• Muhammad Kamran Younis Memon Aga Khan University Hospital
  • Saleem Akhtar Aga Khan University
  • Muhammad Mohsin Aga Khan University
  • Waris Ahmad Aga Khan University
  • Aleena Arshad Ziauddin University
  • Mehnaz Atiq Ahmed Liaquat National Hospital


Background:   Primary repair of ToF between 3–12 months is the preferred mode of treatment worldwide, with low surgical mortality. This study reviews our experience of ToF repair in infancy and its short and midterm outcomes in a single centre from a developing country. Methods:  Data of all patients with Tetralogy of Fallot repair during infancy from January 2007 to Feb 2018 was reviewed. Preoperative, operative, and postoperative data was analysed. Outcome of the infants was assessed through discharge/death, low cardiac output syndrome (LCOS), prolonged intubation, duration of cardiac intensive care unit (CICU) and hospital stay.  Results:  Forty-four patients who underwent TOF repair in infancy during this period were included. The mean age and weight were 9.39±2.32 and 7.20±1.30 respectively, 77.3% (34 patients) were male, 68.18% (30 patients) had saturation >75%. Mean intubation period was 4.05±6.58 days, 12 (27.3%) patients developed LCOS, mean cardiopulmonary bypass (CPB) time, aortic cross clamp (ACC) time and ionotropic score were 133.52±62.4, 98.66±58.62 and 33.27±71.13 respectively. Mean CICU and hospital stay was 6.60±7.18 and 12.05±7.74 respectively. Five (11.3%) patients expired in postoperative period. Baseline saturation ≤75% is independent risk factor for LCOS and prolong intubation period. In the last six years our mortality decreased to 8% from 15.7% during the previous six years, while our mean intubation duration, CPB time, ACC, hospital stay and CICU stay have all shown improvement. Conclusion: TOF repair during infancy is safe procedure in expert hands with acceptable morbidity and mortality. Baseline saturation ≤75% is independent risk factor for LCOS and prolonged intubation period. Last six years have shown considerable improvement in our surgical morbidity and mortality due to improvement in surgical expertise.Keywords: Tetralogy of Fallot repair; Low cardiac output syndrome; Congenital heart disease surgery; Operative mortality ToF

Author Biographies

Muhammad Kamran Younis Memon, Aga Khan University Hospital

Fellow Pediatric Cardiology, Department of pediatrics and Child health

Saleem Akhtar, Aga Khan University

Assistant professor, Pediatrics and Child Health, Aga Khan University

Muhammad Mohsin, Aga Khan University

Fellow Pediatric Cardiology, Department of pediatrics and Child health

Waris Ahmad, Aga Khan University

Assistant professor, Pediatrics and Child Health, Aga Khan University

Aleena Arshad, Ziauddin University

Medical Student, Ziauddin University

Mehnaz Atiq Ahmed, Liaquat National Hospital

Professor, Department of Pediatrics, Liaquat National Hospital


Jacobs ML, Jacobs JP. The early history of surgery for patients with tetralogy of Fallot. Cardiol Young 2008;18(Suppl 3):8–11.

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.

Senthilnathan S, Dragulescu A, Mertens L. Pulmonary regurgitation after tetralogy of fallot repair: a diagnostic and therapeutic challenge. J Cardiovasc Echogr 2013;23(1):1–9.

Baumgartner H, Hung J, Bermejo J, Chambers JB, Evangelista A, Griffin BP, et al. Echocardiographic assessment of valve stenosis: EAE/ASE recommendations for clinical practice. J Am Soc Echocardiogr 2009;22(1):1–23.

Haque A, Siddiqui NR, Munir O, Saleem S, Mian A. Association between vasoactive-inotropic score and mortality in pediatric septic shock. Indian Pediatr 2015;52(4):311–3.

Gaies MG, Gurney JG, Yen AH, Napoli ML, Gajarski RJ, Ohye RG, et al. Vasoactive–inotropic score as a predictor of morbidity and mortality in infants after cardiopulmonary bypass. Pediatr Crit Care Med 2010;11(2):234–8.

Rękawek J, Kansy A, Miszczak-Knecht M, Manowska M, Bieganowska K, Brzezinska-Paszke M, et al. Risk factors for cardiac arrhythmias in children with congenital heart disease after surgical intervention in the early postoperative period. J Thorac Cardiovasc Surg 2007;133(4):900–4.

Chandler HK, Kirsch R. Management of the low cardiac output syndrome following surgery for congenital heart disease. Curr Cardiol Rev 2016;12(2):107–11.

Egbe AC, Uppu SC, Mittnacht AJ, Joashi U, Ho D, Nguyen K, et al. Primary tetralogy of Fallot repair: predictors of intensive care unit morbidity. Asian Cardiovasc Thorac Ann 2014;22(7):794–9.

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.

Saşmazel A, Fedakar A, Baysal A, Çalışkan A, Buğra O, Sunar H. Determinants of short-term mortality and morbidity after the complete repair of tetralogy of Fallot in infant groups under 12 months and one-four years of age. Anadolu Kardiyol Derg 2010;10(6):544–9.

Kaushal SK, Iyer KS, Sharma R, Airan B, Bhan A, Das B, et al. Surgical experience with total correction of tetralogy of Fallot in infancy. Int J Cardiol 1996;56(1):35–40.

Uva MS, 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(5):1291–300.

Apitz C, Webb GD, Redington AN. Tetralogy of fallot. Lancet 2009;374(9699):1462–71.

Knott-Craig CJ, Elkins RC, Lane MM, Holz J, McCue C, Ward KE. A 26-year experience with surgical management of tetralogy of Fallot: risk analysis for mortality or late reintervention. Ann Thorac Surg 1998;66(2):506–10.

Andreasen JB, Johnsen SP, Ravn HB. Junctional ectopic tachycardia after surgery for congenital heart disease in children. Intensive Care Med 2008;34(5):895–902.

Batra AS, Mohari N. Junctional ectopic tachycardia: current strategies for diagnosis and management. Prog Pediatr Cardiol 2013;35(1):49–54.

Dodge-Khatami A, Miller O, Anderson RH, Gil-Jaurena JM, Goldman AP, De Leval M. Impact of junctional ectopic tachycardia on postoperative morbidity following repair of congenital heart defects. Eur J Cardiothorac Surg 2002;21(2):255–9.

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(3):394–9.

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.

Agarwal HS, Wolfram KB, Saville BR, Donahue BS, Bichell DP. Postoperative complications and association with outcomes in pediatric cardiac surgery. J Thorac Cardiovasc Surg 2014;148(2):609–16.

Bouzas B, Kilner PJ, Gatzoulis MA. Pulmonary regurgitation: not a benign lesion. Eur Heart J 2005;26(5):433–9.




Most read articles by the same author(s)