Abstract
As a knowledge base, congenital heart disease has only recently evolved within the expertise of the pediatric or cardiac anesthesiologist as pediatric cardiac anesthesiology has become recognized as a discrete subspecialty (DiNardo et al. 2010). Historical attempts to “simplify” the cardiac malformations that may be encountered typically ignored specific anatomic details in favor of slotting the lesions into broad categories such as “cyanotic” or “noncyanotic.” Early textbooks on anesthesia for the congenital cardiac patient often divided cardiac anomalies in very general ways, pointing out specific memorable features associated with some lesions (Lake). Although useful in the era where most anesthesiologists did not have significant insight into this subspecialty of pediatric disease, rapid advances in the successful surgical treatment of congenital cardiac disease have been paralleled by accumulation of specific cognitive and technical skills by anesthesiologists dedicated to the care of such patients (DiNardo et al. 2010). Contemporary pediatric cardiac anesthesiologists share knowledge of congenital cardiac disease, echocardiography, and the physiologic challenges of catheterization lab procedures with the pediatric cardiologist; the details of surgical technique and management of cardiopulmonary bypass with the cardiac surgeon; and the management and expectations of preoperative care and postoperative convalescence (including the management of extracorporeal membrane oxygenation) with the pediatric cardiac intensive care physician. The assessment of a patient with congenital heart disease by the contemporary pediatric cardiac anesthesiologist is a complex synthesis of the anatomic details of a specific cardiac lesion, their implications on the baseline and expected physiology of the patient, the patient’s status within the natural history of the lesion, consideration of how anesthesia techniques and the operative environment will predictably intrude on the homeostasis of the patient’s cardiac physiology, and how those risks might be tolerated and minimized. The author’s goal for the next two chapters is not to present an encyclopedic review of the material with inarguable advice on anesthesia technique and management. Instead a contextual presentation including embryology and anatomy, physiology correlated with the anatomy, medical and surgical management, and the natural history of the disease is presented with the assumption that a well-informed practitioner can apply their enhanced knowledge of the cardiac disease in making informed choices regarding anesthesia management.
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Alexiou C, Mahmoud H, Al-khaddour A, Gnanapragasam J, Salmon A, Keeton B, Monro J. Outcome after repair of tetralogy of fallot in the first year of life. Ann Thorac Surg. 2001;71(2):494–500.
Allen H, Driscoll D, Shaddy R, Feltes T. Moss & Adams’ heart disease in infants, children, and adolescents: including the fetus and young adult. Lippincott Williams and Wilkins 8th ed. 2013, p. 969–89.
Alsoufi B, Mori M, McCracken C, Williams E, Samai C, Kogon B, Kanter K. Results of primary repair versus shunt palliation in ductal dependent infants with pulmonary atresia and ventricular septal defect. Ann Thorac Surg. 2015;100(2):639–46.
Anderson R, Weinberg P. The clinical anatomy of tetralogy of fallot. Cardiol Young. 2005;15:38–47.
Andreasen J, Jonhsen S, Ravn H. Junctional ectopic tachycardiac after surgery for congenital heart disease in children. Intensive Care Med. 2008;34:895–902.
Babu-Narayan S, Diller G, Gheta R, Bastin A, Karonis T, Li W, Shore D. Clinical outcomes of surgical pulmonary valve replacement after repair of tetralogy of fallot and potential prognostic value of preoperative cardiopulmonary exercise testing. Circulation. 2014;129(1):18–27.
Bacha E. Valve-sparing options in tetralogy of fallot surgery. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2012;15(1):24–6.
Bahnson H. Surgical treatment of pulmonary stenosis: a retrospection. Ann Thorac Surg. 1982;33:96–8.
Barker GM, Affolter J, Saenz J, Cox CS, Forbess JM, Scott WA, Zeltser I. Temporary atrial pacing for cardiac output after pediatric cardiac surgery. Pediatr Cardiol. 2013;34(7):1605–11.
Barron DJ. Tetralogy of fallot: controversies in early management. World J Pediatr Congenital Heart Surg. 2013;4(2):186–91.
Bartelings M, Gittenberger-de Groot A. Morphogenetic considerations on congenital malformations of the outflow tract. Part 1: common arterial trunk and tetralogy of fallot. Int J Cardiol. 1991;32(2):213–30.
