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Long-term outcomes of primary aortic valve repair for isolated congenital aortic stenosis in children

  • Fraser Wallace
    Affiliations
    Department of Cardiac Surgery, Royal Children's Hospital, Melbourne, Australia

    University of Melbourne, Melbourne, Australia
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  • Edward Buratto
    Affiliations
    Department of Cardiac Surgery, Royal Children's Hospital, Melbourne, Australia

    University of Melbourne, Melbourne, Australia

    Murdoch Children's Research Institute, Melbourne, Australia
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  • Antonia Schulz
    Affiliations
    Department of Cardiac Surgery, Royal Children's Hospital, Melbourne, Australia
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  • Yves d’Udekem
    Affiliations
    Department of Cardiac Surgery, Royal Children's Hospital, Melbourne, Australia

    University of Melbourne, Melbourne, Australia

    Murdoch Children's Research Institute, Melbourne, Australia

    Melbourne Centre of Cardiovascular Genomics and Regenerative Medicine; Melbourne, Australia
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  • Robert G. Weintraub
    Affiliations
    University of Melbourne, Melbourne, Australia

    Murdoch Children's Research Institute, Melbourne, Australia

    Melbourne Centre of Cardiovascular Genomics and Regenerative Medicine; Melbourne, Australia

    Department of Cardiology, Royal Children's Hospital, Melbourne, Australia
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  • Christian P. Brizard
    Affiliations
    Department of Cardiac Surgery, Royal Children's Hospital, Melbourne, Australia

    University of Melbourne, Melbourne, Australia

    Murdoch Children's Research Institute, Melbourne, Australia

    Melbourne Centre of Cardiovascular Genomics and Regenerative Medicine; Melbourne, Australia
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  • Igor E. Konstantinov
    Correspondence
    Address for reprints: Igor E. Konstantinov, MD, PhD, FRACS, Royal Children's Hospital, Flemington Rd, Parkville 3052, Australia.
    Affiliations
    Department of Cardiac Surgery, Royal Children's Hospital, Melbourne, Australia

    University of Melbourne, Melbourne, Australia

    Murdoch Children's Research Institute, Melbourne, Australia

    Melbourne Centre of Cardiovascular Genomics and Regenerative Medicine; Melbourne, Australia
    Search for articles by this author

      Abstract

      Objective

      We aimed to assess the long-term outcomes of children with isolated congenital aortic stenosis who underwent primary aortic valve repair.

      Methods

      Records of all children (n = 111) with isolated congenital aortic stenosis who underwent primary aortic valve repair between 1980 and 2016 were reviewed. An optimal operative outcome consisted of a residual left ventricular outflow tract peak systolic gradient <35 mm Hg and trivial or less aortic insufficiency.

      Results

      Median age at surgery was 0.4 years (interquartile range, 1 month-7.9 years) and median weight at surgery was 7.0 kg (interquartile range, 3.7-25.0 kg). Fifty-two patients (46.8%; 52/111) underwent aortic valve repair with the use of patch material. Early mortality was 0.9% (1/111). Late mortality was 0.9% (1/110). Freedom from aortic valve reoperation was 52.1% (95% CI, 38.7-63.8) at 10 years. Freedom from aortic valve replacement was 67.9% (95% CI, 55.4-77.5) at 10 years. An optimal outcome was achieved in 48 patients (43.2%; 48/111). At 10 years, freedom from aortic valve reoperation was 78.2% (95% CI, 63.1-87.8) in patients with an optimal outcome, compared with 39.4% (95% CI, 22.8-55.6) in those with a suboptimal outcome (P = .03). Tricuspid aortic valve was associated with a suboptimal outcome (P = .01).

      Conclusions

      Aortic valve repair achieves relief of congenital aortic stenosis with very low early mortality and excellent long-term survival, even in neonates. Although nearly half of the patients required aortic valve reoperation by 10 years, two-thirds of the patients remain free from aortic valve replacement. An optimal outcome was more commonly achieved with bicuspid aortic valves compared with tricuspid aortic valves.

      Graphical abstract

      Key Words

      Abbreviations and Acronyms:

      AI (aortic insufficiency), AoV (aortic valve), AS (aortic stenosis), CoA (coarctation of the aorta), HR (hazard ratio), IQR (interquartile range), LVOT (left ventricular outflow tract)
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      Linked Article

      • Commentary: Surgical aortic valve repair as the primary option for children with congenital aortic stenosis
        The Journal of Thoracic and Cardiovascular SurgeryVol. 164Issue 5
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          With this latest report,1 the group from Melbourne have added to their prodigious work on aortic valve repair in children. Following their earlier experience that demonstrated the superiority of surgical valvuloplasty,2 they have abandoned transcatheter intervention for this disease.3 While balloon dilatation remains first-line therapy at most centers,4 they have sailed against the prevailing wind and adopted an exclusively surgical approach, regardless of patient age or condition. Notably, no patient in the current series underwent balloon dilatation.
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