Abstract
Tetralogy of Fallot is one of the most common congenital cardiac malformations and consists of (1) ventricular septal defect, (2) right ventricular outflow tract obstruction, (3) aortic override, and (4) right ventricular hypertrophy. The initial presentation of the patient with Tetralogy of Fallot is variable and dependent on the degree of right ventricular outflow tract obstruction. Initial medical management of Tetralogy of Fallot is aimed at monitoring and managing hypoxemia and preventing hypercyanotic spells. A small percentage of patients will present with significant cyanosis in the neonatal period. The majority, however, will have stable pulmonary artery blood flow and require no immediate treatment. Cyanosis gradually progresses as pulmonary blood flow is limited by increasing right ventricular outflow tract obstruction. Diagnosis is usually established with transthoracic echocardiography. Further anatomic detail and some functional data may also be garnered with cardiac MRI or cardiac catheterization, which may help define and characterize additional sources of pulmonary blood flow or abnormalities of systemic venous return.
Indications for surgical intervention are symptoms of hypercyanotic episodes or the presence of oxygen saturations that are persistently below 75–80 %. In most children with Tetralogy of Fallot, elective complete repair is recommended by 1 year of age with most centers recommending elective repair in the first 3–6 months of life. While palliative procedures can be performed to delay the need for definitive complete repair, most centers favor early complete repair, reserving palliative procedures for those patients that have contraindications to open cardiac surgery. In the current era, outcomes for complete repair are excellent, even when definitive repair is required in the neonatal period.
Morphology and associated defects in tetralogy of Fallot may include tetralogy of Fallot with absent pulmonary valve, tetralogy of Fallot with complete atrioventricular septal defect, and tetralogy of Fallot with pulmonary atresia and multiple aortopulmonary collaterals. These entities may behave differently, and strategy and timing for surgical repair are often more complicated than for simple tetralogy of Fallot. Following repair of tetralogy of Fallot, lifelong follow-up is usually indicated in order to monitor for recurrent or persistent pathology of the right ventricular outflow tract and for appropriate intervention if indicated.
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References
Eldadah ZA, Hamosh A, Biery NJ, Montgomery RA, Duke M, Elkins R, Dietz HC (2001) Familial tetralogy of Fallot caused by mutation in the jagged1 gene. Hum Mol Genet 10:163–169
Goldmuntz E, Geiger E, Benson DW (2001) NKX2.5 mutations in patients with tetralogy of Fallot. Circulation 104:2565–2568
Fallot ÉLA (1988) Contribution à l’anatomie pathologique de la maladie bleue (cyanose cardiaque). n.p.
Blalock A, Taussig HB (1945) The surgical treatment of malformation of the heart in which there is pulmonary stenosis or pulmonary atresia. JAMA 128:189
Potts WJ, Smith S, Gibson S (1946) Anastomosis of the aorta to a pulmonary artery; certain types in congenital heart disease. J Am Med Assoc 132:627–631
Waterston DJ (1962) Treatment of Fallot’s tetralogy in children under 1 year of age. Rozhl Chir 41:181–183
