32 cases. Am J Cardiol. 1976;38:910–23.

    Article  CAS  PubMed  Google Scholar 

  67. Rodrigues AG, Mercier LA, Tardif JC, de Guise P. Aneurysm of the membranous ventricular septum and accessory valvular tissue causing obstruction of the pulmonary outflow tract in corrected transposition. J Am Soc Echocardiogr. 1995;8:957–60.

    Article  CAS  PubMed  Google Scholar 

  68. Anderson RH, Becker AE, Gerlis LM. The pulmonary outflow tract in classically corrected transposition. J Thorac Cardiovasc Surg. 1975;69:747–57.

    CAS  PubMed  Google Scholar 

  69. Anderson KR, Danielson GK, McGoon DC, Lie JT. Ebstein’s anomaly of the left-sided tricuspid valve: pathological anatomy of the valvular malformation. Circulation. 1978;58:I87–91.

    Article  CAS  PubMed  Google Scholar 

  70. Castaneda AR, Jonas RA, Mayer JE, Hanley FL. Corrected transposition of the great arteries. In: Cardiac surgery of the neonate and infant. Philadelphia: W.B. Saunders; 1994. p. 445–59.

    Google Scholar 

  71. Jaffe RB. Systemic atrioventricular valve regurgitation in corrected transpositon of the great vessels. Angiographic differentiation of operable and nonoperable valve deformities. Am J Cardiol. 1976;37:395–402.

    Article  CAS  PubMed  Google Scholar 

  72. Graham TP, 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;36:255–61.

    Article  PubMed  Google Scholar 

  73. Presbitero P, Somerville J, Rabajoli F, Stone S, Conte MR. Corrected transposition of the great arteries without associated defects in adult patients: clinical profile and follow up. Br Heart J. 1995;74:57–9.

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  74. Connelly MS, Liu PP, Williams WG, Webb GD, Robertson P, McLaughlin PR. Congenitally corrected transposition of the great arteries in the adult: functional status and complications. J Am Coll Cardiol. 1996;27:1238–43.

    Article  CAS  PubMed  Google Scholar 

  75. Brawn WJ, Jones TI, Anderson RH, Barron DJ. Congenitally corrected transposition. In: Anderson RH, Baker EJ, Redington A, Rigby ML, Penny D, Wernovsky G, editors. Paediatric cardiology. 3rd ed. Philadelphia: Churchill Livingstone/Elsevier; 2009. p. 819–35.

    Google Scholar 

  76. Piacentini G, Digilio MC, Capolino R, et al. Familial recurrence of heart defects in subjects with congenitally corrected transposition of the great arteries. Am J Med Genet A. 2005;137:176–80.

    Article  PubMed  Google Scholar 

  77. Digilio MC, Casey B, Toscano A, et al. Complete transposition of the great arteries: patterns of congenital heart disease in familial precurrence. Circulation. 2001;104:2809–14.

    Article  CAS  PubMed  Google Scholar 

  78. Wallis GA, Debich-Spicer D, Anderson RH. Congenitally corrected transposition. Orphanet J Rare Dis. 2011;6:1–12.

    Article  Google Scholar 

  79. Sreeram N, Stümper OF, Kaulitz R, Hess J, Roelandt JR, Sutherland GR. Comparative value of transthoracic and transesophageal echocardiography in the assessment of congenital abnormalities of the atrioventricular junction. J Am Coll Cardiol. 1990;16:1205–14.

    Article  CAS  PubMed  Google Scholar 

  80. Caso P, Ascione L, Lange A, Palka P, Mininni N, Sutherland GR. Diagnostic value of transesophageal echocardiography in the assessment of congenitally corrected transposition of the great arteries in adult patients. Am Heart J. 1998;135:43–50.

    Article  CAS  PubMed  Google Scholar 

  81. Vargas-Barron J, Rijlaarsdam M, Romero-Cardenas A, Keirns C, Diaz-Moncada S. Transesophageal echocardiography in adults with congenital cardiopathies. Am Heart J. 1993;126:426–32.

    Article  CAS  PubMed  Google Scholar 

  82. Lim H-G, Lee JR, Kim YJ, et al. Outcomes of biventricular repair for congenitally corrected transposition of the great arteries. Ann Thorac Surg. 2010;89:159–67.

    Article  PubMed  Google Scholar 

  83. Rutledge JM, Nihill MR, Fraser CD, Smith OE, McMahon CJ, Bezold LI. Outcome of 121 patients with congenitally corrected transposition of the great arteries. Pediatr Cardiol. 2002;23:137–45.

