Keywords
Arrhythmias
Congenital heart disease
Lifelong care
Long-term outcomes
pulmonary regurgitation
Tetralogy of Fallot
Congenital heart disease
Lifelong care
Long-term outcomes
pulmonary regurgitation
Tetralogy of Fallot
How to Cite
Guli, A., Xhepa, S., & Veshti, A. (2026). Contemporary Management and Long-Term Outcomes of Tetralogy of Fallot: A Narrative Literature Review. Albanian Journal of Trauma and Emergency Surgery, 10(2), 2126-2135. https://doi.org/10.32391/ajtes.v10i2.542
Abstract
Background: Tetralogy of Fallot (TOF) remains the most common cyanotic congenital heart disease and a cornerstone of congenital cardiology. While advances in surgical repair have dramatically improved survival, the condition increasingly presents as a lifelong clinical challenge characterized by evolving complications and ongoing care needs.
This review aims to synthesize current evidence on contemporary management strategies and long-term outcomes in patients with TOF, with particular focus on surgical approaches, late complications, and lifelong follow-up.
Design and Data Sources: A narrative literature review was conducted using a structured search of PubMed, Scopus, Web of Science, and Google Scholar for studies published between 2016 and 2026. Eligible studies included clinical cohorts, registry analyses, systematic reviews, meta-analyses, and international guidelines addressing TOF management and outcomes A thematic narrative synthesis approach was applied to integrate findings across heterogeneous studies. In total, 36 studies met the inclusion criteria and were included in the final synthesis.
Results: Despite excellent survival after repair, long-term morbidity remains significant, driven by right ventricular dysfunction, pulmonary regurgitation, and arrhythmias. Management is individualized, with cardiac magnetic resonance central to follow-up. Neurodevelopment and quality of life are increasingly recognized, while disparities in access to care continue to influence outcomes.
Conclusion: TOF should be regarded as a chronic condition requiring coordinated, multidisciplinary, and lifelong management. Optimizing long-term outcomes will depend on improved risk stratification, timely intervention, and equitable access to specialized care.
References
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21. A. M. Qureshi et al., “Comparison of management strategies for neonates with symptomatic tetralogy of Fallot and weight <2.5 kg,” J. Thorac. Cardiovasc. Surg., vol. 163, no. 1, pp. 192-207.e3, Jan. 2022, doi: 10.1016/j.jtcvs.2021.01.100.
22. X. T. Ye et al., “Young infants with symptomatic tetralogy of Fallot: Shunt or primary repair?,” JTCVS Open, vol. 19, pp. 241–256, Jun. 2024, doi: 10.1016/j.xjon.2024.04.003.
23. I. F. Martins, I. C. Doles, N. J. M. Bravo-Valenzuela, A. O. R. dos Santos, and M. S. P. Varella, “When is the Best Time for Corrective Surgery in Patients with Tetralogy of Fallot between 0 and 12 Months of Age?,” Braz. J. Cardiovasc. Surg., vol. 33, no. 5, 2018, doi: 10.21470/1678-9741-2018-0019.
24. A. B. Ho, T. Bharucha, E. Jones, J. Thuraisingham, M. Kaarne, and N. Viola, “Primary surgical repair of tetralogy of Fallot at under three months of age,” Asian Cardiovasc. Thorac. Ann., vol. 26, no. 7, pp. 529–534, Sep. 2018, doi: 10.1177/0218492318803037.
25. M. A. Law et al., “Palliation Strategy to Achieve Complete Repair in Symptomatic Neonates with Tetralogy of Fallot,” Pediatr. Cardiol., vol. 43, no. 7, pp. 1587–1598, Oct. 2022, doi: 10.1007/s00246-022-02886-0.
26. M. Giacobbe et al., “Outcome of Tetralogy of Fallot Through Initial Palliation and Surgical Repair,” Pediatr. Cardiol., Sep. 2025, doi: 10.1007/s00246-025-04021-1.
