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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.
Objective:
Conotruncal anomalies can develop aortopathy and/or aortic valve (AV) disease and AV replacement (AVR) is occasionally needed. We report long-term results and examine factors affecting survival following AVR in this group.
Methods:
We queried the Pediatric Cardiac Care Consortium (PCCC, US database for interventions for congenital heart diseases) to identify patients with repaired conotruncal anomalies and AVR. Long-term outcomes were provided by the PCCC, the US National Death Index, and Organ Procurement and Transplantation Network. Competing risks analysis examined outcomes following AVR (death/transplantation, reoperation) and multivariable regression analysis assessed significant factors.
Results:
One hundred six children with repaired conotruncal anomalies underwent AVR (1982-2003). Underlying anomaly was truncus (n = 40), d-transposition (n = 22), type-B interrupted arch (n = 16), double-outlet right ventricle (n = 12), pulmonary atresia with ventricular septal defect (n = 9), tetralogy of Fallot (n = 6), corrected transposition (n = 1). 18 (17%) had prior aortic valvuloplasty (surgical = 12, percutaneous = 6). Median age at AVR was 6.9 years (interquartile range = 2.5-12.4). AV pathophysiology was regurgitation (n = 83, 78%), stenosis (n = 9, 9%), and mixed (n = 14, 15%). AVR type was mechanical (n = 72, 68%), homograft (n = 21, 20%), and Ross (n = 13, 12%). Operative mortality was 13(12%). Infant age at AVR was risk factor (odds ratio = 55, 95% confidence interval [CI] = 6-539, P = .0006). On competing risks analysis, five years after AVR, 6% died or received transplantation, 20% had reoperation. Twenty-five years transplant-free survival was 53%. Factors associated with death after hospital discharge included mitral surgery (hazards ratio [HR] = 11, 95% CI = 3-39, P = .0002), underlying defect (HR = 2, 95% CI = 1-5, P = .446). Twenty years transplant-free survival in conotruncal anomalies group was inferior to matched children undergoing AVR for congenital non-conotruncal disease (61% vs 82%, P = .0012).
Conclusions:
Long-term survival following AVR in children with conotruncal anomalies is inferior to that of isolated congenital AV disease and is linked to an underlying cardiac defect. Although valve type was not associated with survival, infant age was a risk factor for operative mortality. Continuous attrition and high reoperation warrant vigilant monitoring.
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