Tetralogy of Fallot

  • Michael A. QuailEmail author
  • Vivek Muthurangu
  • Andrew M. Taylor


Tetralogy of Fallot (TOF) is the most common form of cyanotic congenital heart disease, occurring in 1 in 3,600 live births [1]. Complete repair of TOF was devised over 50 years ago (first reported by Lillehei in 1954) and can result in complete intra-cardiac repair in early infancy [2]. However, despite excellent short- and medium-term survival rates, the 30 year actuarial survival for patients repaired before their 5th birthday is 90 % of the expected survival rate and the annualized risk of death triples in the third postoperative decade [2, 3]. Late morbidity and mortality, in particular related to pulmonary incompetence, has been observed in many patients long after total repair.


Right Ventricular Cardiovascular Magnetic Resonance Ventricular Septal Defect Pulmonary Valve Right Ventricular Outflow Tract 
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Supplementary material

Movie 7.1

3D volume rendered MR angiogram of the RA, RV, pulmonary trunk and branch pulmonary arteries, viewed from the front and then rotated along the horizontal axis. Note the kinked, narrowed origin of the left pulmonary artery(MOV 473 KB)

Movie 7.2

Balanced SSFP oblique sagittal cine through the RVOT and pulmonary trunk. Note the dilated, but contractile RVOT, dilated pulmonary trunk and free pulmonary incompetence (signal loss during diastole in the RV) (MOV 124 KB)

Movie 7.3

Four, balanced SSFP short axis cine images from base to apex, showing a pressure-overloaded RV (delayed RV emptying and septal flattening) with a marked dilated LV, with lateral wall akinesia. The patient had severe RV to pulmonary trunk conduit stenosis and a lateral wall infarction secondary to left circumflex artery damage at the time of surgery (MOV 1628 KB)

Movie 7.4

Volume rendered 4D CT, view from left posterior oblique, showing the dynamic nature of the RVOT and pulmonary trunk in a patient with a RVOT patch repair in infancy and chronic free pulmonary incompetence. Note the position of the left anterior descending coronary artery (MP4 166 KB)