    Article  CAS  PubMed  Google Scholar 

  84. Hraska V, Duncan BW, Mayer JE, Freed M, del Nido PJ, Jonas RA. Long-term outcome of surgically treated patients with corrected transposition of the great arteries. J Thorac Cardiovasc Surg. 2005;129:182–91.

    Article  PubMed  Google Scholar 

  85. Bogers AJJC, Head SJ, de Jong PL, Witsenburg M, Kappetein AP. Long term follow up after surgery in congenitally corrected transposition of the great arteries with a right ventricle in the systemic circulation. J Cardiothorac Surg. 2010;5:74.

    Article  PubMed Central  PubMed  Google Scholar 

  86. Ilbawi MN, DeLeon SY, Backer CL, et al. An alternative approach to the surgical management of physiologically corrected transposition with ventricular septal defect and pulmonary stenosis or atresia. J Thorac Cardiovasc Surg. 1990;100:410–5.

    CAS  PubMed  Google Scholar 

  87. Murtuza B, Barron DJ, Stumper O, et al. Anatomic repair for congenitally corrected transposition of the great arteries: a single-institution 19-year experience. J Thorac Cardiovasc Surg. 2011;142:1348–57.

    Article  PubMed  Google Scholar 

  88. Zias EA, Mavroudis C, Cook KE, Makarewicz AJ, Backer CL, Hernandez JM. The effect of pulmonary circulation hemodynamics on right ventricular unloading via the bidirectional Glenn shunt: implications for congenitally corrected transposition repair. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2003;6:27–32.

    Article  PubMed  Google Scholar 

  89. Malhotra SP, Reddy VM, Qiu M, et al. The hemi-Mustard/bidirectional Glenn atrial switch procedure in the double-switch operation for congenitally corrected transposition of the great arteries: rationale and midterm results. J Thorac Cardiovasc Surg. 2011;141:162–70.

    Article  PubMed  Google Scholar 

  90. Chowdhury UK, Airan B, Sharma R, et al. One and a half ventricle repair with pulsatile bidirectional Glenn: results and guidelines for patient selection. Ann Thorac Surg. 2001;71:1995–2002.

    Article  CAS  PubMed  Google Scholar 

  91. Lee YO, Kim YJ, Lee JR, Kim W-H. Long-term results of one-and-a-half ventricle repair in complex cardiac anomalies. Eur J Cardiothorac Surg. 2011;39:711–5.

    Article  PubMed  Google Scholar 

  92. Kreutzer C, Mayorquim RC, Kreutzer GO, et al. Experience with one and a half ventricle repair. J Thorac Cardiovasc Surg. 1999;117:662–8.

    Article  CAS  PubMed  Google Scholar 

  93. Hraška V, Mattes A, Haun C, et al. Functional outcome of anatomic correction of corrected transposition of the great arteries. Eur J Cardiothorac Surg. 2011;40:1227–34.

    PubMed  Google Scholar 

  94. Quinn DW, McGuirk SP, Metha C, et al. The morphologic left ventricle that requires training by means of pulmonary artery banding before the double-switch procedure for congenitally corrected transposition of the great arteries is at risk of late dysfunction. J Thorac Cardiovasc Surg. 2008;135:1137–44.

    Article  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Division of Cardiology, Department of Pediatrics, The Children’s Hospital of Philadelphia, Perelman School of Medicine at the University of Pennsylvania, 34th St. & Civic Center Blvd., Philadelphia, PA, 19104, USA

    Laura M. Mercer-Rosa MD, MSCE & Meryl S. Cohen MD

Authors
  1. Laura M. Mercer-Rosa MD, MSCE
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Meryl S. Cohen MD
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Meryl S. Cohen MD .

Editor information

Editors and Affiliations

  1. Childrens Hospital Los Angeles Department of Cardiology, University of Southern California Keck School of Medicine, Los Angeles, California, USA

    Pierre C. Wong

  2. Pediatric Cardiovascular Anesthesiology, Texas Children's Hospital, Houston, USA

    Wanda C. Miller-Hance

Electronic Supplementary Material

Below is the link to the electronic supplementary material.