Lillehei CW, Cohen M, Warden HE, Read RC, Aust JB, Dewall RA, Varco RL (1955) Direct vision intracardiac surgical correction of the tetralogy of Fallot, pentalogy of Fallot, and pulmonary atresia defects; report of first ten cases. Ann Surg 142:418–442
Kirklin JW, Dushane JW, Patrick RT, Donald DE, Hetzel PS, Harshbarger HG, Wood EH (1955) Intracardiac surgery with the aid of a mechanical pump-oxygenator system (gibbon type): report of eight cases. Proc Staff Meet Mayo Clin 30:201–206
Bacha EA, Scheule AM, Zurakowski D, Erickson LC, Hung J, Lang P, Mayer JE Jr, del Nido PJ, Jonas RA (2001) Long-term results after early primary repair of tetralogy of Fallot. J Thorac Cardiovasc Surg 122:154–161
Lindberg HL, Saatvedt K, Seem E, Hoel T, Birkeland S (2011) Single-center 50 years’ experience with surgical management of tetralogy of Fallot. Eur J Cardiothorac Surg 40:538–542
Van Praagh R, Van Praagh S, Nebesar RA, Muster AJ, Sinha SN, Paul MH (1970) Tetralogy of Fallot: underdevelopment of the pulmonary infundibulum and its sequelae. Am J Cardiol 26:25–33
Suzuki A, Ho SY, Anderson RH, Deanfield JE (1990) Further morphologic studies on tetralogy of Fallot, with particular emphasis on the prevalence and structure of the membranous flap. J Thorac Cardiovasc Surg 99:528–535
Dickinson DF, Wilkinson JL, Smith A, Hamilton DI, Anderson RH (1982) Variations in the morphology of the ventricular septal defect and disposition of the atrioventricular conduction tissues in tetralogy of Fallot. Thorac Cardiovasc Surg 30:243–249
Kurosawa H, Imai Y (1988) Surgical anatomy of the atrioventricular conduction bundle in tetralogy of Fallot. New findings relevant to the position of the sutures. J Thorac Cardiovasc Surg 95:586–591
Anderson RH, Allwork SP, Ho SY, Lenox CC, Zuberbuhler JR (1981) Surgical anatomy of tetralogy of Fallot. J Thorac Cardiovasc Surg 81:887–896
Vargas FJ, Kreutzer GO, Pedrini M, Capelli H, Rodriguez CA (1986) Tetralogy of Fallot with subarterial ventricular septal defect. Diagnostic and surgical considerations. J Thorac Cardiovasc Surg 92:908–912
Altrichter PM, Olson LJ, Edwards WD, Puga FJ, Danielson GK (1989) Surgical pathology of the pulmonary valve: a study of 116 cases spanning 15 years. Mayo Clin Proc 64:1352–1360
Shimazaki Y, Blackstone EH, Kirklin JW (1984) The natural history of isolated congenital pulmonary valve incompetence: surgical implications. Thorac Cardiovasc Surg 32:257–259
Chiariello L, Meyer J, Wukasch DC, Hallman GL, Cooley DA (1975) Intracardiac repair of tetralogy of Fallot. Five-year review of 403 patients. J Thorac Cardiovasc Surg 70:529–535
Miller RA, Lev M, Paul MH (1962) Congenital absence of the pulmonary valve. The clinical syndrome of tetralogy of Fallot with pulmonary regurgitation. Circulation 26:266–278
Bove EL, Shaher RM, Alley R, McKneally M (1972) Tetralogy of Fallot with absent pulmonary valve and aneurysm of the pulmonary artery: report of two cases presenting as obstructive lung disease. J Pediatr 81:339–343
Humes RA, Driscoll DJ, Danielson GK, Puga FJ (1987) Tetralogy of Fallot with anomalous origin of left anterior descending coronary artery. Surgical options. J Thorac Cardiovasc Surg 94:784–787
Boon AR, Farmer MB, Roberts DF (1972) A family study of Fallot’s tetralogy. J Med Genet 9:179–192