Batra A, Starnes V, Wells W. Does the site of insertion of a systemic-pulmonary shunt influence growth of the pulmonary arteries? Ann Thorac Surg. 2005;79(2):636–40.
Blalock A, Taussig H. The surgical treatment of malformations of the heart in which there is pulmonary stenosis or pulmonary atresia. JAMA. 1945;128:189–92.
Borowski A, Ghodsizad A, Litmathe J, Lawrenz W, Schmidt K, Gams E. Severe pulmonary regurgitation late after total repair of tetralogy of fallot : surgical considerations. Pediatr Cardiol. 2004;25(5):466–71.
Britt J, Moffett B, Bronicki R, Checchia P. Incidence of adverse events requiring intervention after initiation of oral beta-blocker in pediatric cardiac intensive care patients. Pediatr Cardiol. 2014;35(6):1062–6.
Burchill L, Wald R, Harris L, Colman J, Silversides C. Pulmonary valve replacement in adults with repaired tetralogy of fallot. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2011;14(1):92–7.
Bushman G. Essentials of nitric oxide for the pediatric (cardiac) anesthesiologist. Semin Cardiothorac Vasc Anes. 2001;5(1):79–90.
Cheatham J, Hellenbrand W, Zahn E, Jones T, Berman D, Vincent J, McElhinney D. Clinical and hemodynamic outcomes up to 7 years after transcatheter pulmonary valve replacement in the US melody valve investigational device exemption trial. Circulation. 2015;131(22):1960–70.
Cheng J, Russell H, Stewart R, Thomas J, Backer C, Mavroudis C. The role of tricuspid valve surgery in the late management of tetralogy of fallot: collective review. World J Pediatr Congenital Heart Surg. 2012;3(4):492–8.
Chiappini B, Barrea C, Rubay J.Right ventricular outflow tractreconstruction with contegra monocuspid patch in tetralogy of fallot.Ann Thorac Surg. 2007;83(1):185–7.
Cuypers J, Menting E, Konings E, Opić P, et al. The unnatural history of tetralogy of fallot: prospective follow-up of 40 years after surgical correction. Circulation. 2014;130(22):1944–54.
Dabizzi RP, Teodori G, Barletta GA, Caprioli G, Baldrighi G, Baldrighi V. Associated coronary and cardiac anomalies in the tetralogy of fallot. An angiographic study. Eur Heart J. 1990;8:692–704.
DiNardo J, Andropoulos D, Baum V. Special article: a proposal for training in pediatric cardiac anesthesia. Anesth Analg. 2010;110(4):1121–5.
Dyamenahalli U, Mccrindle B, Barker G, Williams W, Freedom R, Bohn D. Influence of perioperative factors on outcomes in children younger than 18 months after repair of tetralogy of fallot. Ann Thorac Surg. 2000;2000(69):1236–42.
Fogel M, Sundareswaran K, de Zelicourt D, Dasi L, Pawlowski T, Rome J, Yoganathan A. Power loss and right ventricular efficiency in patients after tetralogy of fallot repair with pulmonary insufficiency: clinical implications. J Thorac Cardiovasc Surg. 2012;143:1279–85.
Fraser C. The ongoing quest for an ideal surgical repair for tetralogy of fallot: focus on the pulmonary valve. J Thorac Cardiovasc Surg. 2015;149(5):1364.
Fraser CD, Mckenzie ED, Cooley DA. Tetralogy of fallot : surgical management individualized to the patient. Ann Thorac Surg. 2001;71(5):1556–61.
Garson A, Nihill M, McNamara D, Cooley D. Status of the adult and adolescent after repair of tetralogy of fallot. Circulation. 1979;59:1232–40.
Geva T. Indications and timing of pulmonary valve replacement after tetralogy of fallot repair. Semin Thorac Cardiovasc Surg. 2006;9(1):11–22.
Geva T, Ayres N, Pac F, Pignatelli R. Quantitative morphometric analysis of progressive infundibular obstruction in tetralogy of fallot: a prospective longitudinal echocardiographic study. Circulation. 1995;92:886–92.
Gonzalez J, Shirali G, Atz A, Taylor S, Forbus G, Zyblewski S, Hlavacek A. Universal screening for extracardiac abnormalities in neonates with congenital heart disease. Pediatr Cardiol. 2009;30(3):269–73.
