Tetralogy of Fallot

  • R. W. Sprengers
  • A. A. W. Roest
  • L. J. M. KroftEmail author
Part of the Medical Radiology book series (MEDRAD)


Tetralogy of Fallot (TOF) is the most common of cyanotic congenital heart diseases. In this chapter essential background information regarding etiology, clinical presentation, and treatment options in TOF are presented. The main focus is on imaging adult TOF, discussing the full spectrum of image modalities with special attention for CT and MRI. Findings in TOF, late outcome aspects, follow-up of complications, and the role of imaging in guiding therapy are discussed.


  1. Andreucci M et al (2014) Side effects of radiographic contrast media: pathogenesis, risk factors, and prevention. Biomed Res Int 2014:741018PubMedPubMedCentralGoogle Scholar
  2. Apitz C et al (2009) Tetralogy of Fallot. Lancet 374:1462–1471PubMedGoogle Scholar
  3. Van Arsdell GS et al (2000) What is the optimal age for repair of tetralogy of Fallot? Circulation 102(Suppl 3):III123–III129PubMedGoogle Scholar
  4. Van Arsdell G et al (2005) An apology for primary repair of tetralogy of Fallot. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu:128–131Google Scholar
  5. Babu-Narayan SV et al (2006) Ventricular fibrosis suggested by cardiovascular magnetic resonance in adults with repaired tetralogy of Fallot and its relationship to adverse markers of clinical outcome. Circulation 113(3):405–413PubMedGoogle Scholar
  6. Baumgartner B et al (2010) ESC guidelines for the management of grown-up congenital heart disease (new version 2010). Eur Heart J 31:2915–2957Google Scholar
  7. Van den Berg J et al (2007) Diastolic function in repaired tetralogy of Fallot at rest and during stress: assessment with MR imaging. Radiology 243(1):212–219PubMedGoogle Scholar
  8. Bernstein EJ et al (2012) Nephrogenic systemic fibrosis: a systemic fibrosing disease resulting from gadolinium exposure. Best Pract Res Clin Rheumatol 26(4):489–503Google Scholar
  9. Bertranou EG et al (1978) Life expectancy without surgery in tetralogy of Fallot. Am J Cardiol 42:458–466Google Scholar
  10. Berul CI et al (1997) Electrocardiographic markers of late sudden death risk in postoperative tetralogy of Fallot children. J Cardiovasc Electrophysiol 8:1349–1356PubMedGoogle Scholar
  11. Boshoff D et al (2006) A review of the options for treatment of major aortopulmonary collateral arteries in the setting of tetralogy of Fallot with pulmonary atresia. Cardiol Young 16(3):212–220PubMedGoogle Scholar
  12. Boxt LM (1999) Radiology of the right ventricle. Radiol Clin N Am 37(2):379–400PubMedGoogle Scholar
  13. Brenner DJ et al (2003) Cancer risks attributable to low doses of ionizing radiation: assessing what we really know. Proc Natl Acad Sci U S A 100(24):13761–13766PubMedPubMedCentralGoogle Scholar
  14. Broberg CS et al (2011) Prevalence of left ventricular systolic dysfunction in adults with repaired tetralogy of Fallot. Am J Cardiol 107:1215–1220PubMedGoogle Scholar
  15. Carminati M et al (2015) Echocardiographic assessment after surgical repair of tetralogy of Fallot. Front Pediatr 3:3PubMedPubMedCentralGoogle Scholar
  16. Carvalho JS et al (1992) Exercise capacity after complete repair of tetralogy of Fallot: deleterious effects of residual pulmonary regurgitation. Br Heart J 67:470–473PubMedPubMedCentralGoogle Scholar
  17. Chaturvedi RR et al (1997) Increased airway pressure and simulated branch pulmonary artery stenosis increase pulmonary regurgitation after repair of tetralogy of Fallot. Circulation 95:643–649PubMedGoogle Scholar
  18. Chowdhury UK et al (2006) Histopathology of the right ventricular outflow tract and its relationship to clinical outcomes and arrhythmias in patients with tetralogy of Fallot. J Thorac Cardiovasc Surg 132:270–277PubMedGoogle Scholar
