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Tetralogy of Fallot

  • Jonathan Kochav
Chapter
Part of the In Clinical Practice book series (ICP)

Abstract

  • Tetralogy of Fallot is the most common cyanotic congenital heart lesion after infancy, with an incidence of 4–5 per 10,000 live births. It accounts for about ~5% of cases of congenital heart disease worldwide. This chapter describes the anatomy and physiology of the tetralogy of Fallot, the anatomy and physiology of the palliative shunts and repairs used to treat the disease in infancy, the primary modalities used for diagnosis and surveillance, and the options and indications for surgical or percutaneous intervention in adult patients.

References

  1. 1.
    Reller MD, Strickland MJ, Riehle-Colarusso T, Mahle WT, Correa A. Prevalence of congenital heart defects in metropolitan Atlanta, 1998-2005. J Pediatr. 2008;153:807–13.CrossRefGoogle Scholar
  2. 2.
    van 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;58:2241–7.CrossRefGoogle Scholar
  3. 3.
    Neill CA, Clark EB. Tetralogy of Fallot. The first 300 years. Tex Heart Inst J. 1994;21:272–9.PubMedPubMedCentralGoogle Scholar
  4. 4.
    Blalock A, Taussig HB. Landmark article May 19, 1945: the surgical treatment of malformations of the heart in which there is pulmonary stenosis or pulmonary atresia. By Alfred Blalock and Helen B. Taussig. JAMA. 1984;251:2123–38.CrossRefGoogle Scholar
  5. 5.
    Lillehei CW, Varco RL, Cohen M, et al. The first open heart corrections of tetralogy of Fallot. A 26-31 year follow-up of 106 patients. Ann Surg. 1986;204:490–502.CrossRefGoogle Scholar
  6. 6.
    Bonhoeffer P, Boudjemline Y, Saliba Z, et al. Percutaneous replacement of pulmonary valve in a right-ventricle to pulmonary-artery prosthetic conduit with valve dysfunction. Lancet. 2000;356:1403–5.CrossRefGoogle Scholar
  7. 7.
    Gaggin H, Januzzi JL Jr. Chapter 21. MGH Cardiology Board Review Book. New York: Springer; 2014.Google Scholar
  8. 8.
    Niwa K, Siu SC, Webb GD, Gatzoulis MA. Progressive aortic root dilatation in adults late after repair of tetralogy of Fallot. Circulation. 2002;106:1374–8.CrossRefGoogle Scholar
  9. 9.
    Kapur S, Aeron G, Vojta CN. Pictorial review of coronary anomalies in Tetralogy of Fallot. J Cardiovasc Comput Tomogr. 2015;9:593–6.CrossRefGoogle Scholar
  10. 10.
    Swamy P, Bharadwaj A, Varadarajan P, Pai RG. Echocardiographic evaluation of tetralogy of Fallot. Echocardiography. 2015;32(Suppl 2):S148–56.CrossRefGoogle Scholar
  11. 11.
    Warnes CA, Williams RG, Bashore TM, et al. ACC/AHA 2008 Guidelines for the Management of Adults with Congenital Heart Disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (writing committee to develop guidelines on the management of adults with congenital heart disease). Circulation. 2008;118:e714–833.PubMedPubMedCentralGoogle Scholar
  12. 12.
    DeFaria Yeh D, King ME. Congenital heart disease in the adult: what should the adult cardiologist know? Curr Cardiol Rep. 2015;17:25.CrossRefGoogle Scholar
  13. 13.
    Chowdhury D, Gurvitz M, Marelli A, et al. Development of quality metrics in ambulatory pediatric cardiology. J Am Coll Cardiol. 2017;69:541–55.CrossRefGoogle Scholar
  14. 14.
    Cheatham JP, Hellenbrand WE, Zahn EM, et al. Clinical and hemodynamic outcomes up to 7 years after transcatheter pulmonary valve replacement in the US melody valve investigational device exemption trial. Circulation. 2015;131:1960–70.CrossRefGoogle Scholar
  15. 15.
    Fraisse A, Aldebert P, Malekzadeh-Milani S, et al. Melody (R) transcatheter pulmonary valve implantation: results from a French Registry. Arch Cardiovasc Dis. 2014;107:607–14.CrossRefGoogle Scholar
  16. 16.
    Khairy P, Marelli AJ. Clinical use of electrocardiography in adults with congenital heart disease. Circulation. 2007;116:2734–46.CrossRefGoogle Scholar
  17. 17.
    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;356:975–81.CrossRefGoogle Scholar
  18. 18.
    Gatzoulis MA, Till JA, Somerville J, Redington AN. Mechanoelectrical interaction in tetralogy of Fallot. QRS prolongation relates to right ventricular size and predicts malignant ventricular arrhythmias and sudden death. Circulation. 1995;92:231–7.CrossRefGoogle Scholar
  19. 19.
    DeFaria Yeh D, Foster E. Is MRI the preferred method for evaluating right ventricular size and function in patients with congenital heart disease?: MRI is not the preferred method for evaluating right ventricular size and function in patients with congenital heart disease. Circ Cardiovasc Imaging. 2014;7:198–205.CrossRefGoogle Scholar
