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Original article

Vol. 149 No. 2728 (2019)

Late correction of tetralogy of Fallot in children

  • Paul P. Heinisch
  • Laetitia Guarino
  • Damian Hutter
  • Maris Bartkevics
  • Gabor Erdoes
  • Balthasar Eberle
  • Carlos Royo
  • Jaafar Rhissass
  • Jean-Pierre Pfammatter
  • Thierry Carrel
  • Alexander Kadner
Cite this as:
Swiss Med Wkly. 2019;149:w20096



To report our experience of late correction after infancy in patients with tetralogy of Fallot (ToF).


Observational single-centre retrospective analysis of the surgical techniques and perioperative development of patients from developing countries undergoing total surgical correction of ToF after infancy, between 1 November 2011 and 30 November 2016. Variables are presented as numbers with percentages or as mean ± standard deviation. Due to the setting of the humanitarian programme, clinical and echocardiographic follow-up procedures could be conducted for only one month postoperatively.


Twenty-five children (mean age: 70.8 ± 42 months, range 23-163; 44% female) underwent total surgical correction of ToF. Two patients (0.8%) initially received a Blalock-Taussig shunt and underwent subsequent correction 24 and 108 months later, respectively. Preoperative mean right ventricular/pulmonary artery (RV/PA) gradient was 84 ± 32 mm Hg, with a Nakata index of 164 ± 71 mm2/m2. Major aortopulmonary collateral arteries (MAPCAs) were observed in eight children (32%), six (26%) of whom underwent transcatheter closure before surgery. 24 children (96%) underwent a valve-sparing pulmonary valve repair and one patient received a transannular patch (TAP). There were no cases which saw major adverse cardiac and cerebrovascular events (MACCE). Mean duration of mechanical ventilation was 28 ± 19.6 hours (range 7-76). Pre-discharge echocardiography demonstrated a mean RV/PA gradient of 25 ± 5.7 mm Hg, with left ventricular ejection fraction >60% in all cases. Overall length of hospital stay was 11.7 ± 4.5 days. There were no in-hospital mortality cases.


Late surgical correction of ToF can be safely performed and produce highly satisfying early postoperative results comparable to those of classical “timely” correction. A valve-sparing technique can be applied in the majority of children.


