Skip to main content
SpringerLink
Log in

Repaired Tetralogy of Fallot

  • Chapter
  • First Online:
Cardiac CT and MR for Adult Congenital Heart Disease

Abstract

The anatomy of tetralogy of Fallot (TOF), together with the pathophysiological consequences, was first described by Etienne-Louis Fallot in 1888. The tetrad of overriding aorta, right ventricular (RV) outflow tract obstruction, ventricular septal defect (VSD) and consequent RV hypertrophy is all due to antero-cephalad deviation of the outlet septum during fetal development. TOF is the most common cyanotic congenital heart disease (CHD), accounting for 10 % of CHD patients and occurring in 1 in 3,600 births (Shinebourne et al., Heart 92:1353–1359, 2006). It carries a recurrence risk of 3 % in siblings. There is genetic microdeletion in 22q11 in 15–25 % of TOF patients in whom TOF is part of DiGeorge syndrome. There is a spectrum of morphology, despite the four features that comprise ‘tetralogy’, and the severity of the RV outflow tract (RVOT) obstruction is the major determinant of first clinical presentation. Surgical palliation was achieved using the Blalock–Taussig shunt (subclavian artery to ipsilateral pulmonary artery connection) in 1944, a landmark event as these patients underwent the first cardiac surgery. Subsequently, surgical repair was described in the 1950s with the advent of cardiopulmonary bypass. Primary surgical repair involves patch closure of the VSD and intervention to the RVOT to relieve obstruction. RV muscle bundles are resected. Depending on the size of the outflow tract, pulmonary valve and pulmonary arteries; an RVOT patch, transannular patch and/or pulmonary artery (PA) patch may be required for RVOT reconstruction. In the variants with anomalous coronary arteries or in pulmonary atresia-type Fallot, an RV to PA conduit may be used.

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

Access this chapter

Log in via an institution
Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 149.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 199.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 279.99
Price excludes VAT (USA)
  • Durable hardcover 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. Shinebourne EA, Babu-Narayan SV, Carvalho JS. Tetralogy of Fallot: from fetus to adult. Heart. 2006;92(9):1353–9.

    Article  PubMed  Google Scholar 

  2. Babu-Narayan SV GM. Tetralogy of Fallot. In: Gatzoulis MA, Webb GD, Daubeney PEF, editors. Diagnosis and management of adult congenital heart disease. 2nd ed. Philadelphia: Elsevier Press; 2011. p. 316–28.

    Chapter  Google Scholar 

  3. Gatzoulis MA, Balaji S, Webber SA, Siu SC, Hokanson JS, Poile C, Rosenthal M, Nakazawa M, Moller JH, Gillette PC, Webb GD, Redington AN. Risk factors for arrhythmia and sudden cardiac death late after repair of tetralogy of Fallot: a multicentre study. Lancet. 2000;356(9234):975–81.

    Article  PubMed  CAS  Google Scholar 

  4. Hoffmann A, Engelfriet P, Mulder B. Radiation exposure during follow-up of adults with congenital heart disease. Int J Cardiol. 2007;118(2):151–3.

    Article  PubMed  Google Scholar 

  5. Ernst S, Babu-Narayan SV, Keegan J, Horduna I, Lyne J, Till J, Kilner PJ, Pennell D, Rigby ML, Gatzoulis MA. Remote-controlled magnetic navigation and ablation with 3D image integration as an alternative approach in patients with intra-atrial baffle anatomy. Circ Arrhythm Electrophysiol. 2012;5(1): 131–9.

    Article  PubMed  Google Scholar 

  6. Kilner P, Babu-Narayan SV. Adult congenital heart disease. In: Higgins CB, de Roos A, editors. MRI and CT of the cardiovascular system. 2nd ed. Philadelphia: Lippincott Williams & Wilkins; 2005. p. 429–40.

    Google Scholar 

  7. Gatehouse PD, Rolf MP, Bloch KM, Graves MJ, Kilner PJ, Firmin DN, Hofman MB. A multi-center inter-manufacturer study of the temporal stability of phase-contrast velocity mapping background offset errors. J Cardiovasc Magn Reson. 2012;14:72.