27. N. S. Clarke et al., “Contemporary Patterns of Care in Tetralogy of Fallot: Analysis of The Society of Thoracic Surgeons Data,” Ann. Thorac. Surg., vol. 116, no. 4, pp. 768–775, Oct. 2023, doi: 10.1016/j.athoracsur.2023.05.035.
28. A. C. Egbe et al., “Left Ventricular Systolic Dysfunction and Cardiovascular Outcomes in Tetralogy of Fallot: Systematic Review and Meta-analysis,” Canadian Journal of Cardiology, vol. 35, no. 12, pp. 1784–1790, Dec. 2019, doi: 10.1016/j.cjca.2019.07.634.
29. L.-O. Roy et al., “Outcomes After Pediatric Pulmonary Valve Replacement in Patients With Tetralogy of Fallot,” Canadian Journal of Cardiology, vol. 40, no. 12, pp. 2461–2472, Dec. 2024, doi: 10.1016/j.cjca.2024.06.009.
30. J. A. Meca Aguirrezabalaga, J. Silva Guisasola, R. Díaz Méndez, A. E. Escalera Veizaga, and D. Hernández-Vaquero Panizo, “Pulmonary regurgitation after repaired tetralogy of Fallot: surgical versus percutaneous treatment,” Ann. Transl. Med., vol. 8, no. 15, pp. 967–967, Aug. 2020, doi: 10.21037/atm.2020.03.81.
31. A. C. Egbe, S. Vallabhajosyula, and H. M. Connolly, “Trends and outcomes of pulmonary valve replacement in tetralogy of Fallot,” Int. J. Cardiol., vol. 299, pp. 136–139, Jan. 2020, doi: 10.1016/j.ijcard.2019.07.063.
32. G. F. L. Kapel et al., “Arrhythmogenic anatomical isthmuses identified by electroanatomical mapping are the substrate for ventricular tachycardia in repaired tetralogy of Fallot,” Eur. Heart J., p. ehw202, May 2016, doi: 10.1093/eurheartj/ehw202.
33. C. Brouwer, G. F. L. Kapel, M. R. M. Jongbloed, M. J. Schalij, M. de Riva Silva, and K. Zeppenfeld, “Noninvasive Identification of Ventricular Tachycardia–Related Anatomical Isthmuses in Repaired Tetralogy of Fallot,” JACC Clin. Electrophysiol., vol. 4, no. 10, pp. 1308–1318, Oct. 2018, doi: 10.1016/j.jacep.2018.06.017.
34. A. M. Valente and T. Geva, “How to Image Repaired Tetralogy of Fallot,” Circ. Cardiovasc. Imaging, vol. 10, no. 5, May 2017, doi: 10.1161/CIRCIMAGING.116.004270.
35. C. J. Sánchez Ramírez and L. Pérez de Isla, “Tetralogy of Fallot: cardiac imaging evaluation,” Ann. Transl. Med., vol. 8, no. 15, pp. 966–966, Aug. 2020, doi: 10.21037/atm.2020.02.18.
36. J. P. Woo, D. B. McElhinney, and G. K. Lui, “The challenges of an aging tetralogy of Fallot population,” Expert Rev. Cardiovasc. Ther., vol. 19, no. 7, pp. 581–593, Jul. 2021, doi: 10.1080/14779072.2021.1940960.
37. E. Prifti, “Repair of complete atrioventricular septal defect with tetralogy of Fallot,” Transl. Pediatr., vol. 5, no. 1, pp. 1–7, Jan. 2017, doi: 10.21037/tp.2017.01.01.
38. S. Yang et al., “Impact of timing on in-patient outcomes of complete repair of tetralogy of Fallot in infancy: an analysis of the United States National Inpatient 2005–2011 database,” BMC Cardiovasc. Disord., vol. 19, no. 1, p. 46, Dec. 2019, doi: 10.1186/s12872-019-0999-1.
39. C. P. Callahan et al., “Early Outcomes for Management of Atrioventricular Septal Defect—Tetralogy of Fallot in the Last Decade: A Congenital Heart Surgeons’ Society Study,” World J. Pediatr. Congenit. Heart Surg., vol. 16, no. 2, pp. 262–272, Mar. 2025, doi: 10.1177/21501351241293158.