  1. Steiner MB, Tang X, Gossett JM, Malik S, Prodhan P. Timing of complete repair of non-ductal-dependent tetralogy of Fallot and short-term postoperative outcomes, a multicenter analysis. J Thorac Cardiovasc Surg. 2014;147(4):1299–305. doi:.
  2. Van Arsdell GS, Maharaj GS, Tom J, Rao VK, Coles JG, Freedom RM, et al. What is the optimal age for repair of tetralogy of Fallot? Circulation. 2000;102(19, Suppl 3):III123–9. doi:.
  3. 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(7):2279–83. doi:.
  4. 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(3):813–9, discussion 819–20. doi:.
  5. 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(6):1928–35. doi:.
  6. Tchoumi JCT, Ambassa JC, Giamberti A, Cirri S, Frigiola A, Butera G. Late surgical treatment of tetralogy of Fallot. Cardiovasc J Afr. 2011;22(4):179–81. doi:.
  7. Inatomi J, Matsuoka K, Fujimaru R, Nakagawa A, Iijima K. Mechanisms of development and progression of cyanotic nephropathy. Pediatr Nephrol. 2006;21(10):1440–5. doi:.
  8. Derby CD, Pizarro C. Routine primary repair of tetralogy of Fallot in the neonate. Expert Rev Cardiovasc Ther. 2005;3(5):857–63. doi:.
  9. Kumar RK, Tynan MJ. Catheter interventions for congenital heart disease in third world countries. Pediatr Cardiol. 2005;26(3):241–9. doi:.
  10. Bastuji-Garin S, Sbidian E, Gaudy-Marqueste C, Ferrat E, Roujeau J-C, Richard M-A, et al.; European Dermatology Network (EDEN). Impact of STROBE statement publication on quality of observational study reporting: interrupted time series versus before-after analysis. PLoS One. 2013;8(8):e64733. doi:.
  11. Thygesen K, Alpert JS, Jaffe AS, Chaitman BR, White HD, Joint ESC/ACCF/AHA/WHF Task Force for the Universal Definition of Myocardial Infarction, et al. Third universal definition of myocardial infarction. Circulation, vol. 126, American Heart Association, Inc; 2012, pp. 2020–35. doi:
  12. Kairet K, Deen J, Vernieuwe L, de Bruyn A, Kalantary S, Rodrigus I. Cardioplexol, a new cardioplegic solution for elective CABG. J Cardiothorac Surg. 2013;8(S1):P120. doi:.
  13. Bacha E. Valve-Sparing or Valve Reconstruction Options in Tetralogy of Fallot Surgery. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2017;20:79–83. doi:.
  14. Sen DG, Najjar M, Yimaz B, Levasseur SM, Kalessan B, Quaegebeur JM, et al. Aiming to Preserve Pulmonary Valve Function in Tetralogy of Fallot Repair: Comparing a New Approach to Traditional Management. Pediatr Cardiol. 2016;37(5):818–25. doi:.
  15. Benbrik N, Romefort B, Le Gloan L, Warin K, Hauet Q, Guerin P, et al. Late repair of tetralogy of Fallot during childhood in patients from developing countries. Eur J Cardiothorac Surg. 2015;47(3):e113–7. doi:.
  16. Vaidyanathan B, Radhakrishnan R, Sarala DA, Sundaram KR, Kumar RK. What determines nutritional recovery in malnourished children after correction of congenital heart defects? Pediatrics. 2009;124(2):e294–9. doi:.
  17. Sadiq N, Ullah M, Mahmoud A, Akhtar K, Younis U. Perioperative Major Aortopulmonary Collateral Arteries (MAPCAs) Coiling in Tetralogy of Fallot Patients Undergoing for Total Correction. J Cardiol Curr Res. 2015;3:00123. doi:.
  18. Mosca RS. Tetralogy of Fallot: Total Correction. Oper Tech Thorac Cardiovasc Surg. 2002;7(1):22–8. doi:.
  19. Raj R, Puri GD, Jayant A, Thingnam SKS, Singh RS, Rohit MK. Perioperative echocardiography-derived right ventricle function parameters and early outcomes after tetralogy of Fallot repair in mid-childhood: a single-center, prospective observational study. Echocardiography. 2016;33(11):1710–7. doi:.
  20. Stewart RD, Backer CL, Young L, Mavroudis C. Tetralogy of Fallot: results of a pulmonary valve-sparing strategy. Ann Thorac Surg. 2005;80(4):1431–8, discussion 1438–9. doi:.
  21. Balasubramanya S, Zurakowski D, Borisuk M, Kaza AK, Emani SM, Del Nido PJ, et al. Right ventricular outflow tract reintervention after primary tetralogy of Fallot repair in neonates and young infants. J Thorac Cardiovasc Surg. 2018;155(2):726–34. doi:.
  22. Hirsch JC, Mosca RS, Bove EL. Complete repair of tetralogy of Fallot in the neonate: results in the modern era. Ann Surg. 2000;232(4):508–14. doi:.
  23. Knauth AL, Gauvreau K, Powell AJ, Landzberg MJ, Walsh EP, Lock JE, et al. Ventricular size and function assessed by cardiac MRI predict major adverse clinical outcomes late after tetralogy of Fallot repair. Heart. 2008;94(2):211–6. doi:.
  24. Villafañe J, Feinstein JA, Jenkins KJ, Vincent RN, Walsh EP, Dubin AM, et al.; Adult Congenital and Pediatric Cardiology Section, American College of Cardiology. Hot topics in tetralogy of Fallot. J Am Coll Cardiol. 2013;62(23):2155–66. doi:.
  25. Sachdev MS, Bhagyavathy A, Varghese R, Coelho R, Kumar RS. Right ventricular diastolic function after repair of tetralogy of Fallot. Pediatr Cardiol. 2006;27(2):250–5. doi:.

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