    Article  PubMed  Google Scholar 

  8. Rolf MP, Hofman MB, Gatehouse PD, Markenroth-Bloch K, Heymans MW, Ebbers T, Graves MJ, Totman JJ, Werner B, van Rossum AC, Kilner PJ, Heethaar RM. Sequence optimization to reduce velocity offsets in cardiovascular magnetic resonance volume flow quantification – a multi-vendor study. J Cardiovasc Magn Reson. 2011;13:18.

    Article  PubMed  Google Scholar 

  9. Kilner PJ, Gatehouse PD, Firmin DN. Flow measurement by magnetic resonance: a unique asset worth optimising. J Cardiovasc Magn Reson. 2007;9(4): 723–8.

    Article  PubMed  Google Scholar 

  10. Dabizzi RP, Caprioli G, Aiazzi L, Castelli C, Baldrighi G, Parenzan L, Baldrighi V. Distribution and anomalies of coronary arteries in tetralogy of Fallot. Circulation. 1980;61(1):95–102.

    Article  PubMed  CAS  Google Scholar 

  11. Kim RJ, Shah DJ, Judd RM. How we perform delayed enhancement imaging. J Cardiovasc Magn Reson. 2003;5(3):505–14.

    Article  PubMed  Google Scholar 

  12. Babu-Narayan SV. The role of late gadolinium enhancement cardiovascular magnetic resonance in the assessment of congenital and acquired heart disease. Prog Pediatr Cardiol. 2010;28:11–9.

    Article  Google Scholar 

  13. Kilner PJ. Pulmonary resistance in cardiovascular context. Int J Cardiol. 2004;97 Suppl 1:3–6.

    Article  PubMed  Google Scholar 

  14. Kilner PJ, Balossino R, Dubini G, Babu-Narayan SV, Taylor AM, Pennati G, Migliavacca F. Pulmonary regurgitation: the effects of varying pulmonary artery compliance, and of increased resistance proximal or distal to the compliance. Int J Cardiol. 2009;133(2): 157–66.

    Article  PubMed  Google Scholar 

  15. Wald RM, Redington AN, Pereira A, Provost YL, Paul NS, Oechslin EN, Silversides CK. Refining the assessment of pulmonary regurgitation in adults after tetralogy of Fallot repair: should we be measuring regurgitant fraction or regurgitant volume? Eur Heart J. 2009;30(3):356–61.

    Article  PubMed  Google Scholar 

  16. Johansson B, Babu-Narayan SV, Kilner PJ. The effects of breath-holding on pulmonary regurgitation measured by cardiovascular magnetic resonance velocity mapping. J Cardiovasc Magn Reson. 2009;11:1.

    Article  PubMed  Google Scholar 

  17. Davlouros PA, Kilner PJ, Hornung TS, Li W, Francis JM, Moon JC, Smith GC, Tat T, Pennell DJ, Gatzoulis MA. Right ventricular function in adults with repaired tetralogy of Fallot assessed with cardiovascular magnetic resonance imaging: detrimental role of right ventricular outflow aneurysms or akinesia and adverse right-to-left ventricular interaction. J Am Coll Cardiol. 2002;40(11):2044–52.

    Article  PubMed  Google Scholar 

  18. Geva T, Sandweiss BM, Gauvreau K, Lock JE, Powell AJ. Factors associated with impaired clinical status in long-term survivors of tetralogy of Fallot repair evaluated by magnetic resonance imaging. J Am Coll Cardiol. 2004;43(6):1068–74.

    Article  PubMed  Google Scholar 

  19. Bellenger NG, Davies LC, Francis JM, Coats AJ, Pennell DJ. Reduction in sample size for studies of remodeling in heart failure by the use of cardiovascular magnetic resonance. J Cardiovasc Magn Reson. 2000;2(4):271–8.