Di Felice V, Zummo G (2009) Tetralogy of Fallot as a model to study cardiac progenitor cell migration and differentiation during heart development. Trends Cardiovasc Med 19:130–135
Kirklin JW, Barratt-Boyes BG (1993) Cardiac surgery: morphology, diagnostic criteria, natural history, techniques, results, and indications. Churchill Livingstone, New York
Groh MA, Meliones JN, Bove EL, Kirklin JW, Blackstone EH, Lupinetti FM, Snider AR, Rosenthal A (1991) Repair of tetralogy of Fallot in infancy. Effect of pulmonary artery size on outcome. Circulation 84:III206–III212
Ramsay JM, Macartney FJ, Haworth SG (1985) Tetralogy of Fallot with major aortopulmonary collateral arteries. Br Heart J 53:167–172
Shimazaki Y, Maehara T, Blackstone EH, Kirklin JW, Bargeron LM Jr (1988) The structure of the pulmonary circulation in tetralogy of Fallot with pulmonary atresia. A quantitative cineangiographic study. J Thorac Cardiovasc Surg 95:1048–1058
McConnell ME (1990) Echocardiography in classical tetralogy of Fallot. Semin Thorac Cardiovasc Surg 2:2–11
Norton KI, Tong C, Glass RB, Nielsen JC (2006) Cardiac MR imaging assessment following tetralogy of Fallot repair. Radiographics 26:197–211
Chung T (2000) Assessment of cardiovascular anatomy in patients with congenital heart disease by magnetic resonance imaging. Pediatr Cardiol 21:18–26
Garson A Jr, Gillette PC, McNamara DG (1981) Propranolol: the preferred palliation for tetralogy of Fallot. Am J Cardiol 47:1098–1104
Castaneda AR, Freed MD, Williams RG, Norwood WI (1977) Repair of tetralogy of Fallot in infancy. Early and late results. J Thorac Cardiovasc Surg 74:372–381
Castaneda AR (1990) Classical repair of tetralogy of Fallot: timing, technique, and results. Semin Thorac Cardiovasc Surg 2:70–75
Di Donato RM, Jonas RA, Lang P, Rome JJ, Mayer JE Jr, Castaneda AR (1991) Neonatal repair of tetralogy of Fallot with and without pulmonary atresia. J Thorac Cardiovasc Surg 101:126–137
Gustafson RA, Murray GF, Warden HE, Hill RC, Rozar GE Jr (1988) Early primary repair of tetralogy of Fallot. Ann Thorac Surg 45:235–241
Karl TR, Sano S, Pornviliwan S, Mee RB (1992) Tetralogy of Fallot: favorable outcome of nonneonatal transatrial, transpulmonary repair. Ann Thorac Surg 54:903–907
Kirklin JK, Kirklin JW, Blackstone EH, Milano A, Pacifico AD (1989) Effect of transannular patching on outcome after repair of tetralogy of Fallot. Ann Thorac Surg 48:783–791
Kirklin JW, Blackstone EH, Jonas RA, Shimazaki Y, Kirklin JK, Mayer JE Jr, Pacifico AD, Castaneda AR (1992) Morphologic and surgical determinants of outcome events after repair of tetralogy of Fallot and pulmonary stenosis. A two-institution study. J Thorac Cardiovasc Surg 103:706–723
Kirklin JW, Blackstone EH, Kirklin JK, Pacifico AD, Aramendi J, Bargeron LM Jr (1983) Surgical results and protocols in the spectrum of tetralogy of Fallot. Ann Surg 198:251–265
Hennein HA, Mosca RS, Urcelay G, Crowley DC, Bove EL (1995) Intermediate results after complete repair of tetralogy of Fallot in neonates. J Thorac Cardiovasc Surg 109:332–342, 344, discussion 342–333
Remadevi KS, Vaidyanathan B, Francis E, Kannan BR, Kumar RK (2008) Balloon pulmonary valvotomy as interim palliation for symptomatic young infants with tetralogy of Fallot. Ann Pediatr Cardiol 1:2–7
Di Benedetto G, Tiraboschi R, Vanini V, Annecchino P, Aiazzi L, Caprioli C, Parenzan L (1981) Systemic-pulmonary artery shunt using PTFE prosthesis (Gore-Tex). Early results and long-term follow-up on 105 consecutive cases. Thorac Cardiovasc Surg 29:143–147