2. H. Baumgartner et al., “2020 ESC Guidelines for the management of adult congenital heart disease,” Eur. Heart J., vol. 42, no. 6, pp. 563–645, Feb. 2021, doi: 10.1093/eurheartj/ehaa554.
3. S. Ishigami et al., “Long-term outcomes of tetralogy of Fallot repair: A 30-year experience with 960 patients,” J. Thorac. Cardiovasc. Surg., vol. 167, no. 1, pp. 289-302.e11, Jan. 2024, doi: 10.1016/j.jtcvs.2023.04.015.
4. C. A. Smith et al., “Long-term Outcomes of Tetralogy of Fallot,” JAMA Cardiol., vol. 4, no. 1, p. 34, Jan. 2019, doi: 10.1001/jamacardio.2018.4255.
5. S. Blais et al., “The 30-Year Outcomes of Tetralogy of Fallot According to Native Anatomy and Genetic Conditions,” Canadian Journal of Cardiology, vol. 37, no. 6, pp. 877–886, Jun. 2021, doi: 10.1016/j.cjca.2020.10.002.
6. T. Geva et al., “Long-Term Management of Right Ventricular Outflow Tract Dysfunction in Repaired Tetralogy of Fallot: A Scientific Statement From the American Heart Association,” Circulation, vol. 150, no. 25, Dec. 2024, doi: 10.1161/CIR.0000000000001291.
7. F.-P. Mongeon et al., “Pulmonary Valve Replacement for Pulmonary Regurgitation in Adults With Tetralogy of Fallot: A Meta-analysis—A Report for the Writing Committee of the 2019 Update of the Canadian Cardiovascular Society Guidelines for the Management of Adults With Congenital Heart Disease,” Canadian Journal of Cardiology, vol. 35, no. 12, pp. 1772–1783, Dec. 2019, doi: 10.1016/j.cjca.2019.08.031.
8. A. S. Mueller, D. M. McDonald, H. S. Singh, and J. N. Ginns, “Heart failure in adult congenital heart disease: tetralogy of Fallot,” Heart Fail. Rev., vol. 25, no. 4, pp. 583–598, Jul. 2020, doi: 10.1007/s10741-019-09903-0.
9. E. V. Krieger et al., “Arrhythmias in Repaired Tetralogy of Fallot: A Scientific Statement From the American Heart Association,” Circ. Arrhythm. Electrophysiol., vol. 15, no. 11, Nov. 2022, doi: 10.1161/HAE.0000000000000084.
10. E. Sood et al., “Neurodevelopmental Outcomes for Individuals With Congenital Heart Disease: Updates in Neuroprotection, Risk-Stratification, Evaluation, and Management: A Scientific Statement From the American Heart Association,” Circulation, vol. 149, no. 13, Mar. 2024, doi: 10.1161/CIR.0000000000001211.
11. Kordopati-Zilou et al., “Neurodevelopmental Outcomes in Tetralogy of Fallot: A Systematic Review,” Children, vol. 9, no. 2, p. 264, Feb. 2022, doi: 10.3390/children9020264.
12. G. T. Nicholson et al., “Health-Related Quality of Life After Neonatal Treatment of Symptomatic Tetralogy of Fallot: Insights from the Congenital Cardiac Research Collaborative,” Pediatr. Cardiol., vol. 46, no. 8, pp. 2303–2314, Dec. 2025, doi: 10.1007/s00246-024-03650-2.
13. N. Dib et al., “Tetralogy of Fallot in Low- and Middle-Income Countries,” CJC Pediatric and Congenital Heart Disease, vol. 3, no. 2, pp. 67–73, Apr. 2024, doi: 10.1016/j.cjcpc.2023.12.002.
14. N. Halfon and M. Hochstein, “Life Course Health Development: An Integrated Framework for Developing Health, Policy, and Research,” Milbank Q., vol. 80, no. 3, pp. 433–479, Sep. 2002, doi: 10.1111/1468-0009.00019.