  19. Coutu M et al (2004) Late myocardial revascularization in patients with tetralogy of Fallot. Ann Thorac Surg 77:1454–1455PubMedGoogle Scholar
  20. Cullen S et al (1995) Characterization of right ventricular diastolic performance after complete repair of tetralogy of Fallot. Restrictive physiology predicts slow postoperative recovery. Circulation 91:1782–1789PubMedGoogle Scholar
  21. Dobbs GA et al (1997) Aortic valve replacement after repair of pulmonary atresia and ventricular septal defect or tetralogy of Fallot. J Thorac Cardiovasc Surg 113(4):736–741Google Scholar
  22. Fathala A (2010) Quantitative lung perfusion scintigraphy in patients with congenital heart disease. Heart Views 11(3):109–114PubMedPubMedCentralGoogle Scholar
  23. Forghani R (2016) Adverse effects of gadolinium-based contrast agents: changes in practice patterns. Top Magn Reson Imaging 25(4):163–169PubMedGoogle Scholar
  24. Fratz S et al (2013) Guidelines and protocols for cardiovascular magnetic resonance in children and adults with congenital heart disease: SCMR expert consensus group on congenital heart disease. J Cardiovasc Magn Reson 15:51PubMedPubMedCentralGoogle Scholar
  25. Gatzoulis MA et al (1995a) Right ventricular diastolic function 15 to 35 years after repair of tetralogy of Fallot. Restrictive physiology predicts superior exercise performance. Circulation 91:1775–1781PubMedGoogle Scholar
  26. Gatzoulis MA et al (1995b) Mechanoelectrical interaction in tetralogy of Fallot. QRS prolongation relates to right ventricular size and predicts malignant ventricular arrhythmias and sudden death. Circulation 92(2):231–237PubMedGoogle Scholar
  27. Geva T et al (2004) Factors associated with impaired clinical status in long-term survivors of tetralogy of Fallot repair evaluated by magnetic resonance imaging. J Am Coll Cardiol 43:1068–1074PubMedGoogle Scholar
  28. Geva T (2011) Repaired tetralogy of Fallot: the roles of cardiovascular magnetic resonance in evaluating pathophysiology and for pulmonary valve replacement decision support. J Cardiovasc Magn Reson 13:9PubMedPubMedCentralGoogle Scholar
  29. Geva T (2013) Indications for pulmonary valve replacement in repaired tetralogy of Fallot: the quest continues. Circulation 128(17):1855–1857PubMedPubMedCentralGoogle Scholar
  30. Greil GF et al (2002) Coronary magnetic resonance angiography in adolescents and young adults with kawasaki disease. Circulation 105(8):908–911PubMedGoogle Scholar
  31. Grotenhuis et al. (2009) Aortic elasticity and size are associated with aortic regurgitation and left ventricular dysfunction in tetralogy of Fallot after pulmonary valve replacement. Heart 95:1931–1936PubMedGoogle Scholar
  32. Haider EA (2008) The boot-shaped heart sign. Radiology 246(1):328–329PubMedGoogle Scholar
  33. Al Habib HF et al (2010) Contemporary patterns of management of tetralogy of Fallot: data from the Society of Thoracic Surgeons database. Ann Thorac Surg 90:813–819PubMedGoogle Scholar
  34. Hamdan A et al (2011) A prospective study for comparison of MR and CT imaging for detection of coronary artery stenosis. JACC Cardiovasc Imaging 4(1):50–61PubMedGoogle Scholar
  35. Hiratzka LF et al (2010) ACCF/AHA/AATS/ACR/ASA/SCA/SCAI/SIR/STS/SVM guidelines for the diagnosis and management of patients with thoracic aortic disease: a report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines, American Association for Thoracic Surgery, American College of Radiology, American Stroke Association, Society of Cardiovascular Anesthesiologists, Society for Cardiovascular Angiography and Interventions, Society of Interventional Radiology, Society of Thoracic Surgeons, and Society for Vascular Medicine. Circulation 121(13):e266–e369PubMedGoogle Scholar