    Article  PubMed  CAS  Google Scholar 

  20. Winter MM, Bernink FJ, Groenink M, Bouma BJ, van Dijk AP, Helbing WA, Tijssen JG, Mulder BJ. 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 

  21. Hughes ML, Muthurangu V, Taylor A. Cardiovascular MR imaging—indications, techniques and protocols. Prog Pediatr Cardiol. 2010;28:3–10.

    Article  Google Scholar 

  22. Babu-Narayan SV, Bouzas B, Broberg CS, Kilner PJ. Interstudy reproducibility of right and left ventricular volume and mass measurements by cardiovascular magnetic resonance in repaired tetralogy of Fallot patients. J Cardiovasc Magn Reson. 2005;7(1):153.

    Google Scholar 

  23. Babu-Narayan SV, Uebing A, Davlouros PA, Kemp M, Davidson S, Dimopoulos K, Bayne S, Pennell DJ, Gibson DG, Flather M, Kilner PJ, Li W, Gatzoulis MA. Randomised trial of ramipril in repaired tetralogy of Fallot and pulmonary regurgitation: the APPROPRIATE study (Ace inhibitors for Potential PRevention Of the deleterious effects of Pulmonary Regurgitation In Adults with repaired TEtralogy of Fallot). Int J Cardiol. 2012;154(3):299–305.

    Article  PubMed  Google Scholar 

  24. 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 

  25. 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 

  26. van Straten A, Vliegen HW, Hazekamp MG, Bax JJ, Schoof PH, Ottenkamp J, van der Wall EE, de Roos A. Right ventricular function after pulmonary valve replacement in patients with tetralogy of Fallot. Radiology. 2004;233(3):824–9.

    Article  PubMed  Google Scholar 

  27. Vliegen HW, van Straten A, de Roos A, Roest AA, Schoof PH, Zwinderman AH, Ottenkamp J, van der Wall EE, Hazekamp MG. 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 

  28. Buechel ER, Dave HH, Kellenberger CJ, Dodge-Khatami A, Pretre R, Berger F, Bauersfeld U. 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 

  29. Khambadkone S, Coats L, Taylor A, Boudjemline Y, Derrick G, Tsang V, Cooper J, Muthurangu V, Hegde SR, Razavi RS, Pellerin D, Deanfield J, Bonhoeffer P. Percutaneous pulmonary valve implantation in humans: results in 59 consecutive patients. Circulation. 2005;112(8):1189–97.

    Article  PubMed  Google Scholar 

  30. Oosterhof T, van Straten A, Vliegen HW, Meijboom FJ, van Dijk AP, Spijkerboer AM, Bouma BJ, Zwinderman AH, Hazekamp MG, de Roos A, Mulder BJ. 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 

  31. Frigiola A, Tsang V, Bull C, Coats L, Khambadkone S, Derrick G, Mist B, Walker F, van Doorn C, Bonhoeffer P, Taylor AM. 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 

  32. Geva T, Gauvreau K, Powell AJ, Cecchin F, Rhodes J, Geva J, del Nido P. Randomized trial of pulmonary valve replacement with and without right ventricular remodeling surgery. Circulation. 2010;122(11 Suppl):S201–8.

    Article  PubMed  Google Scholar 

  33. Baumgartner H, Bonhoeffer P, De Groot NM, de Haan F, Deanfield JE, Galie N, Gatzoulis MA, Gohlke-Baerwolf C, Kaemmerer H, Kilner P, Meijboom F, Mulder BJ, Oechslin E, Oliver JM, Serraf A, Szatmari A, Thaulow E, Vouhe PR, Walma E. ESC guidelines for the management of grown-up congenital heart disease (new version 2010). Eur Heart J. 2010;31(23):2915–57.

    Article  PubMed  Google Scholar 

  34. Uebing A, Gibson DG, Babu-Narayan SV, Diller GP, Dimopoulos K, Goktekin O, Spence MS, Andersen K, Henein MY, Gatzoulis MA, Li W. Right ventricular mechanics and QRS duration in patients with repaired tetralogy of Fallot: implications of infundibular disease. Circulation. 2007;116(14):1532–9.

