Aortopathy in tetralogy of Fallot—a collective review

  • Krithika RamaprabhuEmail author
  • Mohammed Idhrees
  • Bashi Velayudhan
Review Article


The pathophysiology of aortic diseases in Tetralogy of Fallot has been analyzed in various studies, focusing on the histopathology of the aortic root, altered hemodynamics, and effect of early corrective repair on the incidence of aortic root dilatation. In this review article, we sought to integrate these studies for a better understanding of the natural history and practical implications of aortic root dilatation in Tetralogy of Fallot. This better equips us to appropriately implement potentially effective interventions.


Adult congenital heart disease Aorta/aortic Congenital heart surgery 


Compliance with ethical standards

Conflict of interest

The authors declare that they have no conflict of interest.

Ethical statements, human and animal rights consent

Not applicable being a review article.

Informed consent

Not applicable being a review article.


  1. 1.
    Warnes CA. The adult with congenital heart disease: born to be bad? J Am Coll Cardiol. 2005;46:1–8.CrossRefGoogle Scholar
  2. 2.
    Murphy JG, Gersh BJ, Mair DD, et al. Long-term outcome in patients undergoing surgical repair of tetralogy of Fallot. N Engl J Med. 1993;329:593–9.Google Scholar
  3. 3.
    Sadiq A, Shyamkrishnan KG, Theodore S, Gopalakrishnan S, Tharakan JM, Karunakaran J. Long-term functional assessment after correction of tetralogy of Fallot in adulthood. Ann Thorac Surg. 2007;83:1790–5.CrossRefGoogle Scholar
  4. 4.
    Yetman AT, Graham T. The dilated aorta in patients with congenital cardiac defects. J Am Coll Cardiol. 2009;53:461–7.CrossRefGoogle Scholar
  5. 5.
    Capelli H, Ross D, Somerville J. Aortic regurgitation in tetrad of Fallot and pulmonary atresia. Am J Cardiol. 1982;49:1979–83.CrossRefGoogle Scholar
  6. 6.
    Dodds GA III, Warnes CA, Danielson GK. Aortic valve replacement after repair of pulmonary atresia and ventricular septal defect or tetralogy of Fallot. J Thorac Cardiovasc Surg. 1997;113:736–41.CrossRefGoogle Scholar
  7. 7.
    Niwa K, Siu SC, Webb GD, Gatzoulis MA. Progressive aortic root dilatation in adults late after repair of tetralogy of Fallot. Circulation. 2002;106:1374–8.CrossRefGoogle Scholar
  8. 8.
    Niwa K. Aortic root dilatation in tetralogy of Fallot long-term after repair - histology of the aorta in tetralogy of Fallot: evidence of intrinsic aortopathy. Int J Cardiol. 2005;103:117–9.CrossRefGoogle Scholar
  9. 9.
    Chong WY, Wong WH, Chiu CS, Cheung YF. Aortic root dilatation and aortic elastic properties in children after repair of tetralogy of Fallot. Am J Cardiol. 2006;97:905–9.CrossRefGoogle Scholar
  10. 10.
    Chowdhury UK, Mishra AK, Balakrishnan P, et al. Role of fibrillin-1 genetic mutations and polymorphism in aortic dilatation in patients undergoing intracardiac repair of tetralogy of Fallot. J Thorac Cardiovasc Surg. 2008;136:757–66.Google Scholar
  11. 11.
    Tan JL, Davlouros PA, McCarthy KP, Gatzoulis MA, Ho SY. Intrinsic histologic abnormalities of aortic root and ascending aorta in tetralogy of Fallot: evidence of causative mechanism for aortic dilatation and aorotpathy. Circulation. 2005;112:961–8.CrossRefGoogle Scholar
  12. 12.
