Lifetime Outcomes Following Intervention for Bicuspid Aortic Valve Stenosis in Childhood.

Bicuspid aortic valve (BAV) is the most common congenital heart lesion that can be associated with significant valve dysfunction in childhood. Surgical interventions for this condition often include pulmonary autograft (Ross procedure) or aortic valve replacement (mechanical - mAVR - or bioprosthetic - bAVR). Aortic valve repair (Rpr) was a common primary intervention for BAV with aortic stenosis at our institution during the study period. This single-center cohort study included 150 adults with BAV and aortic stenosis who underwent 197 surgical interventions during childhood. We investigated important clinical demographic differences, long-term freedom from repeat aortic valve intervention, and long-term freedom from adverse events based upon the type of definitive aortic valve surgery. We hypothesized that Ross and mAVR would demonstrate superior temporal durability, but that mAVR would have more associated adverse events, and that Ross would require more lifetime sternotomies. Ross and mAVR demonstrated superior temporal durability when compared to other aortic valve interventions by pairwise comparison (Rpr vs mAVR p < 0.001; Rpr vs Ross p < 0.001; Rpr vs bAVR p = 0.075; mAVR vs Ross p = 0.924; mAVR vs bAVR p = 0.026; Ross vs bAVR p = 0.001). Ross demonstrated superior freedom from adverse events when compared directly to bAVR (Rpr vs mAVR p = 0.755; Rpr vs Ross p = 0.033; Rpr vs bAVR p = 0.816; mAVR vs Ross p = 0.040; mAVR vs bAVR p = 0.847; Ross vs bAVR p = 0.006). Ross procedure was not associated with an increased number of lifetime sternotomies compared to mAVR. Concurrent Konno procedure was not an independent predictor of worse outcomes.