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Long Term Outcomes Following Aortic Valve Replacement In Children With Conotruncal Anomalies
Bahaaldin Alsoufi<4, Jessica Knight1, Geetha Raghuveer2, James St Louis2, Lazaros Kochilas3, Bahaaldin Alsoufi4
1University of Georgia, Atlanta, GA, USA, 2Mercy Children's Hospital, Kansas City, MO, USA, 3Emory University, Atlanta, GA, USA, 4University of Louisville, Louisville, KY, USA.

Background: Aortic valve replacement (AVR) is occasionally needed in children with conotruncal anomalies (CTA) and aortic/AV disease.
Methods: 106 patients with repaired CTA underwent AVR (1982-2003): truncus (PTA,n=40), D-transposition (D-TGA,n=22), type-B interrupted arch (IAA,n=16), double-outlet RV (DORV,n=12), pulmonary atresia/ventriculoseptal defect (PAVSD,n=9), tetralogy (TOF,n=6), L-transposition (L-TGA,n=1). Competing-risks analysis examined outcomes following AVR (death/transplantation, reoperation) and multivariable regression analysis assessed factors influencing survival.
Results: Median age at AVR was 6.9-years (IQR2.5-12.4). 18(17%) had prior aortic valvuloplasty (surgical=12, percutaneous=6). Preoperatively, 83(78%) had regurgitation, 9(9%) stenosis, 14(13%) both. 18(17%) required Konno annular enlargement. AVR type was mechanical (n=72,68%), homograft (n=21,20%), Ross (n=13,12%). Operative mortality was 13(12%). Age <1-year was risk factor (OR=55,(6-539),p=0.0006). On competing-risks analysis, 10-years after AVR, 13% died or received transplantation, 33% had reoperation. 20-years survival was 58% (inferior to concurrent children with isolated congenital AV disease (82%,p<0.0001). Factors associated with death included concomitant mitral surgery (HR=11,(3-40),p=0.0002) and underlying defect IAA/PAVSD/L-TGA (HR=2.2,(1-5),p=0.0672).
Conclusions: Long-term survival following AVR in children with CTA is inferior to that of isolated congenital AV disease, and is linked to underlying cardiac anomaly. While valve type was not associated with survival, infant age was risk factor for early death. Continuous attrition and high reoperation need warrant vigilant monitoring.


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