15. M. Dennis, B. Moore, I. Kotchetkova, L. Pressley, R. Cordina, and D. S. Celermajer, “Adults with repaired tetralogy: low mortality but high morbidity up to middle age,” Open Heart, vol. 4, no. 1, p. e000564, Mar. 2017, doi: 10.1136/openhrt-2016-000564.
16. G. L. Engel, “THE BIOPSYCHOSOCIAL MODEL AND THE EDUCATION OF HEALTH PROFESSIONALS*†,” Ann. N. Y. Acad. Sci., vol. 310, no. 1, pp. 169–181, Jun. 1978, doi: 10.1111/j.1749-6632.1978.tb22070.x.
17. J. Granton and D. Cheng, “Risk Stratification Models for Cardiac Surgery,” Semin. Cardiothorac. Vasc. Anesth., vol. 12, no. 3, pp. 167–174, Sep. 2008, doi: 10.1177/1089253208323681.
18. R. E. Glasgow, C. Tracy Orleans, E. H. Wagner, S. J. Curry, and L. I. Solberg, “Does the Chronic Care Model Serve Also as a Template for Improving Prevention?,” Milbank Q., vol. 79, no. 4, pp. 579–612, Dec. 2001, doi: 10.1111/1468-0009.00222.
19. A. L. DeWaters, E. L. Miller, P. Haidet, and J. D. Gonzalo, “Systems-Based Practice: Expert Perspectives on the Origin and Evolution of an Ambiguous Competency,” Academic Medicine, vol. 99, no. 4, pp. 424–429, Apr. 2024, doi: 10.1097/ACM.0000000000005478.
20. B. H. Goldstein et al., “Comparison of Management Strategies for Neonates With Symptomatic Tetralogy of Fallot,” J. Am. Coll. Cardiol., vol. 77, no. 8, pp. 1093–1106, Mar. 2021, doi: 10.1016/j.jacc.2020.12.048.
21. A. M. Qureshi et al., “Comparison of management strategies for neonates with symptomatic tetralogy of Fallot and weight <2.5 kg,” J. Thorac. Cardiovasc. Surg., vol. 163, no. 1, pp. 192-207.e3, Jan. 2022, doi: 10.1016/j.jtcvs.2021.01.100.
22. X. T. Ye et al., “Young infants with symptomatic tetralogy of Fallot: Shunt or primary repair?,” JTCVS Open, vol. 19, pp. 241–256, Jun. 2024, doi: 10.1016/j.xjon.2024.04.003.
23. I. F. Martins, I. C. Doles, N. J. M. Bravo-Valenzuela, A. O. R. dos Santos, and M. S. P. Varella, “When is the Best Time for Corrective Surgery in Patients with Tetralogy of Fallot between 0 and 12 Months of Age?,” Braz. J. Cardiovasc. Surg., vol. 33, no. 5, 2018, doi: 10.21470/1678-9741-2018-0019.
24. A. B. Ho, T. Bharucha, E. Jones, J. Thuraisingham, M. Kaarne, and N. Viola, “Primary surgical repair of tetralogy of Fallot at under three months of age,” Asian Cardiovasc. Thorac. Ann., vol. 26, no. 7, pp. 529–534, Sep. 2018, doi: 10.1177/0218492318803037.
25. M. A. Law et al., “Palliation Strategy to Achieve Complete Repair in Symptomatic Neonates with Tetralogy of Fallot,” Pediatr. Cardiol., vol. 43, no. 7, pp. 1587–1598, Oct. 2022, doi: 10.1007/s00246-022-02886-0.
26. M. Giacobbe et al., “Outcome of Tetralogy of Fallot Through Initial Palliation and Surgical Repair,” Pediatr. Cardiol., Sep. 2025, doi: 10.1007/s00246-025-04021-1.
27. N. S. Clarke et al., “Contemporary Patterns of Care in Tetralogy of Fallot: Analysis of The Society of Thoracic Surgeons Data,” Ann. Thorac. Surg., vol. 116, no. 4, pp. 768–775, Oct. 2023, doi: 10.1016/j.athoracsur.2023.05.035.