  1. 1.
    Apitz C, Webb GD, Redington AN. Tetralogy of Fallot. Lancet. 2009;374(9699):1462–71.PubMedCrossRefGoogle Scholar
  2. 2.
    Murphy JG, Gersh BJ, Mair DD, Fuster V, McGoon MD, Ilstrup DM, et al. Long-term outcome in patients undergoing surgical repair of tetralogy of Fallot. N Engl J Med. 1993;329(9):593–9.PubMedCrossRefGoogle Scholar
  3. 3.
    Nollert G, Fischlein T, Bouterwek S, Bohmer C, Klinner W, Reichart B. 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. 1997;30(5):1374–83.PubMedCrossRefGoogle Scholar
  4. 4.
    Park MK. Pediatric cardiology for practitioners. 3rd ed. St. Louis: Mosby; 1996.Google Scholar
  5. 5.
    Oechslin EN, Harrison DA, Harris L, Downar E, Webb GD, Siu SS, et al. Reoperation in adults with repair of tetralogy of Fallot: indications and outcomes. J Thorac Cardiovasc Surg. 1999;118(2):245–51.PubMedCrossRefGoogle Scholar
  6. 6.
    Ghez O, Tsang VT, Frigiola A, Coats L, Taylor A, Van Doorn C, et al. Right ventricular outflow tract reconstruction for pulmonary regurgitation after repair of tetralogy of Fallot. Preliminary results. Eur J Cardiothorac Surg. 2007;31(4):654–8.PubMedCrossRefGoogle Scholar
  7. 7.
    Nordmeyer J, Tsang V, Gaudin R, Lurz P, Frigiola A, Jones A, et al. Quantitative assessment of homograft function 1 year after insertion into the pulmonary position: impact of in situ homograft geometry on valve competence. Eur Heart J. 2009;30(17):2147–54.PubMedCrossRefGoogle Scholar
  8. 8.
    Bonhoeffer P, Boudjemline Y, Saliba Z, Merckx J, Aggoun Y, Bonnet D, et al. Percutaneous replacement of pulmonary valve in a right-ventricle to pulmonary-artery prosthetic conduit with valve dysfunction. Lancet. 2000;356(9239):1403–5.PubMedCrossRefGoogle Scholar
  9. 9.
    Khambadkone S, Coats L, Taylor A, Boudjemline Y, Derrick G, Tsang V, et al. Percutaneous pulmonary valve implantation in humans: results in 59 consecutive patients. Circulation. 2005;112(8):1189–97.PubMedCrossRefGoogle Scholar
  10. 10.
    Coats L, Khambadkone S, Derrick G, Sridharan S, Schievano S, Mist B, et al. Physiological and clinical consequences of relief of right ventricular outflow tract obstruction late after repair of congenital heart defects. Circulation. 2006;113(17):2037–44.PubMedCrossRefGoogle Scholar
  11. 11.
    Lurz P, Nordmeyer J, Muthurangu V, Khambadkone S, Derrick G, Yates R, et al. Comparison of bare metal stenting and percutaneous pulmonary valve implantation for treatment of right ventricular outflow tract obstruction: use of an x-ray/magnetic resonance hybrid laboratory for acute physiological assessment. Circulation. 2009;119(23):2995–3001.PubMedCrossRefGoogle Scholar
  12. 12.
    Sorensen TS, Korperich H, Greil GF, Eichhorn J, Barth P, Meyer H, et al. Operator-independent isotropic three-dimensional magnetic resonance imaging for morphology in congenital heart disease: a validation study. Circulation. 2004;110(2):163–9.PubMedCrossRefGoogle Scholar
  13. 13.
    Schievano S, Coats L, Migliavacca F, Norman W, Frigiola A, Deanfield J, et al. Variations in right ventricular outflow tract morphology following repair of congenital heart disease: implications for percutaneous pulmonary valve implantation. J Cardiovasc Magn Reson. 2007;9(4):687–95.PubMedCrossRefGoogle Scholar
  14. 14.
    Geva T, Greil GF, Marshall AC, Landzberg M, Powell AJ. Gadolinium- enhanced 3-dimensional magnetic resonance angiography of pulmonary blood supply in patients with complex pulmonary stenosis or atresia: comparison with x-ray angiography. Circulation. 2002;106(4):473–8.PubMedCrossRefGoogle Scholar
  15. 15.
    Barkhausen J, Ruehm SG, Goyen M, Buck T, Laub G, Debatin JF. MR evaluation of ventricular function: true fast imaging with steady-state precession versus fast low-angle shot cine MR imaging: feasibility study. Radiology. 2001;219(1):264–9.PubMedGoogle Scholar
  16. 16.
    Winter MM, Bernink FJ, Groenink M, Bouma BJ, van Dijk AP, Helbing WA, et al. Evaluating the systemic right ventricle by CMR: the importance of consistent and reproducible delineation of the cavity. J Cardiovasc Magn Reson. 2008;10:40.PubMedCrossRefGoogle Scholar
  17. 17.
    Rebergen SA, Chin JG, Ottenkamp J, van der Wall EE, de Roos A. Pulmonary regurgitation in the late postoperative follow-up of tetralogy of Fallot. Volumetric quantitation by nuclear magnetic resonance velocity mapping. Circulation. 1993;88(5 Pt 1):2257–66.PubMedCrossRefGoogle Scholar
  18. 18.
    Taylor A, Bogaert J. Valvular heart disease. In: Bogaert J, Dymarkowski S, Taylor A, editors. Clinical cardiac MRI. Heidelberg: Springer; 2005. p. 353–79.CrossRefGoogle Scholar
  19. 19.
    Powell AJ, Geva T. Blood flow measurement by magnetic resonance imaging in congenital heart disease. Pediatr Cardiol. 2000;21(1):47–58.PubMedCrossRefGoogle Scholar
  20. 20.
    Grosse-Wortmann L, Al-Otay A, Goo HW, Macgowan CK, Coles JG, Benson LN, et al. Anatomical and functional evaluation of pulmonary veins in children by magnetic resonance imaging. J Am Coll Cardiol. 2007;49(9):993–1002.PubMedCrossRefGoogle Scholar
  21. 21.
    Devos DG, Kilner PJ. Calculations of cardiovascular shunts and regurgitation using magnetic resonance ventricular volume and aortic and pulmonary flow measurements. Eur Radiol. 2010;20(2):410–21.PubMedCrossRefGoogle Scholar
  22. 22.
    Greenberg SB, Crisci KL, Koenig P, Robinson B, Anisman P, Russo P. Magnetic resonance imaging compared with echocardiography in the evaluation of pulmonary artery abnormalities in children with tetralogy of Fallot following palliative and corrective surgery. Pediatr Radiol. 1997;27(12):932–5.PubMedCrossRefGoogle Scholar
