Outcomes Following Aortic Valve Replacement in Children With Conotruncal Anomalies

Bahaaldin Alsoufi1, Jessica H Knight2, James St Louis3

  • 1University of Louisville, Louisville, KY, USA.

Insights

Children with conotruncal anomalies undergoing aortic valve replacement (AVR) have poorer long-term survival compared to those with other congenital heart defects. Infant age at AVR is a risk factor for operative mortality, necessitating vigilant follow-up.

Area of Science:

  • Pediatric Cardiology
  • Congenital Heart Surgery
  • Aortic Valve Disease

Background:

  • Conotruncal anomalies can lead to aortic valve disease, sometimes requiring aortic valve replacement (AVR).
  • Long-term outcomes for AVR in this specific pediatric population are not well-defined.

Purpose of the Study:

  • To report long-term results of aortic valve replacement (AVR) in children with repaired conotruncal anomalies.
  • To identify factors affecting survival after AVR in this cohort.

Main Methods:

  • Utilized the Pediatric Cardiac Care Consortium (PCCC) database to identify 106 children with repaired conotruncal anomalies who underwent AVR.
  • Employed competing risks analysis and multivariable regression to assess outcomes (death, transplantation, reoperation) and significant factors.
  • Long-term outcomes were tracked via PCCC, National Death Index, and Organ Procurement and Transplantation Network.

Main Results:

  • Operative mortality was 12%, with infant age at AVR being a significant risk factor.
  • At five years post-AVR, 6% experienced death or transplantation, and 20% required reoperation.
  • Twenty-year transplant-free survival was 53% in the conotruncal anomaly group, significantly lower than the 82% in a matched non-conotruncal congenital aortic valve disease group (P=.0012).

Conclusions:

  • Long-term survival after AVR in children with conotruncal anomalies is inferior to those with isolated congenital aortic valve disease.
  • Underlying cardiac defect and infant age at AVR are critical factors influencing outcomes.
  • Continuous monitoring and reoperation are crucial due to ongoing attrition and high reoperation rates.
Abstract

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