  36. Horwood L et al (2016) Magnetic resonance imaging in patients with cardiac implanted electronic devices: focus on contraindications to magnetic resonance imaging protocols. Europace. pii: euw122Google Scholar
  37. Van der Hulst AE et al (2010) Tetralogy of Fallot: 3D velocity-encoded MR imaging for evaluation of right ventricular valve flow and diastolic function in patients after correction. Radiology 256(3):724–734PubMedGoogle Scholar
  38. Van der Hulst AE et al (2011) Real-time three-dimensional echocardiography: segmental analysis of the right ventricle in patients with repaired tetralogy of fallot. J Am Soc Echocardiogr 24(11):1183–1190PubMedGoogle Scholar
  39. Hwang YM et al (2016) Cardiac implantable electronic device safety during magnetic resonance imaging. Korean Circ J 46(6):804–810PubMedPubMedCentralGoogle Scholar
  40. Iriart X et al (2009) Right ventricle three-dimensional echography in corrected tetralogy of Fallot: accuracy and variability. Eur J Echocardiogr 10(6):784–792PubMedGoogle Scholar
  41. Jahangiri M et al (1999) Does the modified Blalock-Taussig shunt cause growth of the contralateral pulmonary artery? Ann Thorac Surg 67(5):1397–1399PubMedGoogle Scholar
  42. Joemai RM et al (2008) Clinical evaluation of 64-slice CT assessment of global left ventricular function using automated cardiac phase selection. Circ J 72(4):641–646PubMedGoogle Scholar
  43. Johnson C (1965) Fallot’s tetralogy: a review of the radiological appearances in thirty-three cases. Clin Radiol 16:199–210PubMedGoogle Scholar
  44. Kaiser T et al (2008) Normal values for aortic diameters in children and adolescents—assessment in vivo by contrast-enhanced CMR-angiography. J Cardiovasc Magn Reson 10:56PubMedPubMedCentralGoogle Scholar
  45. Karl TR et al (1992) Tetralogy of Fallot: favorable outcome of nonneonatal transatrial, transpulmonary repair. Ann Thorac Surg 54:903–907PubMedGoogle Scholar
  46. Khairy P et al (2010) Arrhythmia burden in adults with surgically repaired tetralogy of Fallot: a multi- institutional study. Circulation 122:868–875PubMedGoogle Scholar
  47. Khoo NS et al (2009) Assessments of right ventricular volume and function using three-dimensional echocardiography in older children and adults with congenital heart disease: comparison with cardiac magnetic resonance imaging. J Am Soc Echocardiogr 22:1279–1288PubMedGoogle Scholar
  48. Kilner PJ et al (2010) Recommendations for cardiovascular magnetic resonance in adults with congenital heart disease from the perspective workings groups of the European Society of Cardiology. Eur Heart J 31:794–805PubMedPubMedCentralGoogle Scholar
  49. Knauth AL et al (2008) Ventricular size and function assessed by cardiac MRI predict major adverse clinical outcomes late after tetralogy of Fallot repair. Heart 94(2):211–216PubMedGoogle Scholar
  50. Koestenberger M et al (2011) Tricuspid annular plane systolic excursion and right ventricular ejection fraction in pediatric and adolescent patients with tetralogy of Fallot, patients with atrial septal defect, and age-matched normal subjects. Clin Res Cardiol 100(1):67–75PubMedGoogle Scholar
  51. Kolandaivelu A et al (2009) Cardiovascular magnetic resonance guided electrophysiology studies. J Cardiovasc Magn Reson 11(1):21PubMedPubMedCentralGoogle Scholar
  52. Lam YY et al (2007) Restrictive right ventricular physiology: its presence and symptomatic contribution in patients with pulmonary valvular stenosis. J Am Coll Cardiol 50(15):1491–1497PubMedGoogle Scholar
  53. Lanjewar C et al (2012) Aneurysmally dilated major aorto-pulmonary collateral in tetralogy of Fallot. Indian Heart J 64(2):196–197PubMedPubMedCentralGoogle Scholar
  54. Lembcke A et al (2005) Multislice computed tomography for preoperative evaluation of right ventricular volumes and function: comparison with magnetic resonance imaging. Ann Thorac Surg 79(4):1344–1351PubMedGoogle Scholar