Tetralogy of Fallot: Mid esophageal four chamber view showing right ventricular hypertrophy, the large malalignment ventricular septal defect, and overriding aorta. LA left atrium, LV left ventricle, RV right ventricle (MPG 2454 kb)

Tetralogy of Fallot: Modified mid esophageal right ventricular inflow-outflow view (multiplane angle about 90°) showing the malalignment ventricular septal defect (VSD), as well as the narrowing of right ventricular outflow due to a malaligned conal septum. Ao aorta, MPA main pulmonary artery, PV pulmonary valve, RV right ventricle (MPG 3080 kb)

Tetralogy of Fallot: Deep transgastric long axis and sagittal views that simulate transthoracic subcostal coronal and sagittal views, showing the anteriorly malaligned infundibular septum producing subpulmonary stenosis. The spectral Doppler tracing shows the typical “dagger” shape seen with subpulmonary stenosis. Color Doppler displays the aliased flow across the right ventricular outflow tract and right to left shunting across the ventricular septal defect. Ao aorta, LV left ventricle, PA pulmonary artery, RA right atrium, RV right ventricle (MPG 13660 kb)

Mid esophageal four chamber view demonstrating right ventricular hypertrophy in a patient with tetralogy of Fallot. Also note the presence of a ventricular septal defect patch, in good position (MOV 10811 kb)

Preoperative study in a patient with tetralogy of Fallot. A prominent anterior descending coronary artery (arrow) arises from the right coronary artery (RCA) and courses anterior to the right ventricular outflow tract, thereby precluding a transannular patch. Ao aorta, PA main pulmonary artery (MPG 6644 kb)

125052_1_En_12_MOESM6_ESM.mov

Modified mid esophageal long axis view, with multiplane angle 87° in a patient with tetralogy of Fallot demonstrating a residual ventricular septal defect. The defect is located in the superior aspect of the patch just under the aortic valve. Shunting is seen into the right ventricular outflow tract (MOV 994 kb)

Upper esophageal aortic arch short axis with counterclockwise rotation, demonstrating a significant left pulmonary artery stenosis in this patient with tetralogy of Fallot who underwent a complete repair, including repair of discontinuous pulmonary arteries. Spectral continuous wave Doppler tracing shows the significant gradient across the stenotic area. Desc Ao descending aorta, LPA left pulmonary artery, MPA main pulmonary artery (MPG 9138 kb)

Residual right ventricular outflow tract narrowing is seen in a patient after tetralogy of Fallot repair in the mid esophageal right ventricular inflow-outflow view, with slight probe withdrawal to display the outflow tract more clearly (MOV 8121 kb)

Mid esophageal aortic valve long axis view demonstrating an unobstructed left ventricular outflow tract after repair of tetralogy of Fallot (MOV 5809 kb)

Mid esophageal four chamber view with probe withdrawn to evaluate the left ventricular outflow tract demonstrates that there is no residual VSD seen by color Doppler interrogation after tetralogy of Fallot repair. In addition, flow is laminar into the left ventricular outflow tract (MOV 6160 kb)

Mid esophageal four chamber view in a patient who has undergone tetralogy of Fallot and atrioventricular canal repair. A large ventricular septal defect patch is seen. Minimal residual atrioventricular valve regurgitation is seen across the right and left atrioventricular valves (MOV 6885 kb)

Severe tricuspid regurgitation prompted tricuspid valve replacement in this adult long after tetralogy of Fallot repair. This is a short Video 12.sweep that starts in a mid esophageal bicaval view, and as the probe is rotated counterclockwise (leftwards) to a modified mid esophageal right ventricular inflow-outflow view (multiplane angle 97°), the prosthetic valve appears in cross section (MOV 9588 kb)

Mid esophageal four chamber view in a patient with double outlet right ventricle, posterior malalignment of the conal septum, and subpulmonary ventricular septal defect, with the probe withdrawn towards the base of the heart. The aorta and pulmonary artery (PA) come into view, both arising from the right ventricle (RV). The PA is smaller than the aorta because of the conal septal malalignment and pulmonary outflow tract stenosis (MOV 11699 kb)

Mid esophageal aortic valve long axis view in the patient in Video 12.13 with double outlet right ventricle, posterior malalignment of the conal septum with subpulmonary ventricular septal defect highlighting the posterior deviation of the conal septum resulting in severe sub pulmonary stenosis. The conal septum is also hypertrophied (MOV 11746 kb)

