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Tetralogy of Fallot
Randy R. Richardson, MD; Ryan A. Moore, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Clinical Issues

        TERMINOLOGY

        • Definitions

          • Tetralogy of Fallot (TOF): 4 heart defects in combination
            • Obstruction to pulmonary outflow via infundibular right ventricular outflow tract (RVOT) narrowing or pulmonary valve stenosis
            • Secondary RV hypertrophy (RVH) from ↑ right ventricle (RV) pressures/fixed subpulmonary obstruction
            • Anterior malalignment ventricular septal defect (VSD)
            • Aorta overriding VSD
          • Category: Cyanotic, normal heart size, ↓ pulmonary vascularity
            • Most common cyanotic congenital heart lesion
          • Hemodynamics: Obstruction to pulmonary blood flow via infundibular narrowing by anterior deviation of subpulmonary conal septum
            • Spectrum: Classic "blue tet," "pink tet," TOF with pulmonary atresia & major aortopulmonary collaterals (MAPCAs), TOF with absent pulmonary valve

        IMAGING

        • General Features

          • Radiographic Findings

            • CT Findings

              • MR Findings

                • Echocardiographic Findings

                  • Angiographic Findings

                    • Imaging Recommendations

                      DIFFERENTIAL DIAGNOSIS

                        PATHOLOGY

                        • General Features

                          • Gross Pathologic & Surgical Features

                            CLINICAL ISSUES

                            • Presentation

                              • Demographics

                                • Natural History & Prognosis

                                  • Treatment

                                    Selected References

                                    1. Koppel CJ et al: Coronary anomalies in tetralogy of Fallot - a meta-analysis. Int J Cardiol. 306:78-85, 2020
                                    2. Li VW et al: Ventricular myocardial deformation imaging of patients with repaired tetralogy of Fallot. J Am Soc Echocardiogr. 33(7):788-801, 2020
                                    3. Mohamed I et al: Assessment of disease progression in patients with repaired tetralogy of Fallot using cardiac magnetic resonance imaging: a systematic review. Heart Lung Circ. 29(11):1613-20, 2020
                                    4. Ojha V et al: Spectrum of changes on cardiac magnetic resonance in repaired tetralogy of Fallot: imaging according to surgical considerations. Clin Imaging. 69:102-14, 2020
                                    5. Rizk J et al: Magnetic resonance imaging risk factors for ventricular arrhythmias in tetralogy of Fallot. Pediatr Cardiol. 41(5):862-8, 2020
                                    6. Tous C et al: Ex vivo cardiovascular magnetic resonance diffusion weighted imaging in congenital heart disease, an insight into the microstructures of tetralogy of Fallot, biventricular and univentricular systemic right ventricle. J Cardiovasc Magn Reson. 22(1):69, 2020
                                    7. Apostolopoulou SC et al: Cardiovascular imaging approach in pre and postoperative tetralogy of Fallot. BMC Cardiovasc Disord. 19(1):7, 2019
                                    8. Egbe AC et al: Coronary artery disease in adults with tetralogy of Fallot. Congenit Heart Dis. 14(3):491-7, 2019
                                    9. Leonardi B et al: The role of 3D imaging in the follow-up of patients with repaired tetralogy of Fallot. Eur Rev Med Pharmacol Sci. 23(4):1698-709, 2019
                                    10. Larios G et al: Imaging in repaired tetralogy of Fallot with a focus on recent advances in echocardiography. Curr Opin Cardiol. 32(5):490-502, 2017
                                    11. Lapierre C et al: Tetralogy of Fallot: preoperative assessment with MR and CT imaging. Diagn Interv Imaging. 97(5):531-41, 2016
                                    12. Shin YR et al: Factors associated with progression of right ventricular enlargement and dysfunction after repair of tetralogy of Fallot based on serial cardiac magnetic resonance imaging. Eur J Cardiothorac Surg. 50(3):464-9, 2016
                                    13. Vaujois L et al: Imaging of postoperative tetralogy of Fallot repair. Diagn Interv Imaging. 97(5):549-60, 2016
                                    14. Wijesekera VA et al: Sequential right and left ventricular assessment in posttetralogy of Fallot patients with significant pulmonary regurgitation. Congenit Heart Dis. 11(6):606-14, 2016
