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

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)To read this article in full you will need to make a payment
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Article info
Publication history
Published online: March 15, 2022
Accepted:
November 8,
2021
Received in revised form:
September 29,
2021
Received:
May 5,
2021
Identification
Copyright
Crown Copyright © 2022 Published by Elsevier Inc. on behalf of The American Association for Thoracic Surgery
ScienceDirect
Access this article on ScienceDirectLinked Article
- Commentary: Surgical aortic valve repair as the primary option for children with congenital aortic stenosisThe Journal of Thoracic and Cardiovascular SurgeryVol. 164Issue 5
- PreviewWith 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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