  55. Maceira AM et al (2006a) Reference right ventricular systolic and diastolic function normalized to age, gender and body surface area from steady-state free precession cardiovascular magnetic resonance. Eur Heart J 27(23):2879–2888PubMedPubMedCentralGoogle Scholar
  56. Maceira AM et al (2006b) Normalized left ventricular systolic and diastolic function by steady state free precession cardiovascular magnetic resonance. J Cardiovasc Magn Reson 8(3):417–426PubMedGoogle Scholar
  57. McElhinney DB (2011) Stent fracture, valve dysfunction, and right ventricular outflow tract reintervention after transcatheter pulmonary valve implantation: patient-related and procedural risk factors in the US Melody Valve Trial. Circ Cardiovasc Interv 4(6):602–614PubMedGoogle Scholar
  58. Mercer-Rosa L et al (2012) Quantifying pulmonary regurgitation and right ventricular function in surgically repaired tetralogy of Fallot: a comparative analysis of echocardiography and magnetic resonance imaging. Circ Cardiovasc Imaging 5(5):637–643PubMedPubMedCentralGoogle Scholar
  59. Miyazaki O et al (2001) Sudden death due to rupture of major aortopulmonary collateral arteries in a patient with tetralogy of Fallot and pulmonary atresia. Emerg Radiol 8:293–296Google Scholar
  60. Myerson SG (2012) Heart valve disease: investigation by cardiovascular magnetic resonance. J Cardiovasc Magn Reson 14(1):7PubMedPubMedCentralGoogle Scholar
  61. Nishimura RA et al (1997) Evaluation of diastolic filling of left ventricle in health and disease: Doppler echocardiography is the clinician’s Rosetta Stone. J Am Coll Cardiol 30(1):8–18PubMedGoogle Scholar
  62. Niwa K et al (2002) Progressive aortic root dilatation in adults late after repair of tetralogy of Fallot. Circulation 106:1374–1378PubMedGoogle Scholar
  63. Nørgaard MA et al (2006) Major aorto-pulmonary collateral arteries of patients with pulmonary atresia and ventricular septal defect are dilated bronchial arteries. Eur J Cardiothorac Surg 29(5):653–658PubMedGoogle Scholar
  64. Oosterhof T et al (2005) Corrected tetralogy of Fallot: delayed enhancement in right ventricular outflow tract. Radiology 237(3):868–871PubMedGoogle Scholar
  65. Oosterhof T et al (2007) Preoperative thresholds for pulmonary valve replacement in patients with corrected tetralogy of Fallot using cardiovascular magnetic resonance. Circulation 116:545–551PubMedGoogle Scholar
  66. Pacheco Duro R et al (2010) Anatomophysiologic basis of tetralogy of Fallot and its clinical implications. Rev Port Cardiol 29(04):591–630Google Scholar
  67. Prieto LR (2005) Management of tetralogy of Fallot with pulmonary atresia. Images Paediatr Cardiol 7(3):24–42PubMedPubMedCentralGoogle Scholar
  68. Raman SV et al (2006) Multi-detector row cardiac computed tomography accurately quantifies right and left ventricular size and function compared with cardiac magnetic resonance. Am Heart J 151(3):736–744PubMedGoogle Scholar
  69. Rebergen SA et al (1993) Pulmonary regurgitation in the late postoperative follow-up of tetralogy of Fallot. Volumetric quantitation by nuclear magnetic resonance velocity mapping. Circulation 88:2257–2266PubMedGoogle Scholar
  70. Roes SD et al (2009) Flow assessment through four heart valves simultaneously using 3-dimensional 3-directional velocity-encoded magnetic resonance imaging with retrospective valve tracking in healthy volunteers and patients with valvular regurgitation. Investig Radiol 44(10):669–675Google Scholar
  71. Saremi F et al (2013) Right ventricular outflow tract imaging with CT and MRI: part 1, morphology. Am J Roentgenol 200:W39–W50Google Scholar
  72. Sharma A et al (2016) Ruptured aneurysm of major aortopulmonary collateral artery: management using amplatzer vascular plug. Cardiovasc Diagn Ther 6(3):274–277PubMedPubMedCentralGoogle Scholar
