Clinical and Experimental Pediatrics

The Korean Pediatric Society (대한소아청소년과학회)
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Primary repair of symptomatic neonates with tetralogy of Fallot with or without pulmonary atresia

  • Lee, Chang-Ha (Department of Thoracic Cardiovascular Surgery, Sejong General Hospital) ;
  • Kwak, Jae Gun (Department of Thoracic Cardiovascular Surgery, Sejong General Hospital) ;
  • Lee, Cheul (Department of Thoracic Cardiovascular Surgery, Sejong General Hospital)
  • Received : 2013.06.24
  • Accepted : 2013.09.10
  • Published : 2014.01.15
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Abstract

Recently, surgical outcomes of repair of tetralogy of Fallot (TOF) have improved. For patients with TOF older than 3 months, primary repair has been advocated regardless of symptoms. However, a surgical approach to symptomatic TOF in neonates or very young infants remains elusive. Traditionally, there have been two surgical options for these patients: primary repair versus an initial aortopulmonary shunt followed by repair. Early primary repair provides several advantages, including avoidance of shunt-related complications, early relief of hypoxia, promotion of normal lung development, avoidance of ventricular hypertrophy and fibrosis, and psychological comfort to the family. Because of advances in cardiopulmonary bypass techniques and accumulated experience in neonatal cardiac surgery, primary repair in neonates with TOF has been performed with excellent early outcomes (early mortality<5%), which may be superior to the outcomes of aortopulmonary shunting. A remaining question regarding surgical options is whether shunts can preserve the pulmonary valve annulus for TOF neonates with pulmonary stenosis. Symptomatic neonates and older infants have different anatomies of right ventricular outflow tract (RVOT) obstructions, which in neonates are nearly always caused by a hypoplastic pulmonary valve annulus instead of infundibular obstruction. Therefore, a shunt is less likely to preserve the pulmonary valve annulus than is primary repair. Primary repair of TOF can be performed safely in most symptomatic neonates. Patients who have had primary repair should be closely followed up to evaluate the RVOT pathology and right ventricular function.

