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Original research article
Aortic disease and interventions in adults with tetralogy of Fallot
  1. Alexander C Egbe1,
  2. William R Miranda1,
  3. Naser M Ammash1,
  4. Nandan S Anavekar1,
  5. Venkata R Missula1,
  6. Srikanth Kothapalli1,
  7. Arooj R Khan1,
  8. Sameh M Said2,
  9. Heidi M Connolly1
  1. 1 Department of Cardiovascular Medicine, Mayo Clinic, Rochester, Minnesota, USA
  2. 2 Department of Cardiovascular Surgery, Mayo Clinic, Rochester, Minnesota, USA
  1. Correspondence to Dr Alexander C Egbe, Department of Cardiovascular Medicine, Mayo Clinic, Rochester, MN 55905, USA; egbe.alexander{at}


Background Aortic dilation and aortic valve disease are known long-term complication of tetralogy of Fallot (TOF), but the risk of aortic dissection and the indications for prophylactic aortic surgery are unknown in this population. The purpose of this study was to: (1) determine the prevalence of significant aortic valve disease and/or significant aortic aneurysm (AVD-AA); (2) determine the incidence of progressive aortic dilation and aortic dissection in patients with TOF.

Methods Retrospective review of adults with repaired TOF, and no prior aortic valve/aorta surgery, who had ≥2 measurements of the thoracic aorta >12 months apart, 1990–2017. The aortic root and mid-ascending aorta were measured at the onset of QRS complex from leading edge to leading edge. Significant aortic valve disease was defined as the presence of ≥moderate aortic stenosis and/or ≥moderate aortic regurgitation. Significant aortic aneurysm was defined as aortic root or mid-ascending aorta dimension ≥50 mm. Progressive aortic dilation was defined as increase in aortic dimension ≥2 mm.

Results Of the 453 consecutive patients (37±13 years, men 216 (49%)) in the study, aortic aneurysm was present in 312 (69%) based on normative data; progressive aortic dilation occurred in 40 (9%), and there was no case of aortic dissection. Significant AVD-AA occurred in 52 (12%) patients; and 15 of them (29%) underwent aortic surgery without any surgical mortality.

Conclusions Although aortic aneurysm was common, progressive aortic dilation was uncommon and aortic dissection did not occur in our patients with TOF with significant aortic aneurysms who did not undergo aortic surgery. This has important clinical implication in deciding the frequency of imaging follow-up and timing of surgical intervention in this population.

  • tetralogy of Fallot

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  • Contributors Conception and design: ACE, WRM, HMC. Data collection and analysis: ACE, WRM. Manuscript draft and revisions: ACE, WRM, NMA, NSA, VRM,SK, ARK, SMS, HMC. Final approval: ACE, WRM, NMA, NSA, VRM,SK, ARK, SMS, HMC. Responsibility for overall content: ACE, WRM, HMC.

  • Funding ACE is supported by the National Heart, Lung, and Blood Institute (NHLBI; grant K23 HL141448-01).

  • Competing interests None declared.

  • Patient consent Not required.

  • Ethics approval Mayo Clinic Institutional Review Board.

  • Provenance and peer review Not commissioned; externally peer reviewed.