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Simple Transposition of the Great Arteries

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Abstract

Transposition of the great arteries (TGA) is one of the more common cyanotic congenital heart defects with an incidence of about 300 per million live births. In TGA, the ventriculo-arterial connection is reversed such that the majority of the aorta arises from the right ventricle while the majority of the pulmonary artery arises from the left ventricle. This defect is incompatible with long-term survival without surgical correction. Since the arterial switch procedure was introduced in the 1970s, early anatomic and physiologic repair has become the standard of care for these children with an expectation of excellent long-term outcomes for the majority.

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References

  1. Baillie M (1797) The morbid anatomy of some of the most important parts of the human body. Johnson and Nicol, London, pp 38–40

    Google Scholar 

  2. Farre J (1814) On malformations of the human heart. Longman, London

    Google Scholar 

  3. Peacock T (1866) On malformations of the human heart, etc.: with original cases and illustrations. John Churchill and Sons, London

    Google Scholar 

  4. Keith A (1909) The Hunterian lectures on malformation of the human heart. Lancet 174(4485):433–436

    Article  Google Scholar 

  5. Hoffman J, Kaplan S (2002) The incidence of congenital heart disease. J Am Coll Cardiol 39:1890–1900

    Article  PubMed  Google Scholar 

  6. Abu-Sulaiman R, Subaih B (2004) Congenital heart disease in infants of diabetic mothers: echocardiographic study. Pediatr Cardiol 25:137–140

    Article  CAS  PubMed  Google Scholar 

  7. Jenkins K, Correa A et al (2007) Noninherited risk factors and congenital cardiovascular defects: current knowledge: a scientific statement from the American Academy of Pediatrics. Circulation 115:2995–3014

    Article  PubMed  Google Scholar 

  8. Snijder C, Vlot I et al (2012) Congenital heart defects and parental occupational exposure to chemicals. Hum Reprod 27(5):1510–1517

    Article  CAS  PubMed  Google Scholar 

  9. Loffredo C, Silbergeld E et al (2001) Association of transposition of the great arteries in infants with maternal exposures to herbicides and rodenticides. Am J Epidemiol 153:529–536

    Article  CAS  PubMed  Google Scholar 

  10. Muncke N, Jung C et al (2003) Missense mutations and gene interruption in PROSIT240, a novel TRAP240-like gene, in patients with congenital heart disease (transposition of the great arteries). Circulation 108:2843–2850

    Article  CAS  PubMed  Google Scholar 

  11. Karkera J, Lee J et al (2007) Loss of function mutations in growth differentiation factor-1 (GDF-1) are associated with congenital heart defects in humans. Am J Hum Genet 81:987–994

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  12. Goldmuntz E, Bamford R et al (2002) CFC1 mutations in patients with transposition of the great arteries and double-outlet right ventricle. Am J Hum Genet 70:776–780

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  13. Amati F, Diano L et al (2010) Hif1α downregulation is associated with transposition of the great arteries in mice treated with a retinoic acid antagonist. BMC Genomics 11:497

    Article  PubMed  PubMed Central  Google Scholar 

  14. Huang J, Liu Y et al (2010) Molecular mechanisms of congenital heart disease. Cardiovasc Pathol 19(5):e183–e193

    Article  CAS  PubMed  Google Scholar 

  15. Liebman J, Cullum L et al (1969) Natural history of transposition of the great arteries. Anatomy and birth and death characteristics. Circulation 40:237–262

    Article  CAS  PubMed  Google Scholar 

  16. Jaggers J, Carmeron D et al (2000) Congenital heart surgery nomenclature and database project: transposition of the great arteries. Ann Thorac Surg 69:S205–S235

    Article  CAS  PubMed  Google Scholar 

  17. Van Praagh R (1984) Diagnosis of complex congenital heart disease: morphologic-anatomic method and terminology. Cardiovasc Intervent Radiol 7(3–4):115–120

    Article  PubMed  Google Scholar 

  18. Bartram U, Wirbelauer J et al (2005) Heterotaxy syndrome-asplenia and polysplenia as indicators of visceral malposition and complex congenital heart disease. Biol Neonate 88:278–290

    Article  PubMed  Google Scholar 

  19. Sim E, van Son J et al (1994) Coronary artery anatomy in complete transposition of the great arteries. Ann Thorac Surg 57:890–894

    Article  CAS  PubMed  Google Scholar 

  20. Li J, Tulloh R et al (2000) Coronary arterial origins in transposition of the great arteries: a morphologic study. Heart 83:320–325

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  21. Gittenberger-de Groot A, Sauer U et al (1983) Coronary arterial anatomy in transposition of the great arteries: a morphologic study. Pediatr Cardiol (Suppl 1):15–24