Bove EL, Kohman L, Sereika S, Byrum CJ, Kavey RE, Blackman MS, Sondheimer HM, Rosenthal A (1987) The modified Blalock-Taussig shunt: analysis of adequacy and duration of palliation. Circulation 76:III19–III23
McKay R, de Leval MR, Rees P, Taylor JF, Macartney FJ, Stark J (1980) Postoperative angiographic assessment of modified Blalock-Taussig shunts using expanded polytetrafluoroethylene (Gore-Tex). Ann Thorac Surg 30:137–145
Pacifico AD, Sand ME, Bargeron LM Jr, Colvin EC (1987) Transatrial-transpulmonary repair of tetralogy of Fallot. J Thorac Cardiovasc Surg 93:919–924
Binet JP (1988) Correction of tetralogy of Fallot with combined transatrial and pulmonary approach: experience with 184 consecutive cases. J Card Surg 3:97–102
Edmunds LH Jr, Saxena NC, Friedman S, Rashkind WJ, Dodd PF (1976) Transatrial repair of tetralogy of Fallot. Surgery 80:681–688
Dietl CA, Torres AR, Cazzaniga ME, Favaloro RG (1989) Right atrial approach for surgical correction of tetralogy of Fallot. Ann Thorac Surg 47:546–551; discussion 551–542
Pacifico AD, Kirklin JK, Colvin EV, McConnell ME, Kirklin JW (1990) Transatrial-transpulmonary repair of tetralogy of Fallot. Semin Thorac Cardiovasc Surg 2:76–82
Coles JG, Kirklin JW, Pacifico AD, Kirklin JK, Blackstone EH (1988) The relief of pulmonary stenosis by a transatrial versus a transventricular approach to the repair of tetralogy of Fallot. Ann Thorac Surg 45:7–10
Vida VL, Padalino MA, Maschietto N, Biffanti R, Anderson RH, Milanesi O, Stellin G (2012) The balloon dilation of the pulmonary valve during early repair of tetralogy of Fallot. Catheter Cardiovasc Interv 80:915–921
Blackstone EH, Kirklin JW, Pacifico AD (1979) Decision-making in repair of tetralogy of Fallot based on intraoperative measurements of pulmonary arterial outflow tract. J Thorac Cardiovasc Surg 77:526–532
Naito Y, Fujita T, Manabe H, Kawashima Y (1980) The criteria for reconstruction of right ventricular outflow tract in total correction of tetralogy of Fallot. J Thorac Cardiovasc Surg 80:574–581
Pacifico AD, Kirklin JW, Blackstone EH (1977) Surgical management of pulmonary stenosis in tetralogy of Fallot. J Thorac Cardiovasc Surg 74:382–395
Kavey RE, Bove EL, Byrum CJ, Blackman MS, Sondheimer HM (1987) Postoperative functional assessment of a modified surgical approach to repair of tetralogy of Fallot. J Thorac Cardiovasc Surg 93:533–538
Shanley CJ, Lupinetti FM, Shah NL, Beekman RH 3rd, Crowley DC, Bove EL (1993) Primary unifocalization for the absence of intrapericardial pulmonary arteries in the neonate. J Thorac Cardiovasc Surg 106:237–247
Shimazaki Y, Blackstone EH, Kirklin JW, Jonas RA, Mandell V, Colvin EV (1992) The dimensions of the right ventricular outflow tract and pulmonary arteries in tetralogy of Fallot and pulmonary stenosis. J Thorac Cardiovasc Surg 103:692–705
Nakata S, Imai Y, Takanashi Y, Kurosawa H, Tezuka K, Nakazawa M, Ando M, Takao A (1984) 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 88:610–619
Pagani FD, Cheatham JP, Beekman RH 3rd, Lloyd TR, Mosca RS, Bove EL (1995) The management of tetralogy of Fallot with pulmonary atresia and diminutive pulmonary arteries. J Thorac Cardiovasc Surg 110:1521–1532; discussion 1532–1523