Double outlet right ventricle with pulmonary outflow tract stenosis. Mid esophageal four chamber view shows a large ventricular septal defect (arrow). With probe withdrawal and anteflexion, the semilunar valves are visualized in a side-by-side orientation. However the pathway from left ventricle (LV) to aortic valve (AoV) is not clearly shown. Rotation of multiplane angle to about 90° and sweep from right to left shows right atrium (RA) and right ventricle (RV) as well as AoV, but it is still unclear whether the pathway from LV to AoV is unobstructed. The deep transgastric long axis view shows that this pathway is unobstructed; it also shows the origin of both great arteries from the RV. This patient successfully underwent a patch closure of the ventricular septal defect and relief of pulmonary outflow tract stenosis. LA left atrium, PV pulmonary valve (MPG 12812 kb)

Left juxtaposition of the atrial appendages in a patient with double outlet right ventricle and pulmonary stenosis. This Video was obtained with a mid esophageal aortic valve short axis view. Note how the right atrial appendage (RAA) crosses posterior to the great arteries to lie just anterior to the left atrial appendage (LAA). AoV aortic valve, RA right atrium, PV pulmonary valve (MPG 4432 kb)

Mid esophageal sagittal (90°) clockwise sweep (from left to right) in the patient from Videos 12.13 and 12.14 with double outlet right ventricle, posterior malalignment of the conal septum and subpulmonary ventricular septal defect after the Nikaidoh procedure (aortic translocation). The translocation of the aorta has placed the aortic valve closer to the left ventricle, improving left ventricle to aortic valve alignment and reducing the possibility of subaortic obstruction once the ventricular septal defect has been closed by a patch. This video demonstrates the “physiologic” repair achieved by baffling the ventricular septal defect to the aorta in its new position, and the right ventricle to pulmonary artery conduit (MOV 9880 kb)

Truncal valve seen en face from a modified mid esophageal aortic valve short axis view (angle 0°). This shows a quadricuspid truncal valve with thickened edges and a central area of noncoaptation associated with a small amount of valvar regurgitation (MPG 2940 kb)

Truncus arteriosus type “1½ ”. From the mid esophageal ascending aortic short axis, the right pulmonary artery (RPA) and left pulmonary artery (LPA) origins are seen immediately adjacent to each other, arising from the posterior aspect of the trunk (TRUN). Color flow demonstrates unobstructed flow across the origin of the branch pulmonary arteries. From the mid esophageal aortic valve long axis view the posterior origin of the pulmonary arteries (arrow) is also seen and the truncal valve shown to override the ventricular septal defect. Trace truncal valve regurgitation is demonstrated. Ao ascending aorta, LA left atrium, LV left ventricle, RV right ventricle (MPG 12542 kb)

Postop truncus repair, seen from mid esophageal four chamber view with probe anteflexion. The ventricular septal defect patch is shown by the arrow; no residual defect is present. A small amount of truncal valve regurgitation is seen. LV left ventricle, RV right ventricle (MPG 3370 kb)

Postop truncus arteriosus repair, seen from the mid esophageal aortic valve long axis view, multiplane angle about 75°, with imaging and color flow Doppler. The ventricular septal defect patch is seen as well as the takeoff of the conduit (COND) from the right ventricle (RV) to the pulmonary artery. LV left ventricle (MPG 4978 kb)

Transposition of the great arteries. Using a mid esophageal aortic valve short axis view, both semilunar valves are seen en face, with the aortic valve (AoV) anterior and rightward to the pulmonary valve (PV). LA left atrium, RA right atrium (MPG 2896 kb)

Origins of the right and left coronary arteries from the opposite sinuses in transposition of the great arteries, as seen from the mid esophageal aortic valve short axis view. This coronary pattern is the most common type seen in this cardiac defect. Ao aorta, PA pulmonary artery (MPG 5960 kb)

Transposition of the great arteries, as viewed from the mid esophageal four chamber view. With anteflexion and slight probe withdrawal, both great arteries are seen in parallel, with the aorta (Ao) arising from the right ventricle (RV), and the pulmonary artery (PA) from the left ventricle. Color flow Doppler shows continuous flow in the PA from ductal shunting (MPG 4938 kb)

Transposition of the great arteries, as viewed from a mid esophageal right ventricular inflow-outflow/long axis view, multiplane angle about 90°. Both great arteries are seen in parallel, with the aorta arising from the right ventricle, and the pulmonary artery from the left ventricle. Ao aorta, LA left atrium, LV left ventricle, PA pulmonary artery, RA right atrium, RV right ventricle. Color flow Doppler shows continuous flow in the PA (MPG 7050 kb)

