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

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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.

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Acknowledgments

Many thanks to the Knepper Foundation, whose generous gift help fund this effort. Some of Kathryn A. Carson’s work on this project was supported by Grant Number UL1 RR 025005 from the National Center for Research Resources, a component of the National Institutes of Health (NIH) and NIH Roadmap for Medical Research.

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Authors and Affiliations

  1. Division of Pediatric Cardiology, The Johns Hopkins Hospital, The Johns Hopkins Medical Institutions, The Charlotte R. Bloomberg Children’s Center, Room M2303, 1800 Orleans St., Baltimore, MD, 21287, USA

    William J. Ravekes, Jane E. Crosson, Philip J. Spevak, Richard Ringel & Kathryn W. Holmes

  2. Division of Adult Cardiology, The Johns Hopkins Medical Institutions, Baltimore, MD, 21287, USA

    Mary C. Corretti & Thomas A. Traill

  3. Division of Cardiac Surgery, The Johns Hopkins Medical Institutions, Baltimore, MD, 21287, USA

    Diane E. Alejo, Duke E. Cameron & Luca A. Vricella

  4. Institute of Genetic Medicine and Howard Hughes Medical Institute, The Johns Hopkins Medical Institutions, Baltimore, MD, 21287, USA

    Harry C. Dietz

  5. The Johns Hopkins School of Medicine, The Johns Hopkins Medical Institutions, Baltimore, MD, 21287, USA

    Sara Khalil

  6. Department of Epidemiology, Bloomberg School of Public Health, The Johns Hopkins Medical Institutions, Baltimore, MD, 21287, USA

    Kathryn A. Carson

  7. Division of Cardiology, Tufts Medical Center, 800 Washington Street, Boston, MA, 02111, USA

    Christian D. Nagy

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  1. Christian D. Nagy
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  2. Diane E. Alejo
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  3. Mary C. Corretti
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  8. Kathryn A. Carson
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  11. Duke E. Cameron
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  14. Kathryn W. Holmes
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Correspondence to Kathryn W. Holmes.

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Nagy, C.D., Alejo, D.E., Corretti, M.C. et al. Tetralogy of Fallot and Aortic Root Dilation: A Long-Term Outlook. Pediatr Cardiol 34, 809–816 (2013). https://doi.org/10.1007/s00246-012-0537-8

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  • DOI: https://doi.org/10.1007/s00246-012-0537-8

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