Skip to main content

Abstract

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.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 129.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 169.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

References

  1. Apitz C, Webb GD, Redington AN. Tetralogy of Fallot. Lancet. 2009;374(9699):1462–71.

    Article  PubMed  CAS  Google Scholar 

  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.

    Article  PubMed  CAS  Google Scholar 

  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.

    Article  PubMed  CAS  Google Scholar 

  4. Park MK. Pediatric cardiology for practitioners. 3rd ed. St. Louis: Mosby; 1996.

    Google Scholar 

  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.

    Article  PubMed  CAS  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

  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.

    Article  PubMed  CAS  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

  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.

    PubMed  CAS  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

  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.

    Article  PubMed  CAS  Google Scholar 

  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.

    Chapter  Google Scholar 

  19. Powell AJ, Geva T. Blood flow measurement by magnetic resonance imaging in congenital heart disease. Pediatr Cardiol. 2000;21(1):47–58.

    Article  PubMed  CAS  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

  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.

    Article  PubMed  CAS  Google Scholar 

  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.

    Article  Google Scholar 

  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.

    Article  PubMed  CAS  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

  28. Therrien J, Marx GR, Gatzoulis MA. Late problems in tetralogy of Fallot – recognition, management, and prevention. Cardiol Clin. 2002;20(3):395–404.

    Article  PubMed  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

  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.

    Article  PubMed  CAS  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Michael A. Quail MB ChB .

Editor information

Editors and Affiliations

7.1 Electronic Supplementary Material

Below is the link to the electronic supplementary material.

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)

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)

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)

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)

Rights and permissions

Reprints and permissions

Copyright information

© 2012 Springer-Verlag London

About this chapter

Cite this chapter

Quail, M.A., Muthurangu, V., Taylor, A.M. (2012). Tetralogy of Fallot. In: Syed, M., Mohiaddin, R. (eds) Magnetic Resonance Imaging of Congenital Heart Disease. Springer, London. https://doi.org/10.1007/978-1-4471-4267-6_7

Download citation

  • DOI: https://doi.org/10.1007/978-1-4471-4267-6_7

  • Published:

  • Publisher Name: Springer, London

  • Print ISBN: 978-1-4471-4266-9

  • Online ISBN: 978-1-4471-4267-6

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics