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Tetralogy of Fallot
Whitnee Hogan, MD; Michael D. Puchalski, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Clinical Issues

        • Diagnostic Checklist

          TERMINOLOGY

          • Abbreviations

            • Tetralogy of Fallot (ToF)
          • Definitions

            • ToF includes 4 components
              • Overriding aorta
              • Ventricular septal defect (VSD)
              • Right ventricular outflow tract (RVOT) obstruction
              • Right ventricle (RV) hypertrophy
            • Some categorize pulmonary atresia (PA) with VSD (PA-VSD) as severe form of ToF
              • PA-VSD may include major aortopulmonary collateral arteries (MAPCAs)

          IMAGING

          • General Features

            • Echocardiographic Findings

              • Imaging Recommendations

                DIFFERENTIAL DIAGNOSIS

                  PATHOLOGY

                  • General Features

                    • Staging, Grading, & Classification

                      • Gross Pathologic & Surgical Features

                        CLINICAL ISSUES

                        • Demographics

                          • Natural History & Prognosis

                            • Treatment

                              DIAGNOSTIC CHECKLIST

                              • Image Interpretation Pearls

                                Selected References

                                1. DeVore GR et al: Evaluation of fetal cardiac size and shape: a new screening tool to identify fetuses at risk for tetralogy of Fallot. J Ultrasound Med. ePub, 2021
                                2. De Robertis V et al: Tetralogy of Fallot and outlet ventricular septal defect with anterior malalignment detected at early fetal echocardiography. Fetal Diagn Ther. 1-7, 2020
                                3. Dorobantu DM et al: Surgical repair of tetralogy of Fallot with absent pulmonary valve: favorable long-term results. Semin Thorac Cardiovasc Surg. 31(4):847-9, 2019
                                4. Smith CA et al: Long-term outcomes of tetralogy of Fallot: a study from the pediatric cardiac care consortium. JAMA Cardiol. 4(1):34-41, 2019
                                5. Soquet J et al: A review of the management of pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries. Ann Thorac Surg. 108(2):601-12, 2019
                                6. Zhou J et al: Fetal pulmonary atresia with ventricular septal defect: features, associations, and outcome in fetuses with different pulmonary circulation supply types. Prenat Diagn. 39(12):1047-53, 2019
                                7. Axt-Fliedner R et al: Absent pulmonary valve syndrome - diagnosis, associations, and outcome in 71 prenatally diagnosed cases. Prenat Diagn. 37(8):812-9, 2017
                                8. Zhao Y et al: Prenatal and postnatal survival of fetal tetralogy of Fallot: a meta-analysis of perinatal outcomes and associated genetic disorders. J Ultrasound Med. 35(5):905-15, 2016
                                9. Devendran V et al: Tetralogy of Fallot with subarterial ventricular septal defect: surgical outcome in the current era. Ann Pediatr Cardiol. 8(1):4-9, 2015
                                10. Schellen C et al: Fetal MRI detects early alterations of brain development in tetralogy of Fallot. Am J Obstet Gynecol. 213(3):392.e1-7, 2015
                                11. Smith T et al: MicroRNAs in congenital heart disease. Ann Transl Med. 3(21):333, 2015
                                12. Arya B et al: Fetal echocardiographic measurements and the need for neonatal surgical intervention in tetralogy of Fallot. Pediatr Cardiol. 35(5):810-6, 2014
                                13. Fuller S: Tetralogy of Fallot and pulmonary valve replacement: timing and techniques in the asymptomatic patient. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 17(1):30-7, 2014
                                14. Qureshi MY et al: Importance of absent ductus arteriosus in tetralogy of Fallot with absent pulmonary valve syndrome. Tex Heart Inst J. 41(6):664-7, 2014
                                15. Hirji A et al: Outcomes of prenatally diagnosed tetralogy of Fallot: Implications for valve-sparing repair versus transannular patch. Can J Cardiol. 26(1):e1-6, 2010
                                16. Park CS et al: The long-term result of total repair for tetralogy of Fallot. Eur J Cardiothorac Surg. 38(3):311-7, 2010
                                17. Nørgaard MA et al: Absent pulmonary valve syndrome. Surgical and clinical outcome with long-term follow-up. Eur J Cardiothorac Surg. 29(5):682-7, 2006
                                18. Moon-Grady AJ et al: Value of clinical and echocardiographic features in predicting outcome in the fetus, infant, and child with tetralogy of Fallot with absent pulmonary valve complex. Am J Cardiol. 89(11):1280-5, 2002
                                Related Anatomy
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                                Related Differential Diagnoses
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                                References
                                Tables

                                Tables

                                KEY FACTS

                                • Terminology

                                  • Imaging

                                    • Clinical Issues

                                      • Diagnostic Checklist

                                        TERMINOLOGY

                                        • Abbreviations

                                          • Tetralogy of Fallot (ToF)
                                        • Definitions

                                          • ToF includes 4 components
                                            • Overriding aorta
                                            • Ventricular septal defect (VSD)
                                            • Right ventricular outflow tract (RVOT) obstruction
                                            • Right ventricle (RV) hypertrophy
                                          • Some categorize pulmonary atresia (PA) with VSD (PA-VSD) as severe form of ToF
                                            • PA-VSD may include major aortopulmonary collateral arteries (MAPCAs)

                                        IMAGING

                                        • General Features

                                          • Echocardiographic Findings

                                            • Imaging Recommendations

                                              DIFFERENTIAL DIAGNOSIS

                                                PATHOLOGY

                                                • General Features

                                                  • Staging, Grading, & Classification

                                                    • Gross Pathologic & Surgical Features

                                                      CLINICAL ISSUES

                                                      • Demographics

                                                        • Natural History & Prognosis

                                                          • Treatment

                                                            DIAGNOSTIC CHECKLIST

                                                            • Image Interpretation Pearls

                                                              Selected References

                                                              1. DeVore GR et al: Evaluation of fetal cardiac size and shape: a new screening tool to identify fetuses at risk for tetralogy of Fallot. J Ultrasound Med. ePub, 2021
                                                              2. De Robertis V et al: Tetralogy of Fallot and outlet ventricular septal defect with anterior malalignment detected at early fetal echocardiography. Fetal Diagn Ther. 1-7, 2020
                                                              3. Dorobantu DM et al: Surgical repair of tetralogy of Fallot with absent pulmonary valve: favorable long-term results. Semin Thorac Cardiovasc Surg. 31(4):847-9, 2019
                                                              4. Smith CA et al: Long-term outcomes of tetralogy of Fallot: a study from the pediatric cardiac care consortium. JAMA Cardiol. 4(1):34-41, 2019
                                                              5. Soquet J et al: A review of the management of pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries. Ann Thorac Surg. 108(2):601-12, 2019
                                                              6. Zhou J et al: Fetal pulmonary atresia with ventricular septal defect: features, associations, and outcome in fetuses with different pulmonary circulation supply types. Prenat Diagn. 39(12):1047-53, 2019
                                                              7. Axt-Fliedner R et al: Absent pulmonary valve syndrome - diagnosis, associations, and outcome in 71 prenatally diagnosed cases. Prenat Diagn. 37(8):812-9, 2017
                                                              8. Zhao Y et al: Prenatal and postnatal survival of fetal tetralogy of Fallot: a meta-analysis of perinatal outcomes and associated genetic disorders. J Ultrasound Med. 35(5):905-15, 2016
                                                              9. Devendran V et al: Tetralogy of Fallot with subarterial ventricular septal defect: surgical outcome in the current era. Ann Pediatr Cardiol. 8(1):4-9, 2015
                                                              10. Schellen C et al: Fetal MRI detects early alterations of brain development in tetralogy of Fallot. Am J Obstet Gynecol. 213(3):392.e1-7, 2015
                                                              11. Smith T et al: MicroRNAs in congenital heart disease. Ann Transl Med. 3(21):333, 2015
                                                              12. Arya B et al: Fetal echocardiographic measurements and the need for neonatal surgical intervention in tetralogy of Fallot. Pediatr Cardiol. 35(5):810-6, 2014
                                                              13. Fuller S: Tetralogy of Fallot and pulmonary valve replacement: timing and techniques in the asymptomatic patient. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 17(1):30-7, 2014
                                                              14. Qureshi MY et al: Importance of absent ductus arteriosus in tetralogy of Fallot with absent pulmonary valve syndrome. Tex Heart Inst J. 41(6):664-7, 2014
                                                              15. Hirji A et al: Outcomes of prenatally diagnosed tetralogy of Fallot: Implications for valve-sparing repair versus transannular patch. Can J Cardiol. 26(1):e1-6, 2010
                                                              16. Park CS et al: The long-term result of total repair for tetralogy of Fallot. Eur J Cardiothorac Surg. 38(3):311-7, 2010
                                                              17. Nørgaard MA et al: Absent pulmonary valve syndrome. Surgical and clinical outcome with long-term follow-up. Eur J Cardiothorac Surg. 29(5):682-7, 2006
                                                              18. Moon-Grady AJ et al: Value of clinical and echocardiographic features in predicting outcome in the fetus, infant, and child with tetralogy of Fallot with absent pulmonary valve complex. Am J Cardiol. 89(11):1280-5, 2002