    Google Scholar 

  22. Newfeld E, Paul M et al (1974) Pulmonary vascular disease in complete transposition of the great arteries: a study of 200 patients. Am J Cardiol 34(1):75–82

    Article  CAS  PubMed  Google Scholar 

  23. Kumar A, Taylor G et al (1993) Pulmonary vascular disease in neonates with transposition of the great arteries and intact ventricular septum. Br Heart J 69:442–445

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  24. Rivenes S, Grifka R et al (1998) Development of advanced pulmonary vascular disease in D-transposition of the great arteries after neonatal arterial switch operation. Tex Heart Inst J 25(3):201–205

    CAS  PubMed  PubMed Central  Google Scholar 

  25. Huhta J (2005) Evaluating the fetus with transposition. Cardiol Young 15(Suppl 1):88–92

    Article  PubMed  Google Scholar 

  26. Rasiah S, Publicover M et al (2006) A systematic review of the accuracy of first-trimester ultrasound examination for detecting major congenital heart disease. Ultrasound Obstet Gynecol 28(1):110–116

    Article  CAS  PubMed  Google Scholar 

  27. Calderon J, Angeard N et al (2012) Outcomes in children with transposition of the great arteries. J Pediatr

    Google Scholar 

  28. de Wahl Granelli A, Wennergren M et al (2009) Impact of pulse oximetry screening on the detection of duct dependent congenital heart disease: a Swedish prospective screening study in 39,821 newborns. BMJ 338:a3037

    Article  Google Scholar 

  29. Maeno Y, Kamenir S et al (1999) Prenatal features of ductus arteriosus constriction and restrictive foramen ovale in d-transposition of the great arteries. Circulation 99(9):1209–1214

    Article  CAS  PubMed  Google Scholar 

  30. Roofthooft M, Bergman K et al (2007) Persistent pulmonary hypertension of the newborn with transposition of the great arteries. Ann Thorac Surg 83:1446–1450

    Article  PubMed  Google Scholar 

  31. Rashkind W, Miller W (1966) Creation of an atrial septal defect without thoracotomy. A palliative approach to complete transposition of the great arteries. J Am Coll Cardiol 196:991–992

    CAS  Google Scholar 

  32. McQuillen P, Hamrick S et al (2006) Balloon atrial septostomy is associated with preoperative stroke in neonates with transposition of the great arteries. Circulation 113(2):280–285

    Article  PubMed  Google Scholar 

  33. Petit C, Rome J et al (2009) Preoperative brain injury in transposition of the great arteries is associated with oxygenation and time of surgery, not balloon atrial septostomy. Circulation 119(5):709–716

    Article  PubMed  PubMed Central  Google Scholar 

  34. Applegate D, Lim D (2010) Incidence of stroke in patients with d-transposition of the great arteries that undergo balloon atrial septostomy in the University Health System Consortium Clinical Database/Resource Manager. Catheter Cardiovasc Interv 76(1):129–131

    Article  PubMed  Google Scholar 

  35. Muckerjee D, Lindsay M et al (2010) Analysis of 8681 neonates with transposition of the great arteries: outcomes with and without Rashkind balloon atrial septostomy. Cardiol Young 20(4):373–380

    Article  Google Scholar 

  36. Senning A (1959) Surgical correction of transposition of the great vessels. Surgery 45:966–979

    CAS  PubMed  Google Scholar 

  37. Mustard W (1964) Successful two-stage correction of transposition of the great vessels. Surgery 55:469–472

    CAS  PubMed  Google Scholar 

  38. Jatene A, Fontes V et al (1976) Anatomic correction of transposition of the great vessels. J Thorac Cardiovasc Surg 72:364–370

    CAS  PubMed  Google Scholar 

  39. LeCompte Y, Zannini L et al (1981) Anatomic correction of transposition of the great arteries. J Thorac Cardiovasc Surg 82:629–631

    CAS  PubMed  Google Scholar 

  40. el-Said G, Rosenberg H et al (1972) Dysrhythmias after mustard’s operation for transposition of the great arteries. Am J Cardiol 30:526–532

    Article  CAS  PubMed  Google Scholar 

  41. Kramer H, Rammos S et al (1991) Cardiac rhythm after mustard repair and after arterial switch operation for complete transposition. Int J Cardiol 32:5–12

    Article  CAS  PubMed  Google Scholar 

  42. Martin R, Qureshi S et al (1990) An evaluation of right and left ventricular function after anatomical correction and intra-atrial repair operations for complete transposition of the great arteries. Circulation 82:808–816

    Article  CAS  PubMed  Google Scholar 

  43. Backer C, Ilbawi M et al (1989) Transposition of the great arteries: a comparison of results of the mustard procedure versus the arterial switch. Ann Thorac Surg 48:10–14

