Outcomes Following Aortic Valve Replacement in Children With Conotruncal Anomalies

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.