28. A. C. Egbe et al., “Left Ventricular Systolic Dysfunction and Cardiovascular Outcomes in Tetralogy of Fallot: Systematic Review and Meta-analysis,” Canadian Journal of Cardiology, vol. 35, no. 12, pp. 1784–1790, Dec. 2019, doi: 10.1016/j.cjca.2019.07.634.
29. L.-O. Roy et al., “Outcomes After Pediatric Pulmonary Valve Replacement in Patients With Tetralogy of Fallot,” Canadian Journal of Cardiology, vol. 40, no. 12, pp. 2461–2472, Dec. 2024, doi: 10.1016/j.cjca.2024.06.009.
30. J. A. Meca Aguirrezabalaga, J. Silva Guisasola, R. Díaz Méndez, A. E. Escalera Veizaga, and D. Hernández-Vaquero Panizo, “Pulmonary regurgitation after repaired tetralogy of Fallot: surgical versus percutaneous treatment,” Ann. Transl. Med., vol. 8, no. 15, pp. 967–967, Aug. 2020, doi: 10.21037/atm.2020.03.81.
31. A. C. Egbe, S. Vallabhajosyula, and H. M. Connolly, “Trends and outcomes of pulmonary valve replacement in tetralogy of Fallot,” Int. J. Cardiol., vol. 299, pp. 136–139, Jan. 2020, doi: 10.1016/j.ijcard.2019.07.063.
32. G. F. L. Kapel et al., “Arrhythmogenic anatomical isthmuses identified by electroanatomical mapping are the substrate for ventricular tachycardia in repaired tetralogy of Fallot,” Eur. Heart J., p. ehw202, May 2016, doi: 10.1093/eurheartj/ehw202.
33. C. Brouwer, G. F. L. Kapel, M. R. M. Jongbloed, M. J. Schalij, M. de Riva Silva, and K. Zeppenfeld, “Noninvasive Identification of Ventricular Tachycardia–Related Anatomical Isthmuses in Repaired Tetralogy of Fallot,” JACC Clin. Electrophysiol., vol. 4, no. 10, pp. 1308–1318, Oct. 2018, doi: 10.1016/j.jacep.2018.06.017.
34. A. M. Valente and T. Geva, “How to Image Repaired Tetralogy of Fallot,” Circ. Cardiovasc. Imaging, vol. 10, no. 5, May 2017, doi: 10.1161/CIRCIMAGING.116.004270.
35. C. J. Sánchez Ramírez and L. Pérez de Isla, “Tetralogy of Fallot: cardiac imaging evaluation,” Ann. Transl. Med., vol. 8, no. 15, pp. 966–966, Aug. 2020, doi: 10.21037/atm.2020.02.18.
36. J. P. Woo, D. B. McElhinney, and G. K. Lui, “The challenges of an aging tetralogy of Fallot population,” Expert Rev. Cardiovasc. Ther., vol. 19, no. 7, pp. 581–593, Jul. 2021, doi: 10.1080/14779072.2021.1940960.
37. E. Prifti, “Repair of complete atrioventricular septal defect with tetralogy of Fallot,” Transl. Pediatr., vol. 5, no. 1, pp. 1–7, Jan. 2017, doi: 10.21037/tp.2017.01.01.
38. S. Yang et al., “Impact of timing on in-patient outcomes of complete repair of tetralogy of Fallot in infancy: an analysis of the United States National Inpatient 2005–2011 database,” BMC Cardiovasc. Disord., vol. 19, no. 1, p. 46, Dec. 2019, doi: 10.1186/s12872-019-0999-1.
39. C. P. Callahan et al., “Early Outcomes for Management of Atrioventricular Septal Defect—Tetralogy of Fallot in the Last Decade: A Congenital Heart Surgeons’ Society Study,” World J. Pediatr. Congenit. Heart Surg., vol. 16, no. 2, pp. 262–272, Mar. 2025, doi: 10.1177/21501351241293158.