    Article  CAS  PubMed  Google Scholar 

  44. Haas F, Wottke M et al (1999) Long-term survival and functional follow-up in patients after arterial switch operation. Ann Thorac Surg 68:1692–1697

    Article  CAS  PubMed  Google Scholar 

  45. Lupinetti F, Bove E et al (1992) Intermediate-term survival and functional results after arterial repair for transposition of the great arteries. J Thorac Cardiovasc Surg 103:421–427

    CAS  PubMed  Google Scholar 

  46. Davis A, Wilkinson J et al (1993) Transposition of the great arteries with intact ventricular septum: arterial switch repair in patients 21 days of age and older. J Thorac Cardiovasc Surg 106:111–115

    CAS  PubMed  Google Scholar 

  47. Rudra H, Mavroudis C et al (2011) The arterial switch operation: 25-year experience with 258 patients. Ann Thorac Surg 92(5):1742–1746

    Article  PubMed  Google Scholar 

  48. Adhyapak S, Mahala B et al (2007) Impact of left ventricular function on early outcomes after arterial switch for D-transposition of the great arteries with intact ventricular septum. Indian Heart J 59(2):137–141

    PubMed  Google Scholar 

  49. Stoica S, Clarke D et al (2009) Morbidity factors in the arterial switch operation. Cardiol Young 19:148–149

    Google Scholar 

  50. Seear M, Scarfe J et al (2008) Predicting major adverse events after cardiac surgery in children. Pediatr Crit Care Med 9(6):606–611

    Article  PubMed  Google Scholar 

  51. Bhutta A, Ford J et al (2007) Noninvasive cerebral oximeter as a surrogate for mixed venous saturation in children. Pediatr Cardiol 28(1):34–41

    Article  PubMed  Google Scholar 

  52. Mittnacht A (2010) Near infrared spectroscopy in children at high risk of low perfusion. Curr Opin Anaesthesiol 23(3):342–347

    Article  PubMed  Google Scholar 

  53. Gaynor J (2003) The effect of modified ultrafiltration on postoperative course in patients with congenital heart disease. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 6:128–139

    Article  PubMed  Google Scholar 

  54. Falcao G, Ulate K et al (2008) Impact of postoperative hyperglycemia following surgical repair of congenital cardiac defects. Pediatr Cardiol 29(3):628–636

    Article  PubMed  Google Scholar 

  55. Dibardino D, Allison A et al (2004) Current expectations for newborns undergoing the arterial switch operation. Ann Surg 239(5):588–598

    Article  PubMed  PubMed Central  Google Scholar 

  56. Hirsch J, Gurney J et al (2008) Hospital mortality for Norwood and arterial switch operations as a function of institutional volume. Pediatr Cardiol 29(4):713–717

    Article  PubMed  Google Scholar 

  57. Lalezari S, Bruggemans E et al (2011) Thirty-year experience with the arterial switch operation. Ann Thorac Surg 92(3):973–979

    Article  PubMed  Google Scholar 

  58. Wong S, Finucane K et al (2008) Cardiac outcome up to 15 years after the arterial switch operation. Heart Lung Circ 17(1):48–53

    Article  PubMed  Google Scholar 

  59. Qamar Z, Goldberg C et al (2007) Current risk factors and outcomes for the arterial switch operation. Ann Thorac Surg 84(3):871–878

    Article  PubMed  Google Scholar 

  60. Massin M (1999) Midterm results of the neonatal arterial switch operation. A review. J Cardiovasc Surg 40(4):517–522

    CAS  Google Scholar 

  61. Losay J, Touchot A et al (2001) Late outcome after arterial switch operation for transposition of the great arteries. Circulation 104(12 (Suppl 1)):I121–I126

    Article  CAS  PubMed  Google Scholar 

  62. Prifti E, Crucean A et al (2002) Early and long term outcome of the arterial switch operation for transposition of the great arteries: predictors and functional evaluation. Eur J Cardiothorac Surg 22(6):864–873

    Article  PubMed  Google Scholar 

  63. Choi B, Kwon B et al (2010) Long-term outcomes after an arterial switch operation for simple complete transposition of the great arteries. Korean Circ J 40(1):23–30

    Article  PubMed  PubMed Central  Google Scholar 

  64. Gorler H, Ono M et al (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(4):569–574

    Article  PubMed  Google Scholar 

  65. Bonnet D, Bonhoeffer P et al (1996) Long-term fate of the coronary arteries after the arterial switch operation in newborns with transposition of the great arteries. Heart 76(3):274–279

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  66. Manso B, Castellote A et al (2010) Myocardial perfusion magnetic resonance imaging for detecting coronary function anomalies in asymptomatic paediatric patients with a previous arterial switch operation for transposition of the great arteries. Cardiol Young 20(4):410–417

