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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Baillie M (1797) The morbid anatomy of some of the most important parts of the human body. Johnson and Nicol, London, pp 38–40
Farre J (1814) On malformations of the human heart. Longman, London
Peacock T (1866) On malformations of the human heart, etc.: with original cases and illustrations. John Churchill and Sons, London
Keith A (1909) The Hunterian lectures on malformation of the human heart. Lancet 174(4485):433–436
Hoffman J, Kaplan S (2002) The incidence of congenital heart disease. J Am Coll Cardiol 39:1890–1900
Abu-Sulaiman R, Subaih B (2004) Congenital heart disease in infants of diabetic mothers: echocardiographic study. Pediatr Cardiol 25:137–140
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
Snijder C, Vlot I et al (2012) Congenital heart defects and parental occupational exposure to chemicals. Hum Reprod 27(5):1510–1517
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
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
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
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
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
Huang J, Liu Y et al (2010) Molecular mechanisms of congenital heart disease. Cardiovasc Pathol 19(5):e183–e193
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
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
Van Praagh R (1984) Diagnosis of complex congenital heart disease: morphologic-anatomic method and terminology. Cardiovasc Intervent Radiol 7(3–4):115–120
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
Sim E, van Son J et al (1994) Coronary artery anatomy in complete transposition of the great arteries. Ann Thorac Surg 57:890–894
Li J, Tulloh R et al (2000) Coronary arterial origins in transposition of the great arteries: a morphologic study. Heart 83:320–325
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
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
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
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
Huhta J (2005) Evaluating the fetus with transposition. Cardiol Young 15(Suppl 1):88–92
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
Calderon J, Angeard N et al (2012) Outcomes in children with transposition of the great arteries. J Pediatr
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
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
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
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
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
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
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
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
Senning A (1959) Surgical correction of transposition of the great vessels. Surgery 45:966–979
Mustard W (1964) Successful two-stage correction of transposition of the great vessels. Surgery 55:469–472
Jatene A, Fontes V et al (1976) Anatomic correction of transposition of the great vessels. J Thorac Cardiovasc Surg 72:364–370
LeCompte Y, Zannini L et al (1981) Anatomic correction of transposition of the great arteries. J Thorac Cardiovasc Surg 82:629–631
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
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
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
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
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
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
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
Rudra H, Mavroudis C et al (2011) The arterial switch operation: 25-year experience with 258 patients. Ann Thorac Surg 92(5):1742–1746
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
Stoica S, Clarke D et al (2009) Morbidity factors in the arterial switch operation. Cardiol Young 19:148–149
Seear M, Scarfe J et al (2008) Predicting major adverse events after cardiac surgery in children. Pediatr Crit Care Med 9(6):606–611
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
Mittnacht A (2010) Near infrared spectroscopy in children at high risk of low perfusion. Curr Opin Anaesthesiol 23(3):342–347
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
Falcao G, Ulate K et al (2008) Impact of postoperative hyperglycemia following surgical repair of congenital cardiac defects. Pediatr Cardiol 29(3):628–636
Dibardino D, Allison A et al (2004) Current expectations for newborns undergoing the arterial switch operation. Ann Surg 239(5):588–598
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
Lalezari S, Bruggemans E et al (2011) Thirty-year experience with the arterial switch operation. Ann Thorac Surg 92(3):973–979
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
Qamar Z, Goldberg C et al (2007) Current risk factors and outcomes for the arterial switch operation. Ann Thorac Surg 84(3):871–878
Massin M (1999) Midterm results of the neonatal arterial switch operation. A review. J Cardiovasc Surg 40(4):517–522
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
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
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
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
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
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
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
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
Mavroudis C, Stewart R et al (2011) Reoperative techniques for complications after arterial switch. Ann Thorac Surg 92(5):1747–1754
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
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
Tang G, Borger M (2005) Aortic root replacement surgery: indications, techniques, and outcomes. Expert Rev Cardiovasc Ther 3(5):845–856
McElhinney D, Reddy V et al (2000) Impaired distensibility of neoaorta after arterial switch procedure. Ann Thorac Surg 65(1):246–248
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
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
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
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
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
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
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
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
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
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
Mahle W (2011) Boston circulatory arrest study at 16 years: handing over the keys. Circulation 124(12):1319–1320
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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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