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.
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Warnes CA. The adult with congenital heart disease: born to be bad? J Am Coll Cardiol. 2005;46:1–8.
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.
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.
Yetman AT, Graham T. The dilated aorta in patients with congenital cardiac defects. J Am Coll Cardiol. 2009;53:461–7.
Capelli H, Ross D, Somerville J. Aortic regurgitation in tetrad of Fallot and pulmonary atresia. Am J Cardiol. 1982;49:1979–83.
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.
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.
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.
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.
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.
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.
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.
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.
Schlatmann TJ, Becker AE. Histologic changes in the normal aging aorta: implications for dissecting aortic aneurysm. Am J Cardiol. 1977;39:13–20.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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Ramaprabhu, K., Idhrees, M. & Velayudhan, B. Aortopathy in tetralogy of Fallot—a collective review. Indian J Thorac Cardiovasc Surg 35, 575–578 (2019). https://doi.org/10.1007/s12055-019-00791-5
- Adult congenital heart disease
- Congenital heart surgery