EDUCATIONAL REVIEW
Tetralogy of Fallot: Everything you wanted to know but were afraid to ask
Lisa Wise-Faberowski,
Ritu Asija,
Doff B. McElhinney,
Corresponding Author
Lisa Wise-Faberowski
Department of Anesthesiology, Stanford University, Palo Alto, California
Correspondence
Lisa Wise-Faberowski, Department of Anesthesiology, Stanford University, Palo Alto, CA.
Email: [email protected]
Search for more papers by this authorRitu Asija
Department of Pediatrics, Stanford University, Palo Alto, California
Search for more papers by this authorDoff B. McElhinney
Department of Surgery, Stanford University, Palo Alto, California
Search for more papers by this authorLisa Wise-Faberowski,
Ritu Asija,
Doff B. McElhinney,
Corresponding Author
Lisa Wise-Faberowski
Department of Anesthesiology, Stanford University, Palo Alto, California
Correspondence
Lisa Wise-Faberowski, Department of Anesthesiology, Stanford University, Palo Alto, CA.
Email: [email protected]
Search for more papers by this authorRitu Asija
Department of Pediatrics, Stanford University, Palo Alto, California
Search for more papers by this authorDoff B. McElhinney
Department of Surgery, Stanford University, Palo Alto, California
Search for more papers by this authorSummary
Tetralogy of Fallot (TOF) has four anatomic features: right ventricular hypertrophy (RVH), ventriculoseptal defect (VSD), overriding aorta and right ventricular outflow tract obstruction (RVOT) with an occurrence of 3.9 /10,000 births. The pathophysiologic effects in TOF are largely determined by the degree of RVOT and not the VSD. Intra-operative anesthetic management is also dependent on the degree of RVOT obstruction and influenced by the extent of surgical RVOT repair.
DISCLOSURES
There are no conflicts of interest.
REFERENCES
- 1Van der Linde D, Konings EE, Slager MA, et al. Birth prevalence of congenital heart disease worldwide: a systematic review and meta-analysis. J Am Coll Cardiol. 2011; 58: 2241-2247.
- 2Ferencz C, Rubin JD, McCarter RJ, et al. Congenital heart disease: prevalence at live birth. The Baltimore-Washington infant study. Am J Epidemiol. 1985; 121: 31-36.
- 3Perloff JK. The clinical recognition of congenital heart disease, 4th. Philadelphia, PA: WB Saunders; 1994.
- 4Breitbart RE, Fyler DC. Tetralogy of Fallot. In JF Keane, JE Locke, DC Fyler Eds. Nadas' pediatric cardiology, 2nd Ed. Philadelphia, PA: Saunders; 2006: 559.
- 5Hoffman JE. Tetralogy of Fallot. In: JE Hoffman, ed. The Natural and Unnatural History of Congenital Heart Disease. West Sussex, UK: Wiley-Blackwell; 2009: 413-436.
10.1002/9781444314045.ch42 Google Scholar
- 6O'Donnell CP, Landzberg MJ, Mullen MP. Lung abscesses in adults with tetralogy of fallot and pulmonary atresia. Cardiol Young. 2010; 20: 91-93.
- 7Lammer EJ, Chak JS, Iovannisci DM, et al. Chromosomal abnormalities among children born with conotruncal cardiac defects. Birth Defects Res A Clin Mol Teratol. 2009; 85: 30.
- 8Goldmuntz E, Geiger E, Benson DW. NKX2.5 mutations in patients with tetralogy of fallot. Circulation. 2001; 104: 2565-2568.
- 9Griffin HR, Topf A, Glen E, et al. Systemic survey of variants in TBX1 in non-syndromic tetralogy of Fallot identifies a novel 57 base pair deletion that reduces transcriptional activity but finds no association with common variants. Heart. 2010; 96: 1651.
- 10Marinho C, Alho I, Guerra A, et al. The methylenetetrahydrofolate reductase gene variant (C677T) as a susceptibility gene for tetralogy of Fallot. Rev Port Cardiol. 2009; 28: 809.
- 11Freeman SB, Taft LF, Dooley KJ, et al. Population based study of congenital heart defects in Down syndrome. Am J Med Genet. 1998; 80: 213-217.
10.1002/(SICI)1096-8628(19981116)80:3<213::AID-AJMG6>3.0.CO;2-8 CAS PubMed Web of Science® Google Scholar
- 12Bauer RC, Laney AO, Smith R, et al. Jagged1 (JAG 1) mutations in patients with tetralogy of Fallot or pulmonic stenosis. Hum Mutat. 2010; 13: 594-601.
- 13Maeda J, Yamagishi H, Matsuoka R, et al. Frequent association of 22q11.2 deletion with tetralogy of Fallot. Am J Med Genet. 2000; 92: 269-272.
10.1002/(SICI)1096-8628(20000605)92:4<269::AID-AJMG9>3.0.CO;2-L CAS PubMed Web of Science® Google Scholar
- 14Dabizzii RP, Caprioli G, Aiazzi L, et al. Distribution and anomalies of coronary arteries in tetralogy of fallot. Circulation. 1980; 61: 95-102.
- 15Kohli V, Azad S, Sachdev MS, et al. Balloon dilation of the pulmonary valve in premature infants with tetralogy of Fallot. Pediatr Cardiol. 2008; 29: 946-959.
- 16Sivakumar K, Bhagyavathy A, Coelho R, et al. Longevity of neonatal ductal stenting for congenital heart diseases with duct-dependent pulmonary circulation. Congenit Heart Dis. 2012; 7: 526-533.
- 17Jonas RA. Tetralogy of Fallot with pulmonary atresia. In: RA Jonas, ed. Comprehensive Surgical Management of Congenital Heart Disease, 2nd edn. Boca Raton, FL: CRC Press, Taylor & Francis Group; 2014: 585-603.