                                    15. Chan FP et al: Computed tomography and magnetic resonance imaging in neonates with congenital cardiovascular disease. Semin Ultrasound CT MR. 36(2):146-60, 2015
                                    16. Preim U et al: Delayed enhancement imaging in a contemporary patient cohort following correction of tetralogy of Fallot. Cardiol Young. 25(7):1268-75, 2015
                                    17. Schelhorn J et al: Volumetric measurements in patients with corrected tetralogy of Fallot: comparison of short-axis versus axial cardiac MRI and echocardiography. Acta Radiol. 56(11):1315-22, 2015
                                    18. Wald RM et al: Cardiac magnetic resonance markers of progressive RV dilation and dysfunction after tetralogy of Fallot repair. Heart. 101(21):1724-30, 2015
                                    19. Lee C et al: Factors associated with right ventricular dilatation and dysfunction in patients with chronic pulmonary regurgitation after repair of tetralogy of Fallot: analysis of magnetic resonance imaging data from 218 patients. J Thorac Cardiovasc Surg. 148(6):2589-95, 2014
                                    20. Lewis MJ et al: Usefulness of magnetic resonance imaging to guide referral for pulmonary valve replacement in repaired tetralogy of Fallot. Am J Cardiol. 114(9):1406-11, 2014
                                    21. Sabate Rotes A et al: Long-term follow-up in repaired tetralogy of fallot: can deformation imaging help identify optimal timing of pulmonary valve replacement? J Am Soc Echocardiogr. 27(12):1305-10, 2014
                                    22. Sun HY et al: Fetal MRI correlates with postnatal CT angiogram assessment of pulmonary anatomy in tetralogy of Fallot with absent pulmonary valve. Congenit Heart Dis. 9(4):E105-9, 2014
                                    23. Chelliah A et al: Clinical utility of fetal magnetic resonance imaging in tetralogy of Fallot with absent pulmonary valve. Circulation. 127(6):757-9, 2013
                                    24. Villafañe J et al: Hot topics in tetralogy of Fallot. J Am Coll Cardiol. 62(23):2155-66, 2013
                                    25. Gartner RD et al: MRI and computed tomography of cardiac and pulmonary complications of tetralogy of fallot in adults. J Thorac Imaging. 25(2):183-90, 2010
                                    26. Kilner PJ et al: Recommendations for cardiovascular magnetic resonance in adults with congenital heart disease from the respective working groups of the European Society of Cardiology. Eur Heart J. 31(7):794-805, 2010
                                    27. McElhinney DB et al: Short- and medium-term outcomes after transcatheter pulmonary valve placement in the expanded multicenter US melody valve trial. Circulation. 122(5):507-16, 2010
                                    28. Puranik R et al: Congenital heart disease and multi-modality imaging. Heart Lung Circ. 19(3):133-44, 2010
                                    29. Wald RM et al: 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):1370-7, 2009
                                    30. Chen SJ et al: Coronary artery anatomy in children with congenital heart disease by computed tomography. Int J Cardiol. 120(3):363-70, 2007
                                    31. Raman SV et al: Usefulness of multidetector row computed tomography to quantify right ventricular size and function in adults with either tetralogy of Fallot or transposition of the great arteries. Am J Cardiol. 95(5):683-6, 2005
                                    32. van Straten A et al: Right ventricular function late after total repair of tetralogy of Fallot. Eur Radiol. 15(4):702-7, 2005
                                    33. Kang IS et al: Differential regurgitation in branch pulmonary arteries after repair of tetralogy of Fallot: a phase-contrast cine magnetic resonance study. Circulation. 107(23):2938-43, 2003
                                    34. Nieman K et al: Coronary anomaly imaging by multislice computed tomography in corrected tetralogy of Fallot. Heart. 89(6):664, 2003
                                    35. Uebing A et al: Influence of the pulmonary annulus diameter on pulmonary regurgitation and right ventricular pressure load after repair of tetralogy of Fallot. Heart. 88(5):510-4, 2002
                                    Related Anatomy
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                                    Related Differential Diagnoses
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                                    References
                                    Tables