  20. 20.
    Geva T. Is MRIthe preferred method for evaluating right ventricular size and function in patients with congenital heart disease?: MRI is the preferred method for evaluating right ventricular size and function in patients with congenital heart disease. Circ Cardiovasc Imaging. 2014;7:190–7.CrossRefGoogle Scholar
  21. 21.
    Kochav J, Simprini L, Weinsaft JW. Imaging of the right heart--CT and CMR. Echocardiography. 2015;32(Suppl 1):S53–68.CrossRefGoogle Scholar
  22. 22.
    Maffei E, Messalli G, Martini C, et al. Left and right ventricle assessment with cardiac CT: validation study vs. cardiac MR. Eur Radiol. 2012;22:1041–9.CrossRefGoogle Scholar
  23. 23.
    Valente AM, Gauvreau K, Assenza GE, et al. Contemporary predictors of death and sustained ventricular tachycardia in patients with repaired tetralogy of Fallot enrolled in the INDICATOR cohort. Heart. 2014;100:247–53.CrossRefGoogle Scholar
  24. 24.
    Khairy P, Aboulhosn J, Gurvitz MZ, et al. Arrhythmia burden in adults with surgically repaired tetralogy of Fallot: a multi-institutional study. Circulation. 2010;122:868–75.CrossRefGoogle Scholar
  25. 25.
    Buechel ER, Dave HH, Kellenberger CJ, et al. Remodelling of the right ventricle after early pulmonary valve replacement in children with repaired tetralogy of Fallot: assessment by cardiovascular magnetic resonance. Eur Heart J. 2005;26:2721–7.CrossRefGoogle Scholar
  26. 26.
    Dave HH, Buechel ER, Dodge-Khatami A, et al. Early insertion of a pulmonary valve for chronic regurgitation helps restoration of ventricular dimensions. Ann Thorac Surg. 2005;80:1615–20, discussion 1620–1.CrossRefGoogle Scholar
  27. 27.
    Frigiola A, Tsang V, Bull C, et al. Biventricular response after pulmonary valve replacement for right ventricular outflow tract dysfunction: is age a predictor of outcome? Circulation. 2008;118:S182–90.CrossRefGoogle Scholar
  28. 28.
    Oosterhof T, van Straten A, Vliegen HW, et al. Preoperative thresholds for pulmonary valve replacement in patients with corrected tetralogy of Fallot using cardiovascular magnetic resonance. Circulation. 2007;116:545–51.CrossRefGoogle Scholar
  29. 29.
    Therrien J, Provost Y, Merchant N, Williams W, Colman J, Webb G. Optimal timing for pulmonary valve replacement in adults after tetralogy of Fallot repair. Am J Cardiol. 2005;95:779–82.CrossRefGoogle Scholar
  30. 30.
    Bokma JP, Winter MM, Oosterhof T, et al. Preoperative thresholds for mid-to-late haemodynamic and clinical outcomes after pulmonary valve replacement in tetralogy of Fallot. Eur Heart J. 2016;37(10):829–35.CrossRefGoogle Scholar
  31. 31.
    Geva T, Gauvreau K, Powell AJ, et al. Randomized trial of pulmonary valve replacement with and without right ventricular remodeling surgery. Circulation. 2010;122:S201–8.CrossRefGoogle Scholar
  32. 32.
    Geva T. Indications for pulmonary valve replacement in repaired tetralogy of fallot: the quest continues. Circulation. 2013;128(17):1855–7.CrossRefGoogle Scholar
  33. 33.
    Wijesekera VA, Kiess MC, Grewal J, et al. Aortic dissection in a patient with a dilated aortic root following tetralogy of Fallot repair. Int J Cardiol. 2014;174:833–4.CrossRefGoogle Scholar
  34. 34.
    Konstantinov IE, Fricke TA, d’Udekem Y, Robertson T. Aortic dissection and rupture in adolescents after tetralogy of Fallot repair. J Thorac Cardiovasc Surg. 2010;140:e71–3.CrossRefGoogle Scholar
  35. 35.
    Kim WH, Seo JW, Kim SJ, Song J, Lee J, Na CY. Aortic dissection late after repair of tetralogy of Fallot. Int J Cardiol. 2005;101:515–6.CrossRefGoogle Scholar
  36. 36.
    Rathi VK, Doyle M, Williams RB, Yamrozik J, Shannon RP, Biederman RW. Massive aortic aneurysm and dissection in repaired tetralogy of Fallot; diagnosis by cardiovascular magnetic resonance imaging. Int J Cardiol. 2005;101:169–70.CrossRefGoogle Scholar
  37. 37.
    Canobbio MM, Warnes CA, Aboulhosn J, et al. Management of pregnancy in patients with complex congenital heart disease: a scientific statement for healthcare professionals from the American Heart Association. Circulation. 2017;135(8):e50–87.CrossRefGoogle Scholar
  38. 38.
    Veldtman GR, Connolly HM, Grogan M, Ammash NM, Warnes CA. Outcomes of pregnancy in women with tetralogy of Fallot. J Am Coll Cardiol. 2004;44:174–80.CrossRefGoogle Scholar

Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  • Jonathan Kochav
    • 1
  1. 1.Massachusetts General HospitalBostonUSA

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