Transposition of the great arteries, with a large ventricular septal defect and pulmonary outflow obstruction, as seen from modified mid esophageal four chamber, bicaval, and long axis views. A right to left sweep is performed from the bicaval to long axis view, showing the atrioventricular valves, ventricles, and ventricular septal defect. Posteriorly malaligned infundibular septum produces prominent subpulmonary narrowing, and turbulence is seen by color flow Doppler across the pulmonary outflow tract. Ao aorta, LA left atrium, LV left ventricle, PA pulmonary artery, PV pulmonary valve, RA right atrium, RV right ventricle, SVC superior vena cava (MPG 14052 kb)

Postoperative study following arterial switch operation. From the mid esophageal long axis view, the neo-aortic valve is seen with a small amount of regurgitation. The aortic anastomosis is shown by the arrow. Withdrawal of the probe to the mid esophageal ascending aorta long axis view shows the pulmonary artery anastomosis (arrow); the branch pulmonary arteries arise just above (superior to) the anastomosis. In this video, the right pulmonary artery is seen. Ao aorta, LA left atrium, LV left ventricle, MPA main pulmonary artery, RPA right pulmonary artery, RV right ventricle (MPG 12560 kb)

Postoperative study following arterial switch operation, showing the branch pulmonary arteries from the upper esophageal pulmonary artery long axis view. A Lecompte maneuver was performed so that the main and branch pulmonary arteries are situated anterior to the aorta (Ao). Color flow Doppler demonstrates flow in both the main and branch pulmonary arteries. LPA left pulmonary artery, RPA right pulmonary artery (MPG 4122 kb)

Mid esophageal right ventricular inflow-outflow view with the probe advanced toward the liver in an adult who has undergone an atrial switch operation for transposition of the great arteries. The color interrogation demonstrates flow from the pulmonary venous channel to the systemic venous channel (left to right shunt) (MOV 1276 kb)

Mid esophageal right ventricular inflow-outflow view in a patient with an atrial switch and a baffle leak. This video is obtained during device deployment. The image demonstrates an Amplatzer device just prior to release (MOV 3425 kb)

Mid esophageal four chamber view of a patient after atrial switch operation demonstrating the pulmonary venous channel as it makes its way to the tricuspid valve. In this patient, the pathway is unobstructed (MOV 3575 kb)

Mid esophageal bicaval view with probe turned clockwise toward the patient’s right side. This image is from a patient who has superior systemic venous limb obstruction after an atrial switch operation. In this view, both inferior and superior limbs of the systemic venous channel are seen with the pulmonary venous channel coursing in between. Pacing wires are seen in the superior limb. If the probe is turned further clockwise, the right pulmonary veins will be profiled (MOV 4143 kb)

Modified mid esophageal right ventricular inflow-outflow view using color Doppler in the same patient as Video 12.32 after atrial switch operation, with superior limb obstruction. Aliasing of the color flow is seen in the superior limb of the channel (MOV 1309 kb)

Congenitally corrected transposition of the great arteries. A mid esophageal four chamber view shows inferior displacement of the septal leaflet of the left sided tricuspid valve, compared with the right sided mitral valve. Incomplete coaptation of this valve results in a mild to moderate degree of tricuspid insufficiency. LA left atrium, LV left ventricle, RA right atrium, RV right ventricle (MPG 6474 kb)

Congenitally corrected transposition of the great arteries. Using the mid esophageal long axis view, the left sided tricuspid valve is seen, with significant regurgitation. Ao aorta, LA left atrium, PA pulmonary artery, RV right ventricle (MPG 5816 kb)

Mid esophageal four chamber view shows a large perimembranous ventricular septal defect (arrow) in a patient with congenitally corrected transposition of the great arteries. Note the left sided tricuspid valve chordal attachment to ventricular septum, as well as the presence of a moderator band in the left sided right ventricle (RV). LA left atrium, LV left ventricle, RA right atrium (MPG 3428 kb)

Congenitally corrected transposition of the great arteries. Transgastric mid short axis view shows the inverted ventricles in cross section. Note the smooth walled septal surface in the left ventricle (LV), and the prominent moderator band in the right ventricle (RV) (MPG 3158 kb)