10.1201/b13497 Google Scholar
- 18Mercer-Rosa L, Pinto N, Yang W, et al. 22q11 deletion syndrome is associated with perioperative outcome in tetralogy of fallot. J Thorac Cardiovasc Surg. 2013; 146: 868-873.
- 19Morales DL, Zafar F, Fraser CD. Tetralogy of Fallot repair: the right ventricle infundibulum sparing (RVIS) strategy. Semin Thorac Cardiovasc Surg Pediatr Card Surg Ann. 2009; 12: 54-58.
10.1053/j.pcsu.2009.02.001 Google Scholar
- 20Yang S, Novello R, Nicolson S, et al. Evaluation of ventricular septal defect repair using intraoperative transesophageal echocardiography: frequency and significance of residual defects in infants and children. Echocardiography. 2000; 17: 681-684.
- 21Knauth AL, Gauvreau AJ, Powell AJ, et al. Ventricular size and function assessed by cardiac MRI predict major adverse clinical outcomes late after tetralogy of Fallot repair. Heart. 2008; 94: 211-216.
- 22Andreasen JB, Johnsen SP, Raven HB. Junctional ectopic tachycardia after surgery for congenital heart disease in children. Intensive Care Med. 2008; 34: 895-902.
- 23Imamura M, Dossey AM, Garcia X, et al. Prophylactic amiodarone reduces junctional ectopic tachycardia after tetralogy of Fallot repair. J Thorac Cardiovasc Surg. 2012; 143: 152-156.
- 24Rivenes SM, Lewin MB, Stayer SA, et al. Cardiovascular effects of sevoflurane, isoflurane, halothane, and fentanyl-midazolam in children with congenital heart disease: an echocardiographic study of myocardial contractility and hemodynamics. Anesthesiology. 2001; 94: 223-229.
- 25Kadam SV, Tailor KB, Kulkarni S, et al. Effect of dexmedetomidine on postoperative junctional ectopic tachycardia after complete surgical repair of tetralogy of Fallot; A prospective randomized controlled study. Ann Card Anaesth. 2015; 18: 323.
- 26Malhotra SP, Hanley FL. Surgical management of pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals: a protocol-based approach. Semin Thorac Cardiovasc Surg: Pediatr Cardiac Surg Annu. 2009; 12: 145-151.
10.1053/j.pcsu.2009.01.017 Google Scholar
- 27d'Udekem Y, Alphonso N, Norgaard MA, et al. Pulmonary atresia with ventricular septal defects and major aortopulmonary collateral arteries: unifocalization brings no long-term benefits. J Thorac Cardiovasc Surg. 2005; 130: 1496-1502.
- 28Brizard CP, Liava'a M, d'Udekem Y. Pulmonary atresia, VSD, and MAPCAs: repair without unifocalization. Semin Thorac Cardiovasc Surg Pediatr. Card Surg Ann. 2009; 12: 139-144.
- 29Liava'a M, Brizard CP, Konstantinov IE et al. Pulmonary atresia, ventricular septal defect, and major aortopulmonary collaterals: neonatal pulmonary artery rehabilitation without unifocalization. Ann Thorac Surg. 2012; 93: 185-192.
- 30Mainwaring RD, Patrick WL, Roth S, et al. Surgical algorithm and results for repair of pulmonary atresia with ventriculoseptal defect and major aortopulmonary collaterals. J Cardiovasc Surg. 2018; 12: pii. S0022-S5223.
- 31Sidell DR, Koth AM, Bauser-Heaton H, et al. Bronchoscopy in children with tetralogy of fallot, pulmonary atresia and major aortopulmonary collaterals. Pediatr Pulmonol. 2017; 9999: 1-6.
- 32Leopold C, DeBarros A, Cellier C, et al. Laryngeal abnormalities are frequent in 22q11 deletion syndrome. Int J Pediatr Otorhinolaryngol. 2012; 76: 36-40.
- 33Chen Q, Ma K, Hua Z, et al. Multistage pulmonary artery rehabilitation in patients with pulmonary atresia, ventricular septal defect and hypoplastic pulmonary artery. Eur J Cardiothoracic Surg. 2014; 46: 297-303.
- 34Bauser-Heaton H, Borquez A, Koth A, et al. Programmatic approach to management of Tetralogy of Fallot with major aortopulmonary collateral arteries: A 15-year experience with 458 patients. Circ Cardiovasc Interv. 2017; 10: pii: e004952.
- 35Watanabe N, Mainwaring RD, Reddy, et al. Early complete repair of pulmonary atresia with ventriculoseptal defect and major aortopulmonary collaterals. Ann Thorac Surg. 2104; 97: 909-915.
- 36Zhu J, Meza J, Kato A, et al. Pulmonary blood flow study predicts survival in pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries. J Thorac Cardiovasc Surg. 2016; 152: 1494-1503.
- 37Quinonez Z, Abbasi R, Downey L, et al. Anesthetic management of pulmonary atresia, ventriculo-septal defect with aortopulmonary collaterals. World J Pediatr Congenit Heart Surg. 2018; 9: 236-241.
- 38Talbot NP, Balanos GM, Dorrington KL et al. Two temporal components within the human pulmonary vascular response to ~2 h of isocapnic hypoxia. J Appl Physiol. 2005; 8: 1125-1139.
- 39Rudolph AM, Yuan S. Response of the pulmonary vasculature to hypoxia and H+ ion concentration changes. J Clin Invest. 1966; 45(3): 399-411.
- 40Meng L, Gelb A. RegulaCon of Cerebral Autoregulation by Carbon Dioxide. Anesthesiology. 2015; 122(1): 196-205.