Greeley W, Bushman G, Davis D, Reves J. Comparative effects of halothane and ketamine on systemic oxygen saturation in children with cyanotic heart disease. Anesthesiology. 1986;65:666.
Hallbergson A, Gauvreau K, Powell A. Right ventricular remodeling after pulmonary valve replacement : early gains, late losses. Ann Thorac Surg. 2014;99(2):660–6.
Hansen P, Slane P, Rueckert P, Clark S. Squatting revisited: comparison of haemodynamic responses in normal individuals and heart transplantation recipients. Br Heart J. 1995;74(2):154–8.
Hirsch J, Mosca R, Bove E. Complete repair of tetralogy of fallot in the neonate: results in the modern era. Ann Surg. 2000;232(4):508–14.
Hoohenkerk G, Schoof P, Bruggemans E, Rijlaarsdam M, Hazekamp M. 28 years’ experience with transatrial-transpulmonary repair of atrioventricular septal defect with tetralogy of fallot. Ann Thorac Surg. 2008;85(5):1686–9.
Hraska V. Repair of tetralogy of fallot with absent pulmonary valve using a new approach. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2005;8:132–4.
Humpl T, Söderberg B, Mccrindle BW, Nykanen DG, Freedom RM, Williams WG, Benson LN. Percutaneous balloon valvotomy in pulmonary atresia with intact ventricular septum. Circulation. 2003;108:826–32.
Karadeniz C, Atalay S, Demir F, Tutar E, et al. Does surgically induced right bundle branch block really effect ventricular function in children after ventricular septal defect closure? Pediatr Cardiol. 2014;36:481–8.
Karl T. Tetralogy of fallot: current surgical perspective. Ann Pediatr Cardiol. 2008;1(2):93–100.
Kaza A, Lim H, Dibardino D, Bautista-Hernandez V, Robinson J, Allan C, Pigula F. Long-term results of right ventricular outflow tract reconstruction in neonatal cardiac surgery: options and outcomes. J Thorac Cardiovasc Surg. 2009;138(4):911–6.
Keckler S, St Peter S, Spilde T, Ostlie D, Snyder C. The influence of trisomy 21 on the incidence and severity of congenital heart defects in patients with duodenal atresia. Pediatr Surg Int. 2008;24(8):921–3.
Kirklin J, Karp R. The tetralogy of fallot from a surgical viewpoint. Philadelphia: WB Saunders; 1970.
Kirsch R, Glatz A, Gaynor J, Nicolson S, Spray T, Wernovsky G, Bird G. Results of elective repair at 6 months or younger in 277 patients with tetralogy of fallot : a 14-year experience at a single center. J Thorac Cardiovasc Surg. 2009;147(2):713–7.
Lake C. Pediatric cardiac anesthesia. McGraw-Hill/Appleton and Lange. 2nd ed. 1993.
Lamberti J, Carlisle J, Waldman J, et al. Systemic-pulmonary shunts in infants and children. Early and late results. J Thorac Cardiovasc Surg. 1984;88(1):76–81.
Lammer E, Chak J, Iovannisci D, Schultz K, et al. Chromosomal abnormalities among children born with conotruncal cardiac defects. Birth Defects Res A Clin Mol Teratol. 2009;85(1):30–5.
Levy S, Blalock A. Experimental observations on the effects of connecting by suture the left main pulmonary artery to the systemic circulation. J Thorac Surg. 1939;8:525–30.
Lillehei C, Cohen M, Warden H, et al. Direct vision intracardiac correction of the tetralogy of fallot, pentalogy of fallot, and pulmonary atresia defects: report of first ten cases. Ann Surg. 1955;142(3):418–42.
Lillehei C, Varco R, Cohen M, Warden H, et al. The first open heart corrections of tetralogy of fallot: a 26–31 year follow-up of 106 patients. Ann Surg. 1986;204(4):490–502.
Lindsey C, Parks W, Kogon B, Sallee D, Mahle W. Pulmonary valve replacement after tetralogy of fallot repair in preadolescent patients. Ann Thorac Surg. 2010;89(1):147–51.
Little J, Lavender P, DeSanctis R. The narrow infundibulum in pulmonary valvar stenosis. Circulation. XXVIII August. 1963:182–9.
Michielon G, Marino B, Formigari R, Gargiulo G, Picchio F, Digilio M, Di Donato R. Genetic syndromes and outcome after surgical correction of tetralogy of fallot. Ann Thorac Surg. 2006;81(3):968–75.