Congenitally corrected transposition of the great arteries, as seen from the mid esophageal window in an approximately 90° sagittal plane. There is a “windsock” aneurysm of tissue (arrow) originating from the anterior leaflet of mitral valve and protruding into the pulmonary outflow tract, just below the pulmonary valve. In addition fibrous tissue from the mitral valve extends across the outflow tract and attaches to the ventricular septum. Neither of these was obstructive. A pulmonary artery band is seen in the main pulmonary artery, with flow acceleration noted across it. LA left atrium, LV left ventricle, PA pulmonary artery, RA right atrium (MPG 5940 kb)

Deep transgastric long axis view in a patient with congenitally corrected transposition of the great arteries and ventricular septal defect, in whom a pulmonary artery band (Band) was placed. The transducer first visualizes the right ventricle (RV) and ascending aorta (Ao). Further probe anteflexion and advancement is required to visualize the most posterior pulmonary artery (PA). Significant aliasing is noted across the band, and spectral Doppler calculated gradient is 84 mmHg. Note the excellent angle for Doppler interrogation across the band. LV left ventricle, RA right atrium, RV right ventricle (MPG 9990 kb)

Mid esophageal four chamber view in a patient with congenitally corrected transposition of the great arteries and ventricular septal defect. A sweep is performed in which the probe is simultaneously anteflexed and withdrawn to a view approximating a mid esophageal aortic valve short axis view. During this sweep, the aortic valve is seen to arise from the left sided right ventricle (RV), and there is subaortic conal muscle seen, along with discontinuity between the left sided tricuspid valve and aortic valve (AoV). At the same time, the pulmonary valve (PV), which arises from the right sided left ventricle (LV), is shown to have fibrous continuity with the right sided mitral valve. The coronary arteries are clearly seen arising from the anterior aorta. LA left atrium, RA right atrium (MPG 9066 kb)

Mid esophageal long axis view in congenitally corrected transposition of the great arteries, showing the parallel course of both aorta (Ao) and pulmonary artery (PA) as they arise from the heart. In this view, the ventriculoarterial discordance, and parallel arrangement of the great arteries, is very similar to that seen in D-transposition of the great arteries. Thus complementary views and sweeps are necessary to determine visceroatrial situs and atrioventricular connections. LA left atrium (MPG 4334 kb)

Deep transgastric long axis view in congenitally corrected transposition of the great arteries. With the probe advanced in the stomach to point the tip more posteriorly, the pulmonary artery (PA) is seen arising from the left ventricle (LV). The right atrium (RA) is also seen emptying into the LV. There is unobstructed flow across the pulmonary outflow tract. The probe is then withdrawn slightly, pointing the tip more anteriorly, to visualize the anterior aorta (Ao) arising from the right ventricle (RV). Flow across the aorta is also unobstructed. Note the moderator band present in the RV (MPG 10416 kb)

Mid esophageal sagittal sweep in congenitally corrected transposition of the great arteries. Starting with the mid esophageal bicaval view, the left atrium (LA), right atrium (RA), mitral valve and left ventricle (LV) are noted. The transducer is rotated counterclockwise (leftward) to visualize further the LV and pulmonary artery (PA). With further leftward probe rotation, the LA, tricuspid valve, right ventricle (RV) and aorta (Ao) are visualized. RAA right atrial appendage, SVC superior vena cava (MPG 9256 kb)

Same patient as Video 12.38. The pulmonary artery band is clearly seen well above the pulmonary valve, and color flow Doppler demonstrates significant aliasing across the band. LV left ventricle, PA pulmonary artery, RA right atrium, RV right ventricle (MPG 3578 kb)

Video 12.6

Modified mid esophageal long axis view, with multiplane angle 87° in a patient with tetralogy of Fallot demonstrating a residual ventricular septal defect. The defect is located in the superior aspect of the patch just under the aortic valve. Shunting is seen into the right ventricular outflow tract (MOV 994 kb)

Rights and permissions

Reprints and permissions

Copyright information

© 2014 Springer-Verlag London

About this chapter

Cite this chapter

Mercer-Rosa, L.M., Cohen, M.S. (2014). Evaluation of Conotruncal Abnormalities. In: Wong, P., Miller-Hance, W. (eds) Transesophageal Echocardiography for Congenital Heart Disease. Springer, London. https://doi.org/10.1007/978-1-84800-064-3_12

Download citation

  • DOI: https://doi.org/10.1007/978-1-84800-064-3_12

  • Published:

  • Publisher Name: Springer, London

  • Print ISBN: 978-1-84800-061-2

  • Online ISBN: 978-1-84800-064-3

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics

Search

Navigation