    Article  PubMed  Google Scholar 

  35. Babu-Narayan SV, Kilner PJ, Li W, Moon JC, Goktekin O, Davlouros PA, Khan M, Ho SY, Pennell DJ, Gatzoulis MA. 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 

  36. Wald RM, Haber I, Wald R, Valente AM, Powell AJ, Geva T. Effects of regional dysfunction and late gadolinium enhancement on global right ventricular function and exercise capacity in patients with repaired tetralogy of Fallot. Circulation. 2009;119(10):1370–7.

    Article  PubMed  Google Scholar 

  37. Bonello B, Kempny A, Uebing A, Li W, Kilner P, Diller G, Pennell D, Ernst S, Shore D, Babu-Narayan S. Right atrial area and right ventricular outflow tract akinetic region length predict sustained tachyarrhythmia in repaired tetralogy of Fallot. Eur Heart J Cardiovasc Imaging. 2012;13 Suppl 1:i40–1.

    Article  Google Scholar 

  38. Khairy P, Aboulhosn J, Gurvitz MZ, Opotowsky AR, Mongeon FP, Kay J, Valente AM, Earing MG, Lui G, Gersony DR, Cook S, Ting JG, Nickolaus MJ, Webb G, Landzberg MJ, Broberg CS. Arrhythmia burden in adults with surgically repaired tetralogy of Fallot: a multi-institutional study. Circulation. 2010;122(9):868–75.

    Article  PubMed  Google Scholar 

  39. Sridharan S, Derrick G, Deanfield J, Taylor AM. Assessment of differential branch pulmonary blood flow: a comparative study of phase contrast magnetic resonance imaging and radionuclide lung perfusion imaging. Heart. 2006;92(7):963–8.

    Article  PubMed  CAS  Google Scholar 

  40. Niwa K, Siu SC, Webb GD, Gatzoulis MA. Progressive aortic root dilatation in adults late after repair of tetralogy of Fallot. Circulation. 2002;106(11):1374–8.

    Article  PubMed  Google Scholar 

  41. Tan JL, Davlouros PA, McCarthy KP, Gatzoulis MA, Ho SY. Intrinsic histological abnormalities of aortic root and ascending aorta in tetralogy of Fallot: evidence of causative mechanism for aortic dilatation and aortopathy. Circulation. 2005;112(7):961–8.

    Article  PubMed  CAS  Google Scholar 

  42. Prasad SK, Soukias N, Hornung T, Khan M, Pennell DJ, Gatzoulis MA, Mohiaddin RH. Role of magnetic resonance angiography in the diagnosis of major aortopulmonary collateral arteries and partial anomalous pulmonary venous drainage. Circulation. 2004;109(2):207–14.

    Article  PubMed  Google Scholar 

  43. 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 

  44. 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 

  45. Gartner RD, Sutton NJ, Weinstein S, Spindola-Franco H, Haramati LB. MRI and computed tomography of cardiac and pulmonary complications of tetralogy of Fallot in adults. J Thorac Imaging. 2010;25(2): 183–90.

    PubMed  Google Scholar 

  46. Bradlow WM, Assomull R, Kilner PJ, Gibbs JS, Sheppard MN, Mohiaddin RH. Understanding late gadolinium enhancement in pulmonary hypertension. Circ Cardiovasc Imaging. 2010;3(4):501–3.

    Article  PubMed  Google Scholar 

  47. Tsai SF, Chan DP, Ro PS, Boettner B, Daniels CJ. Rate of inducible ventricular arrhythmia in adults with congenital heart disease. Am J Cardiol. 2010;106(5):730–6.

    Article  PubMed  Google Scholar 

  48. Witte KK, Pepper CB, Cowan JC, Thomson JD, English KM, Blackburn ME. Implantable cardioverter-defibrillator therapy in adult patients with tetralogy of Fallot. Europace. 2008;8:926–30.

    Article  Google Scholar 

  49. Yap SC, Roos-Hesselink JW, Hoendermis ES, Budts W, Vliegen HW, Mulder BJ, van Dijk AP, Schalij MJ, Drenthen W. Outcome of implantable cardioverter defibrillators in adults with congenital heart disease: a multi-centre study. Eur Heart J. 2007;28(15):1854–61.