  73. Shimada YJ et al (2010) Accuracy of right ventricular volumes and function determined by three-dimensional echocardiography in comparison with magnetic resonance imaging: a meta-analysis study. J Am Soc Echocardiogr 23(9):943–953PubMedGoogle Scholar
  74. Shimazaki Y et al (1984) The natural history of isolated congenital pulmonary valve incompetence: surgical implications. Thorac Cardiovasc Surg 32:257–259PubMedGoogle Scholar
  75. Shinbane JS et al (2011) Magnetic resonance imaging in patients with cardiac pacemakers: era of “MR Conditional” designs. J Cardiovasc Magn Reson 13:63PubMedPubMedCentralGoogle Scholar
  76. Sommer RJ et al (2008) Pathophysiology of congenital heart disease in the adult. Part III: complex congenital heart disease. Circulation 117:1340–1350PubMedGoogle Scholar
  77. Strait JB et al (2012) Aging-associated cardiovascular changes and their relationship to heart failure. Heart Fail Clin 8(1):143–164PubMedPubMedCentralGoogle Scholar
  78. Tharakan JA et al (2014) Post cardiac surgery junctional ectopic tachycardia: a ‘Hit and Run’ tachyarrhythmia as yet unchecked. Ann Pediatr Cardiol 7(1):25–28PubMedPubMedCentralGoogle Scholar
  79. Thierrien J et al (2000) Pulmonary valve replacement in adults late after repair of tetralogy of Fallot: are we operating too late? J Am Coll Cardiol 36(5):1670Google Scholar
  80. Troughton RW et al (2014) Effect of B-type natriuretic peptide-guided treatment of chronic heart failure on total mortality and hospitalization: an individual patient meta-analysis. Eur Heart J 35(23):1559–1567PubMedPubMedCentralGoogle Scholar
  81. Valente AM et al (2014) Multimodality imaging guidelines for patients with repaired tetralogy of Fallot: A report from the American Society of Echocardiography. Developed in collaboration with the Society for Cardiovascular Magnetic Resonance and the Society for Pediatric Radiology. J Am Soc Echocardiogr 27:111–141PubMedGoogle Scholar
  82. Vogel-Claussen J et al (2006) Delayed enhancement MR imaging: utility in myocardial assessment. Radiographics 26(3):795–810PubMedGoogle Scholar
  83. Wald RM et al (2009) Effects of regional dysfunction and late gadolinium enhancement on global right ventricular function and exercise capacity in patients with repaired tetralogy of Fallot. Circulation 119(10):1370–1377PubMedPubMedCentralGoogle Scholar
  84. Wallerson DC et al (1987) Assessment of cardiac hemodynamics and valvular function by Doppler echocardiography. Bull N Y Acad Med 63(8):762–796PubMedPubMedCentralGoogle Scholar
  85. Warnes CA et al (2008) ACC/AHA 2008 guidelines for the management of adult 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 118:e714–e833PubMedPubMedCentralGoogle Scholar
  86. Weber OM et al (2006) MR evaluation of cardiovascular physiology in congenital heart disease: flow and function. J Cardiovasc Magn Reson 8(4):607–617PubMedGoogle Scholar
  87. Weisbord SD et al (2008) Incidence and outcomes of contrast-induced AKI following computed tomography. Clin J Am Soc Nephrol 3(5):1274–1281PubMedPubMedCentralGoogle Scholar
  88. Westhoff-Bleck M et al (2016) NT-proBNP indicates left ventricular impairment and adverse clinical outcome in patients with tetralogy of Fallot and pulmonary regurgitation. Can J Cardiol 32(10):1247.e29–1247.e36Google Scholar

Copyright information

© Springer International Publishing AG 2017

Authors and Affiliations

  • R. W. Sprengers
    • 1
  • A. A. W. Roest
    • 2
  • L. J. M. Kroft
    • 1
    Email author
  1. 1.Department of RadiologyLeiden University Medical CenterLeidenThe Netherlands
  2. 2.Department of Pediatric CardiologyLeiden University Medical CenterLeidenThe Netherlands

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