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References

  1. Apitz C, Webb GD, Redington AN. Tetralogy of Fallot. Lancet 2009;374:1462-71. https://doi.org/10.1016/S0140-6736(09)60657-7
  2. Van Praagh R. The first Stella van Praagh memorial lecture: the history and anatomy of tetralogy of Fallot. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2009:19-38.
  3. Starr JP. Tetralogy of fallot: yesterday and today. World J Surg 2010;34:658-68. https://doi.org/10.1007/s00268-009-0296-8
  4. Blalock A, Taussig HB. The surgical treatment of malformations of the heartin in which there is pulmonary stenosis or pulmonary atresia. JAMA 1945;128:189-202. https://doi.org/10.1001/jama.1945.02860200029009
  5. Lillehei CW, Cohen M, Warden HE, Read RC, Aust JB, Dewall RA, et al. Direct vision intracardiac surgical correction of the tetralogy of Fallot, pentalogy of Fallot, and pulmonary atresia defects; report of first ten cases. Ann Surg 1955;142:418-42. https://doi.org/10.1097/00000658-195509000-00010
  6. Kirklin JW, Dushane JW, Patrick RT, Donald DE, Hetzel PS, Harshbarger HG, et al. Intracardiac surgery with the aid of a mechanical pump-oxygenator system (gibbon type): report of eight cases. Proc Staff Meet Mayo Clin 1955;30:201-6.
  7. Castaneda AR, Freed MD, Williams RG, Norwood WI. Repair of tetralogy of Fallot in infancy: early and late results. J Thorac Cardiovasc Surg 1977;74:372-81.
  8. Barratt-Boyes BG, Neutze JM. Primary repair of tetralogy of Fallot in infancy using profound hypothermia with circulatory arrest and limited cardiopulmonary bypass: a comparison with conventional two stage management. Ann Surg 1973;178:406-11. https://doi.org/10.1097/00000658-197310000-00003
  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(6 Suppl):S592-6. https://doi.org/10.1016/0003-4975(95)00732-6
  10. 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. https://doi.org/10.1097/00000658-200010000-00006
  11. 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. https://doi.org/10.1016/j.ejcts.2005.05.014
  12. Tamesberger MI, Lechner E, Mair R, Hofer A, Sames-Dolzer E, Tulzer G. Early primary repair of tetralogy of fallot in neonates and infants less than four months of age. Ann Thorac Surg 2008;86:1928-35. https://doi.org/10.1016/j.athoracsur.2008.07.019
  13. Kwak JG, Lee CH, Lee C, Park CS. Surgical management of pulmonary atresia with ventricular septal defect: early total correction versus shunt. Ann Thorac Surg 2011;91:1928-34. https://doi.org/10.1016/j.athoracsur.2011.01.014
  14. Kanter KR, Kogon BE, Kirshbom PM, Carlock PR. Symptomatic neonatal tetralogy of Fallot: repair or shunt? Ann Thorac Surg 2010;89:858-63. https://doi.org/10.1016/j.athoracsur.2009.12.060
  15. Anderson RH, Jacobs ML. The anatomy of tetralogy of Fallot with pulmonary stenosis. Cardiol Young 2008;18 Suppl 3:12-21. https://doi.org/10.1017/S1047951108003259
  16. Bailliard F, Anderson RH. Tetralogy of Fallot. Orphanet J Rare Dis 2009;4:2. https://doi.org/10.1186/1750-1172-4-2
  17. Hirsch JC, Bove EL. Tetralogy of Fallot. In: Mavroudis C, Backer CL, editors. Pediatric cardiac surgery. 3rd ed. Philadelphia: Mosby, 2003:383-97.
  18. Parry AJ, McElhinney DB, Kung GC, Reddy VM, Brook MM, Hanley FL. Elective primary repair of acyanotic tetralogy of Fallot in early infancy: overall outcome and impact on the pulmonary valve. J Am Coll Cardiol 2000;36:2279-83. https://doi.org/10.1016/S0735-1097(00)00989-X
  19. Chiariello L, Meyer J, Wukasch DC, Hallman GL, Cooley DA. Intracardiac repair of tetralogy of Fallot. Five-year review of 403 patients. J Thorac Cardiovasc Surg 1975;70:529-35.
  20. Farouk A, Zahka K, Siwik E, Erenberg F, Al-Khatib Y, Golden A, et al. Individualized approach to the surgical treatment of tetralogy of Fallot with pulmonary atresia. Cardiol Young 2009;19:76-85. https://doi.org/10.1017/S1047951108003430
  21. Stewart RD, Mavroudis C, Backer CL. Tetralogy of Fallot. In: Mavroudis C, Backer CL, editors. Pediatric cardiac surgery. 4th ed. Chichester: Wiley-Blackwell, 2013:410-27.
  22. Al Habib HF, Jacobs JP, Mavroudis C, Tchervenkov CI, O'Brien SM, Mohammadi S, et al. Contemporary patterns of management of tetralogy of Fallot: data from the Society of Thoracic Surgeons Database. Ann Thorac Surg 2010;90:813-9. https://doi.org/10.1016/j.athoracsur.2010.03.110