                                    Tables

                                    KEY FACTS

                                    • Terminology

                                      • Imaging

                                        • Clinical Issues

                                          TERMINOLOGY

                                          • Definitions

                                            • Tetralogy of Fallot (TOF): 4 heart defects in combination
                                              • Obstruction to pulmonary outflow via infundibular right ventricular outflow tract (RVOT) narrowing or pulmonary valve stenosis
                                              • Secondary RV hypertrophy (RVH) from ↑ right ventricle (RV) pressures/fixed subpulmonary obstruction
                                              • Anterior malalignment ventricular septal defect (VSD)
                                              • Aorta overriding VSD
                                            • Category: Cyanotic, normal heart size, ↓ pulmonary vascularity
                                              • Most common cyanotic congenital heart lesion
                                            • Hemodynamics: Obstruction to pulmonary blood flow via infundibular narrowing by anterior deviation of subpulmonary conal septum
                                              • Spectrum: Classic "blue tet," "pink tet," TOF with pulmonary atresia & major aortopulmonary collaterals (MAPCAs), TOF with absent pulmonary valve

                                          IMAGING

                                          • General Features

                                            • Radiographic Findings

                                              • CT Findings

                                                • MR Findings

                                                  • Echocardiographic Findings

                                                    • Angiographic Findings

                                                      • Imaging Recommendations

                                                        DIFFERENTIAL DIAGNOSIS

                                                          PATHOLOGY

                                                          • General Features

                                                            • Gross Pathologic & Surgical Features

                                                              CLINICAL ISSUES

                                                              • Presentation

                                                                • Demographics

                                                                  • Natural History & Prognosis

                                                                    • Treatment

                                                                      Selected References

                                                                      1. Koppel CJ et al: Coronary anomalies in tetralogy of Fallot - a meta-analysis. Int J Cardiol. 306:78-85, 2020
                                                                      2. Li VW et al: Ventricular myocardial deformation imaging of patients with repaired tetralogy of Fallot. J Am Soc Echocardiogr. 33(7):788-801, 2020
                                                                      3. Mohamed I et al: Assessment of disease progression in patients with repaired tetralogy of Fallot using cardiac magnetic resonance imaging: a systematic review. Heart Lung Circ. 29(11):1613-20, 2020
                                                                      4. Ojha V et al: Spectrum of changes on cardiac magnetic resonance in repaired tetralogy of Fallot: imaging according to surgical considerations. Clin Imaging. 69:102-14, 2020
                                                                      5. Rizk J et al: Magnetic resonance imaging risk factors for ventricular arrhythmias in tetralogy of Fallot. Pediatr Cardiol. 41(5):862-8, 2020
                                                                      6. Tous C et al: Ex vivo cardiovascular magnetic resonance diffusion weighted imaging in congenital heart disease, an insight into the microstructures of tetralogy of Fallot, biventricular and univentricular systemic right ventricle. J Cardiovasc Magn Reson. 22(1):69, 2020
                                                                      7. Apostolopoulou SC et al: Cardiovascular imaging approach in pre and postoperative tetralogy of Fallot. BMC Cardiovasc Disord. 19(1):7, 2019
                                                                      8. Egbe AC et al: Coronary artery disease in adults with tetralogy of Fallot. Congenit Heart Dis. 14(3):491-7, 2019
                                                                      9. Leonardi B et al: The role of 3D imaging in the follow-up of patients with repaired tetralogy of Fallot. Eur Rev Med Pharmacol Sci. 23(4):1698-709, 2019
                                                                      10. Larios G et al: Imaging in repaired tetralogy of Fallot with a focus on recent advances in echocardiography. Curr Opin Cardiol. 32(5):490-502, 2017
                                                                      11. Lapierre C et al: Tetralogy of Fallot: preoperative assessment with MR and CT imaging. Diagn Interv Imaging. 97(5):531-41, 2016
                                                                      12. Shin YR et al: Factors associated with progression of right ventricular enlargement and dysfunction after repair of tetralogy of Fallot based on serial cardiac magnetic resonance imaging. Eur J Cardiothorac Surg. 50(3):464-9, 2016
