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Congenital: Aortic Valve| Volume 164, ISSUE 1, P186-196.e2, July 2022

Outcomes of aortic valve repair in children resulting in bicuspid anatomy: Is there a need for tricuspidization?

Published:January 25, 2022DOI:https://doi.org/10.1016/j.jtcvs.2022.01.022

      Abstract

      Objective

      We aimed to assess outcomes after aortic valve repair leading to bicuspid valve anatomy in children.

      Methods

      This is a retrospective study of patients who underwent aortic valve repair with creation/preservation of bicuspid aortic valve anatomy or tricuspidization of bicuspid valves between 1980 and 2016.

      Results

      Overall, 127 patients underwent bicuspid repair. Median age was 0.73 years (interquartile range, 0.1-8.9), and median weight was 8.15 kg (interquartile range, 3.9-31.7). The cohort included 22.8% neonates (n = 29), 28.3% infants (n = 36), and 48.8% children (n = 62). Repair was performed without a patch in 54.3% (n = 69). Survival at 10 years was 94.8% (95% confidence interval, 87.5-97.85). Freedom from aortic valve reoperation at 5 and 10 years was 79.9% (95% confidence interval, 71.2-86.2) and 65.6% (95% confidence interval, 53.4-75.3), respectively. Re-repair was undertaken in 53.7% (22/41). Freedom from aortic valve replacement at 5 and 10 years was 90.3% (95% confidence interval, 83.1-94.5) and 75.8% (95% confidence interval, 63-84.7), respectively. Risk factors for reoperation were age less than 1 year, unicuspid valve, and the presence of Shone complex and concomitant aortic arch repair. There were 107 patients (107/127, 84.25%) with preoperative bicuspid aortic valve morphology that was preserved. They were compared with a separate cohort of 44 patients who underwent tricuspidization of bicuspid aortic valve during the same period. There was no difference in survival or freedom from aortic valve reoperation. However, freedom from aortic valve replacement was lower after tricuspidization with 49.7% (95% confidence interval, 26.3-69.3) versus 75.8% (95% confidence interval, 62.1-85.1) after 10 years (P = .0118).

      Conclusions

      Aortic valve repair leading to bicuspid valve anatomy in children has satisfactory long-term results. Reoperation remains common, but the need for early valve replacement can be effectively delayed.

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      Key Words

      Abbreviations and Acronyms:

      AoV (aortic valve), CI (confidence interval), EFE (endocardial fibroelastosis), HR (hazard ratio), IQR (interquartile range)
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      Linked Article

      • Commentary: Can we ever “cure” aortic valve disease in children?
        The Journal of Thoracic and Cardiovascular SurgeryVol. 164Issue 1
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          The group from Melbourne1 presents a retrospective study spanning 36 years on primary aortic valve (AoV) repair performed in children (23% neonates, 29% infants). The primary goal was to compare results in the cohort that resulted in a bicuspid valve configuration with those that resulted in a tricuspid configuration. They conclude that repair strategies resulting in a bicuspid valve yields satisfactory outcomes. The bicuspid group had excellent long-term survival, and a freedom from AoV reoperation at 15 years of 32%.
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