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Tetralogy of Fallot

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Critical Care of Children with Heart Disease

Abstract

Tetralogy of Fallot (TOF) is a relatively common congenital heart defect occurring in approximately 15% of patients with congenital heart disease. The four main anatomic features of TOF include right ventricular outflow tract (RVOT) obstruction [1, 2], ventricular septal defect (VSD), aortic dextroposition overridding the VSD, and right ventricular hypertrophy (Fig. 19.1). Current teaching postulates that the basic pathology of TOF results from underdevelopment of the right ventricular infundibulum. This underdevelopment causes an anterior malalignment of the infundibular septum which subsequently determines the degree of RVOT obstruction. The VSD that results from this malalignment is almost always large, and thus unrestrictive, permitting similar pressures between the right and left ventricles to occur. In addition to these features the pulmonary valve is frequently hypoplastic and thickened and the level of obstruction may extend to the main pulmonary artery and right and left pulmonary arteries.

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References

  1. Siwik E, Erenberg F, Zahka K, et al. Epidemiology and genetics of tetralogy of Fallot. In: Allen HD, Driscoll DJ, Shaddy RE, Feltes TF, eds. Moss and Adams’ Heart Disease in Infants, Children, and Adolescents. 7th ed. Philadelphia, PA: Lippincot Williams & Wilkins; 2008:888–910.

    Google Scholar 

  2. Dabizzi RP, Caprioli G, Aiazzi L, et al. Distribution and anomalies of coronary arteries in tetralogy of Fallot. Circulation. 1980;61:95–102.

    Article  PubMed  CAS  Google Scholar 

  3. 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;109:332–344.

    Article  PubMed  CAS  Google Scholar 

  4. Marino B, Digilio MC, Grazioli S, et al. Associated cardiac anomalies in isolated and syndromic patients with tetralogy of Fallot. Am J Cardiol. 1996;77:505–508.

    Article  PubMed  CAS  Google Scholar 

  5. Mulder TJ, Pyles LA, Stolfi A, Pickoff AS, Moller JH. A multicenter analysis of the choice of initial surgical procedure in tetralogy of Fallot. Pediatr Cardiol. 2002;23:580–586.

    Article  PubMed  CAS  Google Scholar 

  6. Pigula FA, Khalil PN, Mayer JE, del Nido PJ, Jonas RA. Repair of tetralogy of Fallot in neonates and young infants. Circulation. 1999;100:II157–II161.

    Article  PubMed  CAS  Google Scholar 

  7. Dodge-Khatami A, Miller OI, Anderson RH, Gil-Jaurena JM, Goldman AP, De Leval MR. Impact of junctional ectopic tachycardia on postoperative morbidity following repair of congenital heart defects. Eur J Cardiothorac Surg. 2002;21:255–259.

    Article  PubMed  CAS  Google Scholar 

  8. Daliento L, Rizzoli G, Menti L, et al. Accuracy of electrocardiographic and echocardiographic indices in predicting life threatening ventricular arrhythmias in patients operated for tetralogy of Fallot. Heart. 1999;81:650–655.

    PubMed  CAS  Google Scholar 

  9. Daliento L. Total correction of tetralogy of Fallot: late clinical follow-up. Ital Heart J. 2002;3:24–27 (Review).

    Google Scholar 

  10. Senzaki H, Ishido H, Iwamoto Y, et al. Sedation of hypercyanotic spells in a neonate with tetralogy of Fallot using dexmedetomidine. J Pediatr. 2008;84:377–380.

    Article  Google Scholar 

  11. Derby CD, Kolcz J, Gidding S, Pizarro C. Outcomes following non-valved autologous reconstruction of the right ventricular outflow tract in neonates and infants. Eur J Cardiothorac Surg. 2008;34(4):726–31.

    Article  PubMed  Google Scholar 

  12. Verheugt CL, Uiterwaal CS, Grobbee DE, Mulder BJ. Long-term prognosis of congenital heart defects: A systematic review. Int J Cardiol. 2008;131(1):25–32.

    Article  PubMed  Google Scholar 

  13. Frigiola A, Tsang V, Nordmeyer J, Lurz P, van Doorn C, Taylor AM, Bonhoeffer P, de Leval M. Current approaches to pulmon-ary regurgitation. Eur J Cardiothorac Surg. 2008;34(3):576–80.

    Article  PubMed  Google Scholar 

  14. Samman A, Schwerzmann M, Balint OH, et al. Exercise capacity and biventricular function in adult patients with repaired tetralogy of Fallot. Am Heart J. 2008;156: 100–105.

    Article  PubMed  Google Scholar 

  15. Redington AN. Physiopathology of right ventricular failure. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2006: 3–10 (Review).

    Google Scholar 

  16. Frigiola A, Redington AN, Cullen S, Vogel M. Pulmonary regurgitation is an important determinant of right ventricular contractile dysfunction in patients with surgically repaired tetralogy of Fallot. Circulation. 2004;110:II153–II157.

    Article  PubMed  CAS  Google Scholar 

  17. Vogel M, Sponring J, Cullen S, Deanfield JE, Redington AN. Regional wall motion and abnormalities of electrical depolarization and repolarization in patients after surgical repair of tetralogy of Fallot. Circulation. 2001;103:1669–1673.

    Article  PubMed  CAS  Google Scholar 

  18. Gatzoulis MA, Balaji S, Webber SA, et al. Risk factors for arrhythmia and sudden cardiac death late after repair of tetralogy of Fallot: a multicentre study. Lancet. 2000;356: 975–981.

    Article  PubMed  CAS  Google Scholar 

  19. Chaturvedi RR, Shore DF, Lincoln C, et al. Acute right ventricular restrictive physiology after repair of tetralogy of Fallot: association with myocardial injury and oxidative stress. Circulation. 1999;100:1540–1547.

    Article  PubMed  CAS  Google Scholar 

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Correspondence to Constantinos Chrysostomou .

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Chrysostomou, C., Domnina, Y.A., Kazmerski, T.M., Muñoz, R., Morell, V.O. (2009). Tetralogy of Fallot. In: Munoz, R., Morell, V., Cruz, E., Vetterly, C. (eds) Critical Care of Children with Heart Disease. Springer, London. https://doi.org/10.1007/978-1-84882-262-7_19

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  • DOI: https://doi.org/10.1007/978-1-84882-262-7_19

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