                                                                      13. Vaujois L et al: Imaging of postoperative tetralogy of Fallot repair. Diagn Interv Imaging. 97(5):549-60, 2016
                                                                      14. Wijesekera VA et al: Sequential right and left ventricular assessment in posttetralogy of Fallot patients with significant pulmonary regurgitation. Congenit Heart Dis. 11(6):606-14, 2016
                                                                      15. Chan FP et al: Computed tomography and magnetic resonance imaging in neonates with congenital cardiovascular disease. Semin Ultrasound CT MR. 36(2):146-60, 2015
                                                                      16. Preim U et al: Delayed enhancement imaging in a contemporary patient cohort following correction of tetralogy of Fallot. Cardiol Young. 25(7):1268-75, 2015
                                                                      17. Schelhorn J et al: Volumetric measurements in patients with corrected tetralogy of Fallot: comparison of short-axis versus axial cardiac MRI and echocardiography. Acta Radiol. 56(11):1315-22, 2015
                                                                      18. Wald RM et al: Cardiac magnetic resonance markers of progressive RV dilation and dysfunction after tetralogy of Fallot repair. Heart. 101(21):1724-30, 2015
                                                                      19. Lee C et al: Factors associated with right ventricular dilatation and dysfunction in patients with chronic pulmonary regurgitation after repair of tetralogy of Fallot: analysis of magnetic resonance imaging data from 218 patients. J Thorac Cardiovasc Surg. 148(6):2589-95, 2014
                                                                      20. Lewis MJ et al: Usefulness of magnetic resonance imaging to guide referral for pulmonary valve replacement in repaired tetralogy of Fallot. Am J Cardiol. 114(9):1406-11, 2014
                                                                      21. Sabate Rotes A et al: Long-term follow-up in repaired tetralogy of fallot: can deformation imaging help identify optimal timing of pulmonary valve replacement? J Am Soc Echocardiogr. 27(12):1305-10, 2014
                                                                      22. Sun HY et al: Fetal MRI correlates with postnatal CT angiogram assessment of pulmonary anatomy in tetralogy of Fallot with absent pulmonary valve. Congenit Heart Dis. 9(4):E105-9, 2014
                                                                      23. Chelliah A et al: Clinical utility of fetal magnetic resonance imaging in tetralogy of Fallot with absent pulmonary valve. Circulation. 127(6):757-9, 2013
                                                                      24. Villafañe J et al: Hot topics in tetralogy of Fallot. J Am Coll Cardiol. 62(23):2155-66, 2013
                                                                      25. Gartner RD et al: MRI and computed tomography of cardiac and pulmonary complications of tetralogy of fallot in adults. J Thorac Imaging. 25(2):183-90, 2010
                                                                      26. Kilner PJ et al: Recommendations for cardiovascular magnetic resonance in adults with congenital heart disease from the respective working groups of the European Society of Cardiology. Eur Heart J. 31(7):794-805, 2010
                                                                      27. McElhinney DB et al: Short- and medium-term outcomes after transcatheter pulmonary valve placement in the expanded multicenter US melody valve trial. Circulation. 122(5):507-16, 2010
                                                                      28. Puranik R et al: Congenital heart disease and multi-modality imaging. Heart Lung Circ. 19(3):133-44, 2010
                                                                      29. Wald RM et al: 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):1370-7, 2009
                                                                      30. Chen SJ et al: Coronary artery anatomy in children with congenital heart disease by computed tomography. Int J Cardiol. 120(3):363-70, 2007
                                                                      31. Raman SV et al: Usefulness of multidetector row computed tomography to quantify right ventricular size and function in adults with either tetralogy of Fallot or transposition of the great arteries. Am J Cardiol. 95(5):683-6, 2005
                                                                      32. van Straten A et al: Right ventricular function late after total repair of tetralogy of Fallot. Eur Radiol. 15(4):702-7, 2005
                                                                      33. Kang IS et al: Differential regurgitation in branch pulmonary arteries after repair of tetralogy of Fallot: a phase-contrast cine magnetic resonance study. Circulation. 107(23):2938-43, 2003
                                                                      34. Nieman K et al: Coronary anomaly imaging by multislice computed tomography in corrected tetralogy of Fallot. Heart. 89(6):664, 2003
                                                                      35. Uebing A et al: Influence of the pulmonary annulus diameter on pulmonary regurgitation and right ventricular pressure load after repair of tetralogy of Fallot. Heart. 88(5):510-4, 2002