  23. Karl TR, Sano S, Pornviliwan S, Mee RB. Tetralogy of Fallot: favorable outcome of nonneonatal transatrial, transpulmonary repair. Ann Thorac Surg 1992;54:903-7. https://doi.org/10.1016/0003-4975(92)90646-L
  24. Fraser CD Jr, McKenzie ED, Cooley DA. Tetralogy of Fallot: surgical management individualized to the patient. Ann Thorac Surg 2001; 71:1556-61. https://doi.org/10.1016/S0003-4975(01)02475-4
  25. Li JS, Yow E, Berezny KY, Rhodes JF, Bokesch PM, Charpie JR, et al. Clinical outcomes of palliative surgery including a systemic-to-pulmonary artery shunt in infants with cyanotic congenital heart disease: does aspirin make a difference? Circulation 2007;116:293-7. https://doi.org/10.1161/CIRCULATIONAHA.106.652172
  26. Guzzetta NA, Foster GS, Mruthinti N, Kilgore PD, Miller BE, Kanter KR. In-hospital shunt occlusion in infants undergoing a modified blalock-taussig shunt. Ann Thorac Surg 2013;96:176-82. https://doi.org/10.1016/j.athoracsur.2013.03.026
  27. Fermanis GG, Ekangaki AK, Salmon AP, Keeton BR, Shore DF, Lamb RK, et al. Twelve year experience with the modified Blalock-Taussig shunt in neonates. Eur J Cardiothorac Surg 1992;6:586-9. https://doi.org/10.1016/1010-7940(92)90131-G
  28. Fenton KN, Siewers RD, Rebovich B, Pigula FA. Interim mortality in infants with systemic-to-pulmonary artery shunts. Ann Thorac Surg 2003;76:152-6. https://doi.org/10.1016/S0003-4975(03)00168-1
  29. Dodge-Khatami A, Tulevski II, Hitchcock JF, de Mol BA, Bennink GB. Neonatal complete correction of tetralogy of Fallot versus shunting and deferred repair: is the future of the right ventriculo-arterial junction at stake, and what of it? Cardiol Young 2001;11:484-90. https://doi.org/10.1017/S1047951101000701
  30. Redington AN. Physiopathology of right ventricular failure. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2006:3-10.
  31. Sousa Uva M, Lacour-Gayet F, Komiya T, Serraf A, Bruniaux J, Touchot A, et al. Surgery for tetralogy of Fallot at less than six months of age. J Thorac Cardiovasc Surg 1994;107:1291-300.
  32. Sousa Uva M, Chardigny C, Galetti L, Lacour Gayet F, Roussin R, Serraf A, et al. Surgery for tetralogy of Fallot at less than six months of age. Is palliation "old-fashioned"? Eur J Cardiothorac Surg 1995;9:453-9. https://doi.org/10.1016/S1010-7940(05)80082-X
  33. Di Donato RM, Jonas RA, Lang P, Rome JJ, Mayer JE Jr, Castaneda AR. Neonatal repair of tetralogy of Fallot with and without pulmonary atresia. J Thorac Cardiovasc Surg 1991;101:126-37.
  34. Emani SM, Beroukhim R, Zurakowski D, Pigula FA, Mayer JE, del Nido PJ, et al. Outcomes after anatomic repair for d-transposition of the great arteries with left ventricular outflow tract obstruction. Circulation 2009;120(11 Suppl):S53-8. https://doi.org/10.1161/CIRCULATIONAHA.108.843102
  35. Kirklin JW, Blackstone EH, Jonas RA, Shimazaki Y, Kirklin JK, Mayer JE Jr, et al. Morphologic and surgical determinants of outcome events after repair of tetralogy of Fallot and pulmonary stenosis: a two-institution study. J Thorac Cardiovasc Surg 1992; 103:706-23.
  36. Pigula FA, Khalil PN, Mayer JE, del Nido PJ, Jonas RA. Repair of tetralogy of Fallot in neonates and young infants. Circulation 1999; 100(19 Suppl):II157-61. https://doi.org/10.1161/01.CIR.100.25.e157
  37. Pozzi M, Trivedi DB, Kitchiner D, Arnold RA. Tetralogy of Fallot: what operation, at which age. Eur J Cardiothorac Surg 2000;17:631-6. https://doi.org/10.1016/S1010-7940(00)00415-2
  38. Jonas RA. Early primary repair of tetralogy of Fallot. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2009:39-47.
  39. Derby CD, Pizarro C. Routine primary repair of tetralogy of Fallot in the neonate. Expert Rev Cardiovasc Ther 2005;3:857-63. https://doi.org/10.1586/14779072.3.5.857
  40. Van Arsdell G, Yun TJ. An apology for primary repair of tetralogy of Fallot. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2005:128-31.
  41. McGoon DC, Baird DK, Davis GD. Surgical management of large bronchial collateral arteries with pulmonary stenosis or atresia. Circulation 1975;52:109-18. https://doi.org/10.1161/01.CIR.52.1.109
  42. Nakata S, Imai Y, Takanashi Y, Kurosawa H, Tezuka K, Nakazawa M, et al. A new method for the quantitative standardization of cross-sectional areas of the pulmonary arteries in congenital heart diseases with decreased pulmonary blood flow. J Thorac Cardiovasc Surg 1984;88:610-9.

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