Original Article

Pediatric Cardiology

, Volume 34, Issue 4, pp 809-816

Tetralogy of Fallot and Aortic Root Dilation: A Long-Term Outlook

  • Christian D. NagyAffiliated withDivision of Cardiology, Tufts Medical Center
  • , Diane E. AlejoAffiliated withDivision of Cardiac Surgery, The Johns Hopkins Medical Institutions
  • , Mary C. CorrettiAffiliated withDivision of Adult Cardiology, The Johns Hopkins Medical Institutions
  • , William J. RavekesAffiliated withDivision of Pediatric Cardiology, The Johns Hopkins Hospital, The Johns Hopkins Medical Institutions
  • , Jane E. CrossonAffiliated withDivision of Pediatric Cardiology, The Johns Hopkins Hospital, The Johns Hopkins Medical Institutions
  • , Philip J. SpevakAffiliated withDivision of Pediatric Cardiology, The Johns Hopkins Hospital, The Johns Hopkins Medical Institutions
  • , Richard RingelAffiliated withDivision of Pediatric Cardiology, The Johns Hopkins Hospital, The Johns Hopkins Medical Institutions
  • , Kathryn A. CarsonAffiliated withDepartment of Epidemiology, Bloomberg School of Public Health, The Johns Hopkins Medical Institutions
  • , Sara KhalilAffiliated withThe Johns Hopkins School of Medicine, The Johns Hopkins Medical Institutions
    • , Harry C. DietzAffiliated withInstitute of Genetic Medicine and Howard Hughes Medical Institute, The Johns Hopkins Medical Institutions
    • , Duke E. CameronAffiliated withDivision of Cardiac Surgery, The Johns Hopkins Medical Institutions
    • , Luca A. VricellaAffiliated withDivision of Cardiac Surgery, The Johns Hopkins Medical Institutions
    • , Thomas A. TraillAffiliated withDivision of Adult Cardiology, The Johns Hopkins Medical Institutions
    • , Kathryn W. HolmesAffiliated withDivision of Pediatric Cardiology, The Johns Hopkins Hospital, The Johns Hopkins Medical Institutions Email author 

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Abstract

Dilation of the sinus of Valsalva (SoV) has been increasingly observed after repaired tetralogy of Fallot (TOF). We estimate the prevalence of SoV dilation in adults with repaired TOF and analyze possible factors related to aortic disease. Adults with TOF [n = 109, median age 33.2 years (range 18.1 to 69.5)] evaluated at Johns Hopkins Hospital from 2001 to 2009 were reviewed in an observational retrospective cohort study. Median follow-up was 27.3 (range 0.1–48.8) years. SoV dilation was defined as >95 % confidence interval adjusted for age and body surface area (z-score > 2). The prevalence of SoV dilation was 51 % compared with that of a normal population with a mean z-score of 2.03. Maximal aortic diameters were ≥4 cm in 39 % (42 of 109), ≥4.5 cm in 21 % (23 of 109), ≥5 cm in 8 % (9 of 109), and ≥5.5 cm in 2 % (2 of 109). There was no aortic dissection or death due contributable to aortic disease. Aortic valve replacement was performed in 1.8 % and aortic root or ascending aorta (AA) replacement surgery in 2.8 % of patients. By multivariate logistic regression analysis, aortic regurgitation (AR) [odds ratio (OR) = 3.09, p = 0.005], residual ventricular septal defect (VSD) (OR = 4.14, p < 0.02), and TOF with pulmonary atresia (TOF/PA) (OR = 6.75, p = 0.03) were associated with increased odds of dilated aortic root. SoV dilation after TOF repair is common and persists with aging. AR, residual VSD, and TOF/PA are associated with increased odds of dilation. AA evaluation beyond the SoV is important. Indexed values are imperative to avoid bias on the basis of age and body surface area.

Keywords

Tetralogy of Fallot Congenital heart disease Aorta Aneurysm Dissection