    Article  CAS  PubMed  Google Scholar 

  67. Legendre A, Losay J, Losay J, Losay J et al (2003) Coronary events after arterial switch operation for transposition of the great arteries. Circulation 108(Suppl 33):II186–II190

    PubMed  Google Scholar 

  68. El-Segaier M, Lundin A et al (2010) Late coronary complications after arterial switch operation and their treatment. Catheter Cardiovasc Interv 76(7):1027–1032

    Article  PubMed  Google Scholar 

  69. Mavroudis C, Stewart R et al (2011) Reoperative techniques for complications after arterial switch. Ann Thorac Surg 92(5):1747–1754

    Article  PubMed  Google Scholar 

  70. Lange R, Cleuziou J et al (2008) Risk factors for aortic insufficiency and aortic valve replacement after the arterial switch operation. Eur J Cardiothorac Surg 34(4):711–717

    Article  PubMed  Google Scholar 

  71. McMahon C, Ravekes W et al (2004) Risk factors for neo-aortic root enlargement and aortic regurgitation following arterial switch operation. Pediatr Cardiol 25(4):329–335

    Article  CAS  PubMed  Google Scholar 

  72. Tang G, Borger M (2005) Aortic root replacement surgery: indications, techniques, and outcomes. Expert Rev Cardiovasc Ther 3(5):845–856

    Article  PubMed  Google Scholar 

  73. McElhinney D, Reddy V et al (2000) Impaired distensibility of neoaorta after arterial switch procedure. Ann Thorac Surg 65(1):246–248

    Article  Google Scholar 

  74. Moll J, Michalak K et al (2012) Long-term outcome of direct neopulmonary artery reconstruction during the arterial switch procedure. Ann Thorac Surg 93(1):177–184

    Article  PubMed  Google Scholar 

  75. Wernovsky G, Mayer J Jr et al (1995) Factors influencing early and late outcome of the arterial switch operation for transposition of the great arteries. J Thorac Cardiovasc Surg 109(2):282–289

    Article  Google Scholar 

  76. Prapa M, Dimopoulos K (2012) Arterial switch repair to transposition of the great arteries: so far so good. Int J Cardiol 160(1):1–3

    Article  PubMed  Google Scholar 

  77. De Koning W, Van Osch-Gevers M et al (2008) Follow-up outcomes 10 years after arterial switch operation for transposition of the great arteries: comparison of cardiological health status and health-related quality of life to those of the normal reference population. Eur J Pediatr 167(9):995–1004

    Article  PubMed  Google Scholar 

  78. Tobler D, Williams W et al (2010) Cardiac outcomes in young adult survivors of the arterial switch operation for transposition of the great arteries. J Am Coll Cardiol 56(1):58–64

    Article  PubMed  Google Scholar 

  79. Newburger J, Jonas R et al (1993) A comparison of the perioperative neurologic effects of hypothermic circulatory arrest versus low-flow cardiopulmonary bypass in infant heart surgery. N Engl J Med 329(15):1057–1064

    Article  CAS  PubMed  Google Scholar 

  80. Bellinger D, Wypij D et al (2003) Neurodevelopmental status at eight years in children with dextro-transposition of the great arteries: the Boston circulatory arrest trial. J Thorac Cardiovasc Surg 126(5):1385–1396

    Article  PubMed  Google Scholar 

  81. Bellinger D, Newburger J et al (2009) Behaviour at eight years in children with surgically corrected transposition: the Boston circulatory arrest trial. Cardiol Young 19(1):86–97

    Article  PubMed  Google Scholar 

  82. Wypij D, Newburger J et al (2003) The effect of duration of deep hypothermic circulatory arrest in infant heart surgery on late neurodevelopment: the Boston circulatory arrest trial. J Thorac Cardiovasc Surg 126(5):1397–1403

    Article  PubMed  Google Scholar 

  83. Bellinger D, Wypij D et al (2011) Adolescents with d-transposition of the great arteries corrected with the arterial switch procedure: neuropsychological assessment and structural brain imaging. Circulation 124(12):1361–1369

    Article  PubMed  PubMed Central  Google Scholar 

  84. Mahle W (2011) Boston circulatory arrest study at 16 years: handing over the keys. Circulation 124(12):1319–1320

    Article  PubMed  Google Scholar 

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Correspondence to Paul M. Kirshbom .

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Deshpande, S., Wolf, M.J., Kim, D.W., Kirshbom, P.M. (2014). Simple Transposition of the Great Arteries. In: Da Cruz, E., Ivy, D., Jaggers, J. (eds) Pediatric and Congenital Cardiology, Cardiac Surgery and Intensive Care. Springer, London. https://doi.org/10.1007/978-1-4471-4619-3_45

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  • DOI: https://doi.org/10.1007/978-1-4471-4619-3_45

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