  23. 23.
    Nordmeyer J, Gaudin R, Tann OR, Lurz PC, Bonhoeffer P, Taylor AM, et al. MRI may be sufficient for noninvasive assessment of great vessel stents: an in vitro comparison of MRI, CT, and conventional angiography. Am J Roentgenol. 2010;195(4):865–71.CrossRefGoogle Scholar
  24. 24.
    Babu-Narayan SV, Kilner PJ, Li W, Moon JC, Goktekin O, Davlouros PA, et al. Ventricular fibrosis suggested by cardiovascular magnetic resonance in adults with repaired tetralogy of fallot and its relationship to adverse markers of clinical outcome. Circulation. 2006;113(3):405–13.PubMedCrossRefGoogle Scholar
  25. 25.
    Tulevski II, Hirsch A, Dodge-Khatami A, Stoker J, van der Wall EE, Mulder BJ. Effect of pulmonary valve regurgitation on right ventricular function in patients with chronic right ventricular pressure overload. Am J Cardiol. 2003;92(1):113–6.PubMedCrossRefGoogle Scholar
  26. 26.
    Lurz P, Muthurangu V, Schievano S, Nordmeyer J, Bonhoeffer P, Taylor AM, et al. Feasibility and reproducibility of biventricular volumetric assessment of cardiac function during exercise using real- time radial k-t SENSE magnetic resonance imaging. J Magn Reson Imaging. 2009;29(5):1062–70.PubMedCrossRefGoogle Scholar
  27. 27.
    Lurz P, Puranik R, Nordmeyer J, Muthurangu V, Hansen MS, Schievano S, et al. Improvement in left ventricular filling properties after relief of right ventricle to pulmonary artery conduit obstruction: contribution of septal motion and interventricular mechanical delay. Eur Heart J. 2009;30(18):2266–74.PubMedCrossRefGoogle Scholar
  28. 28.
    Therrien J, Marx GR, Gatzoulis MA. Late problems in tetralogy of Fallot – recognition, management, and prevention. Cardiol Clin. 2002;20(3):395–404.PubMedCrossRefGoogle Scholar
  29. 29.
    Oosterhof T, Meijboom FJ, Vliegen HW, Hazekamp MG, Zwinderman AH, Bouma BJ, et al. Long-term follow-up of homograft function after pulmonary valve replacement in patients with tetralogy of Fallot. Eur Heart J. 2006;27(12):1478–84.PubMedCrossRefGoogle Scholar
  30. 30.
    Shebani SO, McGuirk S, Baghai M, Stickley J, De Giovanni JV, Bu’lock FA, et al. Right ventricular outflow tract reconstruction using Contegra valved conduit: natural history and conduit performance under pressure. Eur J Cardiothorac Surg. 2006;29(3):397–405.PubMedCrossRefGoogle Scholar
  31. 31.
    Vliegen HW, van Straten A, de Roos A, Roest AA, Schoof PH, Zwinderman AH, et al. Magnetic resonance imaging to assess the hemodynamic effects of pulmonary valve replacement in adults late after repair of tetralogy of fallot. Circulation. 2002;106(13):1703–7.PubMedCrossRefGoogle Scholar
  32. 32.
    Therrien J, Siu SC, McLaughlin PR, Liu PP, Williams WG, Webb GD. Pulmonary valve replacement in adults late after repair of tetralogy of Fallot: are we operating too late? J Am Coll Cardiol. 2000;36(5):1670–5.PubMedCrossRefGoogle Scholar
  33. 33.
    Frigiola A, Tsang V, Bull C, Coats L, Khambadkone S, Derrick G, et al. Biventricular response after pulmonary valve replacement for right ventricular outflow tract dysfunction: is age a predictor of outcome? Circulation. 2008;118(14 Suppl):S182–90.PubMedCrossRefGoogle Scholar
  34. 34.
    Oosterhof T, van Straten A, Vliegen HW, Meijboom FJ, van Dijk AP, Spijkerboer AM, et al. Preoperative thresholds for pulmonary valve replacement in patients with corrected tetralogy of Fallot using cardiovascular magnetic resonance. Circulation. 2007;116(5):545–51.PubMedCrossRefGoogle Scholar
  35. 35.
    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(6):779–82.PubMedCrossRefGoogle Scholar
  36. 36.
    Buechel ER, Dave HH, Kellenberger CJ, Dodge-Khatami A, Pretre R, Berger F, 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(24):2721–7.PubMedCrossRefGoogle Scholar
  37. 37.
    Coats L, Khambadkone S, Derrick G, Hughes M, Jones R, Mist B, et al. Physiological consequences of percutaneous pulmonary valve implantation: the different behaviour of volume- and pressure-overloaded ventricles. Eur Heart J. 2007;28(15):1886–93.PubMedCrossRefGoogle Scholar
  38. 38.
    Schievano S, Migliavacca F, Coats L, Khambadkone S, Carminati M, Wilson N, et al. Percutaneous pulmonary valve implantation based on rapid prototyping of right ventricular outflow tract and pulmonary trunk from MR data. Radiology. 2007;242(2):490–7.PubMedCrossRefGoogle Scholar
  39. 39.
    Sridharan S, Coats L, Khambadkone S, Taylor AM, Bonhoeffer P. Images in cardiovascular medicine. Transcatheter right ventricular outflow tract intervention: the risk to the coronary circulation. Circulation. 2006;113(25):e934–5.PubMedCrossRefGoogle Scholar
  40. 40.
    Schievano S, Taylor AM, Capelli C, Coats L, Walker F, Lurz P, et al. First-in-man implantation of a novel percutaneous valve: a new approach to medical device development. EuroIntervention. 2010;5(6):745–50.PubMedCrossRefGoogle Scholar

Copyright information

© Springer-Verlag London 2012

Authors and Affiliations

  • Michael A. Quail
    • 1
    Email author
  • Vivek Muthurangu
    • 2
  • Andrew M. Taylor
    • 3
  1. 1.Academic Clinical Fellow, Pediatric Cardiology, Centre for Cardiovascular ImagingUCL Institute of Cardiovascular Sciences and Great Ormond Street Hospital for ChildrenLondonUK
  2. 2.UCL Centre for Cardiovascular MRUniversity College LondonLondonUK
  3. 3.Cardio-Respiratory UnitUCL Institute of Child Health and Great Ormond Street Hospital for ChildrenLondonUK

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