Montero J, Nieto N, Vallejo I, Montero S. Intranasal midazolam for the emergency management of hypercyanotic spells in tetralogy of fallot. Pediatr Emerg Care. 2015;4:269–71.
Mulinari LA1, Navarro FB, Pimentel GK, Miyazaki SM, Binotto CN, Pelissari EC, Miyague NI, da Costa FD. The use and midium-term evaluation of decellularized allograft cusp in the surgical treatment of the tetralogy of fallot. Rev Bras Cir Cardiovasc. 2008; 23(2):197–203.
Myers J, Ghanayem N, Cao Y, et al. Outcomes of systemic to pulmonary artery shunts in patients weighing less than 3 kg : analysis of shunt type, size, and surgical approach. J Thorac Cardiovasc Surg. 2014;147(2):672–7.
Nasr A, McNamara PJ, Mertens L, Levin D, James A, Holtby H, Langer JC. Is routine preoperative 2-dimensional echocardiography necessary for infants with esophageal atresia, omphalocele, or anorectal malformations? J Pediatr Surg. 2010;45(5):876–9.
Newfeld E, Waldman D, et al. Pulmonary vascular disease after systemic-pulmonary arterial shunt operations. Am J Cardiol. 1977;39(5):715–20.
Odegard KC, Schure A, Saiki Y, Hansen DD, Jonas RA, Laussen PC. Anesthetic considerations during caval inflow occlusion in children with congenital heart disease. J Clin Ultrasound. 2004;18(2):144–7.
Ong J, Brizard C, d’Udekem Y, Weintraub R, Robertson T, Cheung M, Konstantinov I. Repair of atrioventricular septal defect associated with tetralogy of fallot or double-outlet right ventricle: 30 years of experience. Ann Thorac Surg. 2012;94(1):172–8.
Parker J, Brooks D, Kozar L, Render-Teixeira C, Horner R, et al. Acute and chronic effects of airway obstruction on canine left ventricular performance. Am J Respir Crit Care Med. 1999;160(6):1888–96.
Pickering D, Trusler G, Lipton I, Keith J. Waterston anastomosis: comparison of results of operation before and after age 6 months. Thorax. 1971;26:457–9.
Pierce J, Sharma S, Hunter C, Bhombal S, Fagan B, Corchado Y, Bushman G. Intraoperative hypercyanosis in a patient with pulmonary artery band: case report and review of the literature. J Clin Anesth. 2012;24(8):652–5.
Quail M, Frigiola A, Giardini A, Muthurangu V, Hughes M, Lurz P, Taylor A. Impact of pulmonary valve replacement in tetralogy of fallot with pulmonary regurgitation: a comparison of intervention and nonintervention. Ann Thorac Surg. 2012;94(5):1619–26.
Ritter S, Tany L, Shaddy R, Minich L. Are screening echocardiograms warranted for neonates with meningomyelocele? Arch Pediatr Adolesc Med. 1999;153(12):1264–6.
Rudolph A. Congenital diseases of the heart: clinical-physiological considerations. Wiley-Blackwell 3rd ed. 2009; p. 345–85.
Said S, Burkhart H, Schaff H, Johnson J, Connolly H, Dearani J. When should a mechanical tricuspid valve replacement be considered? J Thorac Cardiovasc Surg. 2014;148(2):603–8.
Schwartz MC, Glatz AC, Dori Y, Rome JJ, Gillespie MJ. Outcomes and predictors of reintervention in patients with pulmonary atresia and intact ventricular septum treated with radiofrequency perforation and balloon pulmonary valvuloplasty. Pediatr Cardiol. 2013;35(1):22–9.
Sharkey A, Sharma A. Tetralogy of fallot: anatomic variants and their impact on surgical management. Semin Cardiothorac Vasc Anesth. 2012;2:88–96.
Short S, Pierce J, Burke R, et al. Is routine preoperative screening echocardiogram indicated in all children with congenital duodenal obstruction? Pediatr Surg Int. 2014;6:609–14.
Shprintzen RJ, Goldberg RB, Young D, Wolford L. The velo-cardio-facial syndrome: a clinical and genetic analysis. Pediatrics. 1981;67:167–72.