Kirklin JW, Blackstone EH, Shimazaki Y, Maehara T, Pacifico AD, Kirklin JK, Bargeron LM Jr (1988) Survival, functional status, and reoperations after repair of tetralogy of Fallot with pulmonary atresia. J Thorac Cardiovasc Surg 96:102–116
Clarke DR, Campbell DN, Pappas G (1989) Pulmonary allograft conduit repair of tetralogy of Fallot. An alternative to transannular patch repair. J Thorac Cardiovasc Surg 98:730–736; discussion 736–737
Snir E, de Leval MR, Elliott MJ, Stark J (1991) Current surgical technique to repair Fallot’s tetralogy with absent pulmonary valve syndrome. Ann Thorac Surg 51:979–982
Hraska V (2005) Repair of tetralogy of Fallot with absent pulmonary valve using a new approach. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 8:132–134
Hraska V, Kantorova A, Kunovsky P, Haviar D (2002) Intermediate results with correction of tetralogy of Fallot with absent pulmonary valve using a new approach. Eur J Cardiothorac Surg 21:711–714; discussion 714–715
McDonnell BE, Raff GW, Gaynor JW, Rychik J, Godinez RI, DeCampli WM, Spray TL (1999) Outcome after repair of tetralogy of Fallot with absent pulmonary valve. Ann Thorac Surg 67:1391–1395; discussion 1395–1396
Ilbawi M, Cua C, DeLeon S, Muster A, Paul M, Cutilletta A, Arcilla R, Idriss F (1990) Repair of complete atrioventricular septal defect with tetralogy of Fallot. Ann Thorac Surg 50:407–412
Oechslin EN, Harrison DA, Harris L, Downar E, Webb GD, Siu SS, Williams WG (1999) Reoperation in adults with repair of tetralogy of Fallot: indications and outcomes. J Thorac Cardiovasc Surg 118:245–251
Hoffman TM, Wernovsky G, Atz AM, Kulik TJ, Nelson DP, Chang AC, Bailey JM, Akbary A, Kocsis JF, Kaczmarek R, Spray TL, Wessel DL (2003) Efficacy and safety of milrinone in preventing low cardiac output syndrome in infants and children after corrective surgery for congenital heart disease. Circulation 107:996–1002
Cullen S, Shore D, Redington A (1995) Characterization of right ventricular diastolic performance after complete repair of tetralogy of Fallot. Restrictive physiology predicts slow postoperative recovery. Circulation 91:1782–1789
Rajagopal SK, Thiagarajan RR (2011) Perioperative care of children with tetralogy of Fallot. Curr Treat Options Cardiovasc Med 13:464–474
Plumpton K, Justo R, Haas N (2005) Amiodarone for post-operative junctional ectopic tachycardia. Cardiol Young 15:13–18
Hoffman TM, Bush DM, Wernovsky G, Cohen MI, Wieand TS, Gaynor JW, Spray TL, Rhodes LA (2002) Postoperative junctional ectopic tachycardia in children: incidence, risk factors, and treatment. Ann Thorac Surg 74:1607–1611
Manrique AM, Arroyo M, Lin Y, El Khoudary SR, Colvin E, Lichtenstein S, Chrysostomou C, Orr R, Jooste E, Davis P, Wearden P, Morell V, Munoz R (2010) Magnesium supplementation during cardiopulmonary bypass to prevent junctional ectopic tachycardia after pediatric cardiac surgery: a randomized controlled study. J Thorac Cardiovasc Surg 139:162–169 e162
Hirsch JC, Mosca RS, Bove EL (2000) Complete repair of tetralogy of Fallot in the neonate: results in the modern era. Ann Surg 232:508–514
Park CS, Lee JR, Lim HG, Kim WH, Kim YJ (2010) The long-term result of total repair for tetralogy of Fallot. Eur J Cardiothorac Surg 38:311–317
Knott-Craig CJ, Elkins RC, Lane MM, Holz J, McCue C, Ward KE (1998) A 26-year experience with surgical management of tetralogy of Fallot: risk analysis for mortality or late reintervention. Ann Thorac Surg 66:506–511