    Article  PubMed  Google Scholar 

  50. Berul CI, Van Hare GF, Kertesz NJ, Dubin AM, Cecchin F, Collins KK, Cannon BC, Alexander ME, Triedman JK, Walsh EP, Friedman RA. Results of a multicenter retrospective implantable cardioverter-defibrillator registry of pediatric and congenital heart disease patients. J Am Coll Cardiol. 2008;51(17):1685–91.

    Article  PubMed  Google Scholar 

  51. Khairy P, Harris L, Landzberg MJ, Viswanathan S, Barlow A, Gatzoulis MA, Fernandes SM, Beauchesne L, Therrien J, Chetaille P, Gordon E, Vonder Muhll I, Cecchin F. Implantable cardioverter-defibrillators in tetralogy of Fallot. Circulation. 2008;117(3):363–70.

    Article  PubMed  Google Scholar 

  52. Schievano S, Petrini L, Migliavacca F, Coats L, Nordmeyer J, Lurz P, Khambadkone S, Taylor AM, Dubini G, Bonhoeffer P. Finite element analysis of stent deployment: understanding stent fracture in percutaneous pulmonary valve implantation. J Interv Cardiol. 2007;20(6):546–54.

    Article  PubMed  Google Scholar 

  53. Schievano S, Migliavacca F, Coats L, Khambadkone S, Carminati M, Wilson N, Deanfield JE, Bonhoeffer P, Taylor AM. 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 

  54. Schievano S, Coats L, Migliavacca F, Norman W, Frigiola A, Deanfield J, Bonhoeffer P, Taylor AM. 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 

  55. Gatzoulis MA, Clark AL, Cullen S, Newman CG, Redington AN. Right ventricular diastolic function 15 to 35 years after repair of tetralogy of Fallot. Restrictive physiology predicts superior exercise performance. Circulation. 1995;91(6):1775–81.

    Article  PubMed  CAS  Google Scholar 

Download references

Acknowledgments

Sonya Babu-Narayan is supported by the British Heart Foundation. This work was supported by the NIHR Cardiovascular Biomedical Research Unit of Royal Brompton and Harefield NHS Foundation Trust and Imperial College London. This report is independent research by the National Institute for Health Research Biomedical Research Unit Funding Scheme. The views expressed in this publication are those of the authors and not necessarily those of the NHS, the National Institute for Health Research or the Department of Health.

Author information

Authors and Affiliations

  1. NIHR Biomedical Research Unit, Cardiology Specialty Registrar, Royal Brompton and Harefield NHS Foundation Trust, London, UK

    Ee Ling Heng BSc, MRCP

  2. NIHR Cardiovascular Biomedical Research Unit, Royal Brompton Hospital and National Heart and Lung Institute, Imperial College London, Sydney Street, London, SW3 6NP, UK

    Sonya V. Babu-Narayan MBBS, BSc, MRCP, PhD

Authors
  1. Ee Ling Heng BSc, MRCP
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Sonya V. Babu-Narayan MBBS, BSc, MRCP, PhD
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Sonya V. Babu-Narayan MBBS, BSc, MRCP, PhD .

Editor information

Editors and Affiliations

  1. Department of Radiology, USC University Hospital Keck School of Medicine, Los Angeles, California, USA

    Farhood Saremi

Rights and permissions

Reprints and permissions

Copyright information

© 2014 Springer Science+Business Media New York

About this chapter

Cite this chapter

Heng, E.L., Babu-Narayan, S.V. (2014). Repaired Tetralogy of Fallot. In: Saremi, F. (eds) Cardiac CT and MR for Adult Congenital Heart Disease. Springer, New York, NY. https://doi.org/10.1007/978-1-4614-8875-0_9

Download citation

  • DOI: https://doi.org/10.1007/978-1-4614-8875-0_9

  • Published:

  • Publisher Name: Springer, New York, NY

  • Print ISBN: 978-1-4614-8874-3

  • Online ISBN: 978-1-4614-8875-0

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics

Search

Navigation