Shuhaiber J, Robinson B, Gauvreau K, Breitbart R, Mayer J, Del Nido P, Pigula F. Outcome after repair of atrioventricular septal defect with tetralogy of fallot. J Thorac Cardiovasc Surg. 2012;143(2):338–43.
Soto B, Pacifico A, Ceballos R, Bargeron L. Tetralogy of fallot: an angiographic-pathologic correlative study. Circulation. 1981;64(3):558–66.
Steiner M, Tang X, Gossett J, 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.
Stephenson L, Friedman S, Edmonds L. Staged surgical management in tetralogy of fallot in infants. Circulation. 1978;58(5):837–41.
Tamesberger M, 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.
Tanaka K, Kitahata H, Kawahito S, Nozaki J, Tomiyama Y, Oshita S. Phenylephrine increases pulmonary blood flow in children with tetralogy of fallot. Can J Anaesth. 2003;50(9):926–9.
Tempe D, Virmani S. Coagulation abnormalities in patients with cyanotic congenital heart disease. J Cardiothorac Vasc Anesth. 2002;16(6):752–65.
Thapar M, Rao P. Use of propranolol for severe dynamic infundibular obstruction prior to balloon pulmonary valvuloplasty (a brief communication). Cathet Cardiovasc Diagn. 1990;4:240–1.
Tharakan J. Admixture lesions in congenital cyanotic heart disease. Ann Pediatr Cardiol. 2011;4(1):53–9.
Trucoone N, Bowman F, Malm J, Gersony W. Systemic to pulmonary artery shunts in the first year of life. Circulation. 1974;49:508–11.
Tug M. Ketamine infusion versus isoflurane for the maintenance of anesthesia in the prebypass period in children with tetralogy of fallot. Children. 2000;14(5):557–61.
Turrentine M, Mccarthy R, Vijay P, Mcconnell K, Brown J. PTFE monocusp valve reconstruction of the right ventricular outflow tract. Ann Thorac Surg. 2002;73(3):871–9.
Vida V, Guariento A, Castaldi B, et al. Evolving strategies for preserving the pulmonary valve during early repair of tetralogy of fallot : mid-term results. J Thorac Cardiovasc Surg. 2014;147(2):687–96.
Waldman J, Wernly J. Cyanotic congenital heart disease with decreased pulmonary blood flow in children. Pediatr Clin North Am. 1999;46(2):385–404.
Walker A, Stokes M, Moriarty A. Anesthesia for major general surgery in neonates with complex cardiac defects. Paediatr Anaesth. 2009;19:119–25.
Wells W, Arroyo H, Bremner R, Wood J, Starnes V. Homograft conduit failure in infants is not due to somatic outgrowth. J Thorac Cardiovasc Surg. 2002;124(1):88–96.
White M. Approach to managing children with heart disease for noncardiac surgery. Paediatr Anaesth. 2011;21(5):522–9.
Wyse R, Mars M, al-Mahdawi S, Russell-Eggitt IM, Blake KD. Congenital heart anomalies in patients with clefts of the lip and/or palate. Cleft Palate J. 1990;27(3):258–64.
Yoo B, Kim J, Kim Y, Choi J, Park H, Park Y, Sul J. Impact of pressure load caused by right ventricular outflow tract obstruction on right ventricular volume overload in patients with repaired tetralogy of fallot. J Thorac Cardiovasc Surg. 2012;143(6):1299–304.
Yoshimura N, Yamaguchi M, Ohashi H, Oshima Y, Oka S, Yoshida M, Tei T. Pulmonary atresia with intact ventricular septum: strategy based on right ventricular morphology. J Thorac Cardiovasc Surg. 2002;126(5):1417–26.
Zabala L, Guzzetta N. Cyanotic congenital heart disease (CCHD): focus on hypoxemia, secondary erythrocytosis, and coagulation alterations. Pediatr Anaesth. 2015;25(10):981–9.
Zampi JD, Hirsch JC, Gurney JG, Donohue JE, Yu S, Lapage MJ, Charpie JR. Junctional ectopic tachycardia after infant heart surgery: incidence and outcomes. Pediatr Cardiol. 2012;33(8):1362–9.
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Bushman, G.A. (2017). Tetralogy of Fallot. In: Dabbagh, A., Conte, A., Lubin, L. (eds) Congenital Heart Disease in Pediatric and Adult Patients. Springer, Cham. https://doi.org/10.1007/978-3-319-44691-2_19
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