Total Correction of Tetralogy of Fallot in the First 60 Days of Life in Symptomatic Infants: Is It The Gold Standard?

Abstract

Background The timing of surgical repair of tetralogy of Fallot (TOF) is a key to alleviate complications and for long-term survival. Total correction was usually performed at the age of 6 months or older under the notion of decreasing the surgical risk. However, avoiding palliation with an aortopulmonary shunt and early correction of systemic hypoxia appear to be of more benefit than the inborn surgical risk in low body weight patients. Our objective was to assess early/midterm survival and operative complications and to analyze patients, surgical techniques, and morphological risk factors to determine their effects on outcomes.

Patients and Methods We retrospectively reviewed 152 patients with TOF who were ≤60 days of age when they underwent total correction of TOF. All patients had either duct-dependent pulmonary blood flow or arterial blood oxygen saturation less than 65% on room air requiring urgent surgical correction. Exclusion criteria included TOF with pulmonary atresia, TOF with nonconfluent pulmonary arteries, TOF with multiple aortopulmonary collateral arteries, and associated complete atrioventricular septal defects.

Results The mean age at repair was 34 ± 19 days, and the mean weight was 3.8 ± 0.9 kg. Before surgery, 96 patients received an infusion of prostaglandin, 45 were mechanically ventilated, and 32 required inotropic support. Right ventricular outflow tract obstruction was managed with a transannular patch in 112 patients, and all the others had a main pulmonary artery patch. Cardiopulmonary bypass (CPB) with moderate hypothermia was the standard, and the CPB time averaged 48 ± 21 minutes. The postoperative intensive care unit stay was 5.7 ± 6 days, with 2.8 ± 4 days of mechanical ventilation. Early mortality was 4.6% (7 of 152), and actuarial survival rates were 95% at 1 year and 92% at 5 years. Univariable and multivariable analyses of the patients' demographics, anatomical characteristics, and operative techniques revealed the presence of small pulmonary arteries and low body weight to be the only independent risk factors for death.

Conclusion Early total correction of TOF during the first 60 days of life can be performed with low mortality and good intermediate-term survival and, from our point of view, “should be the gold standard for TOFs.”

Keywords

correction of TOF - early age - congenital heart diseases

Disclosure

None.

• References

• 1 Fallot E. Contribution a l'anatomie pathologique de la maladie bleue (cyanose cardiaque). Mars Med 1888; 25: 77
• 2 Blalock A, Taussig HB. The surgical treatment of malformation of the heart in which there is pulmonary stenosis or pulmonary atresia. JAMA 1945; 128: 189
• 3 Waterston DJ. Treatment of Fallot's tetralogy in children under 1 year of age [in Czech]. Rozhl Chir 1962; 41: 181-183
• 4 Potts WJ, Smith S, Gibson S. Anastomosis of the aorta to a pulmonary artery; certain types in congenital heart disease. J Am Med Assoc 1946; 132 (11) 627-631
• 5 Lillehei CW, Cohen M, Warden HE. , 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 (03) 418-442
• 6 Kirklin JW, Dushane JW, Patrick RT. , 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 (10) 201-206
• 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 (03) 372-381
• 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 (04) 406-411
• 9 Di Donato RM, Jonas RA, Lang P, Rome JJ, Mayer Jr JE, Castaneda AR. Neonatal repair of tetralogy of Fallot with and without pulmonary atresia. J Thorac Cardiovasc Surg 1991; 101 (01) 126-137
• 10 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 (06) 1928-1935
• 11 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 (06) 1928-1934 , discussion 1934–1935
• 12 Sousa Uva M, Chardigny C, Galetti L. , et al. Surgery for tetralogy of Fallot at less than six months of age. Is palliation “old-fashioned”?. Eur J Cardiothorac Surg 1995; 9 (08) 453-459 , discussion 459–460
• 13 Pozzi M, Trivedi DB, Kitchiner D, Arnold RA. Tetralogy of Fallot: what operation, at which age. Eur J Cardiothorac Surg 2000; 17 (06) 631-636
• 14 Munkhammar P, Cullen S, Jögi P, de Leval M, Elliott M, Norgård G. Early age at repair prevents restrictive right ventricular (RV) physiology after surgery for tetralogy of Fallot (TOF): diastolic RV function after TOF repair in infancy. J Am Coll Cardiol 1998; 32 (04) 1083-1087
• 15 Castaneda AR, Lamberti J, Sade RM, Williams RG, Nadas AS. Open-heart surgery during the first three months of life. J Thorac Cardiovasc Surg 1974; 68 (05) 719-731
• 16 Kirklin JW, Blackstone EH, Jonas RA. , 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 (04) 706-723
• 17 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-S596
• 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 (07) 2279-2283
• 19 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-II161
• 20 Alassal M, Ibrahim BM, Elrakhawy HM. , et al. Total correction of tetralogy of Fallot at early age: a study of 183 cases. Heart Lung Circ 2018; 27 (02) 248-253
• 21 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 (03) 394-399
• 22 Kanter KR, Kogon BE, Kirshbom PM, Carlock PR. Symptomatic neonatal tetralogy of Fallot: repair or shunt?. Ann Thorac Surg 2010; 89 (03) 858-863
• 23 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 (02) 506-511
• 24 Al Habib HF, Jacobs JP, Mavroudis C. , et al. Contemporary patterns of management of tetralogy of Fallot: data from the Society of Thoracic Surgeons Database. Ann Thorac Surg 2010; 90 (03) 813-819 , discussion 819–820
• 25 Hirsch JC, Mosca RS, Bove EL. Complete repair of tetralogy of Fallot in the neonate: results in the modern era. Ann Surg 2000; 232 (04) 508-514
• 26 Seliem MA, Wu YT, Glenwright K. Relation between age at surgery and regression of right ventricular hypertrophy in tetralogy of Fallot. Pediatr Cardiol 1995; 16 (02) 53-55
• 27 Turley K, Mavroudis C, Ebert PA. Repair of congenital cardiac lesions during the first week of life. Circulation 1982; 66 (2 Pt 2, 2pt2): I214-I219
• 28 Hennein HA, Mosca RS, Urcelay G, Crowley DC, Bove EL. Intermediate results after complete repair of tetralogy of Fallot in neonates. J Thorac Cardiovasc Surg 1995; 109 (02) 332-342 , 344, discussion 342–343
• 29 Shanley CJ, Lupinetti FM, Shah NL, Beekman III RH, Crowley DC, Bove EL. Primary unifocalization for the absence of intrapericardial pulmonary arteries in the neonate. J Thorac Cardiovasc Surg 1993; 106 (02) 237-247
• 30 Lee C-H, Kwak JG, Lee C. Primary repair of symptomatic neonates with tetralogy of Fallot with or without pulmonary atresia. Korean J Pediatr 2014; 57 (01) 19-25
• 31 Ungerleider RM, Kanter RJ, O'Laughlin M. , et al. Effect of repair strategy on hospital cost for infants with tetralogy of Fallot. Ann Surg 1997; 225 (06) 779-783 , discussion 783–784