Murphy JG, Gersh BJ, Mair DD, Fuster V, McGoon MD, Ilstrup DM, McGoon DC, Kirklin JW, Danielson GK (1993) Long-term outcome in patients undergoing surgical repair of tetralogy of Fallot. N Engl J Med 329:593–599
Nollert G, Fischlein T, Bouterwek S, Bohmer C, Klinner W, Reichart B (1997) Long-term survival in patients with repair of tetralogy of Fallot: 36-year follow-up of 490 survivors of the first year after surgical repair. J Am Coll Cardiol 30:1374–1383
Bove EL, Byrum CJ, Thomas FD, Kavey RE, Sondheimer HM, Blackman MS, Parker FB Jr (1983) The influence of pulmonary insufficiency on ventricular function following repair of tetralogy of Fallot. Evaluation using radionuclide ventriculography. J Thorac Cardiovasc Surg 85:691–696
Burchill LJ, Wald RM, Harris L, Colman JM, Silversides CK (2011) Pulmonary valve replacement in adults with repaired tetralogy of Fallot. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 14:92–97
Therrien J, Siu SC, McLaughlin PR, Liu PP, Williams WG, Webb GD (2000) Pulmonary valve replacement in adults late after repair of tetralogy of Fallot: are we operating too late? J Am Coll Cardiol 36:1670–1675
Frigiola A, Tsang V, Bull C, Coats L, Khambadkone S, Derrick G, Mist B, Walker F, van Doorn C, Bonhoeffer P, Taylor AM (2008) Biventricular response after pulmonary valve replacement for right ventricular outflow tract dysfunction: is age a predictor of outcome? Circulation 118:S182–S190
Ilbawi MN, Idriss FS, DeLeon SY, Muster AJ, Gidding SS, Berry TE, Paul MH (1987) Factors that exaggerate the deleterious effects of pulmonary insufficiency on the right ventricle after tetralogy repair. Surgical implications. J Thorac Cardiovasc Surg 93:36–44
Yemets IM, Williams WG, Webb GD, Harrison DA, McLaughlin PR, Trusler GA, Coles JG, Rebeyka IM, Freedom RM (1997) Pulmonary valve replacement late after repair of tetralogy of Fallot. Ann Thorac Surg 64:526–530
Knauth AL, Gauvreau K, Powell AJ, Landzberg MJ, Walsh EP, Lock JE, del Nido PJ, Geva T (2008) Ventricular size and function assessed by cardiac MRI predict major adverse clinical outcomes late after tetralogy of Fallot repair. Heart 94:211–216
Lurz P, Bonhoeffer P, Taylor AM (2009) Percutaneous pulmonary valve implantation: an update. Expert Rev Cardiovasc Ther 7:823–833
McElhinney DB, Hellenbrand WE, Zahn EM, Jones TK, Cheatham JP, Lock JE, Vincent JA (2010) Short- and medium-term outcomes after transcatheter pulmonary valve placement in the expanded multicenter us melody valve trial. Circulation 122:507–516
Hirsch JC, Bove EL (2003) Tetralogy of Fallot. In: Mavroudis C, Backer CL (eds) Pediatric cardiac surgery, 3rd edn. Mosby, Philadelphia
Geva T (2011) Repaired tetralogy of Fallot: the roles of cardiovascular magnetic resonance in evaluating pathophysiology and for pulmonary replacement decision support. J Cardiov Magn Reson 13(9)
Ammash NM, Dearani JA, Burkhart HM et al. (2007) Pulmonary regurgitation after tetralogy of Fallot repair: clinical features, sequelae, and timing of pulmonary valve replacement. Congenital Heart Dis 2(6):386–403
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Nelson, J.S., Bove, E.L., Hirsch-Romano, J.C. (2014). Tetralogy of Fallot. 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_18
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