    Chowdhury UK, Mishra AK, Ray R, Kalaivani M, Reddy SM, Venugopal P. Histopathological changes in ascending aorta and the risk factors related to histopathological conditions and aortic dilatation in patients with tetralogy of Fallot. J Thorac Cardiovasc Surg. 2008;135:69–77.CrossRefGoogle Scholar
  13. 13.
    de Sa M, Moshkovitz Y, Butany J, David TE. Histologic abnormalities of the ascending aorta and pulmonary trunk in patients with bicuspid aortic value disease: clinical relevance to the Ross procedure. J Thorac Cardiovasc Surg. 1999;118:588–94.CrossRefGoogle Scholar
  14. 14.
    Schlatmann TJ, Becker AE. Histologic changes in the normal aging aorta: implications for dissecting aortic aneurysm. Am J Cardiol. 1977;39:13–20.CrossRefGoogle Scholar
  15. 15.
    Senzaki H, Iwamoto Y, Ishido H, et al. Arterial haemodynamics in patients after repair of tetralogy of Fallot: influence on left ventricular after load and aortic dilation. Heart. 2008;94:70–4.Google Scholar
  16. 16.
    Christensen JT, Lu JC, Donohue J, et al. Relation of aortic stiffness and strain by cardiovascular magnetic resonance imaging to age in repaired tetralogy of Fallot. Am J Cardiol. 2014;113:1031–5.Google Scholar
  17. 17.
    Seki M, Kurishima C, Saiki H, et al. Progressive aortic dilation and aortic stiffness in children with repaired tetralogy of Fallot. Heart Vessel. 2014;29:83–7.Google Scholar
  18. 18.
    Saiki H, Kojima T, Seki M, Masutani S, Senzaki H. Marked disparity in mechanical wall properties between ascending and descending aorta in patients with tetralogy of Fallot. Eur J Cardiothorac Surg. 2012;41:570–3.CrossRefGoogle Scholar
  19. 19.
    Francois K, Zaquot M, Bove T, et al. The fate of the aortic root after early repair of tetralogy of Fallot. Eur JCardiothorac Surg. 2010;37:1254–8.CrossRefGoogle Scholar
  20. 20.
    Bhat AH, Smith CJ, Hawker RE. Late aortic root dilatation in tetralogy of Fallot may be prevented by early repair in infancy. Pediatr Cardiol. 2004;25:654–9.CrossRefGoogle Scholar
  21. 21.
    Frischhertz BP, Shamszad P, Pedroza C, Milewicz DM, Morris SA. Thoracic aortic dissection and rupture in conotruncal cardiac defects: a population- based study. Int J Cardiol. 2015;184:521–7.CrossRefGoogle Scholar
  22. 22.
    Kim WH, Seo JW, Kim SJ, Song J, Lee J, Na CY. Aortic dissection late after repair of tetralogy of Fallot. Int J Cardiol. 2005;101:515–6.CrossRefGoogle Scholar
  23. 23.
    Rathi VK, Doyle M, Williams RB. Massive aortic aneurysm and dissection in repaired tetralogy of Fallot; diagnosis by cardiovascular magnetic resonance imaging. Int J Cardiol. 2005;101:169–70.CrossRefGoogle Scholar
  24. 24.
    Karamlou T, McCrindle BW, Williams WG. Surgery insight: late complications following repair of tetralogy of Fallot and related surgical strategies for management. Nat Clin Pract Cardiovasc Med. 2006;3:611–22.CrossRefGoogle Scholar
  25. 25.
    Dearani JA, Burkhart HM, Stulak JM, Sundt TM, Schaff HV. Management of the aortic root in adult patients with conotruncal anomalies. Semin Thorac Cardiovasc Surg Pediatr Card Surg Ann. 2009;12:122–9.CrossRefGoogle Scholar

Copyright information

© Indian Association of Cardiovascular-Thoracic Surgeons 2019

Authors and Affiliations

  • Krithika Ramaprabhu
    • 1
    Email author
  • Mohammed Idhrees
    • 2
  • Bashi Velayudhan
    • 2
  1. 1.Department of Cardiothoracic SurgeryMIOT HospitalsChennaiIndia
  2. 2.Department of Cardiothoracic SurgerySIMSChennaiIndia

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