Abstract
Background Hypoplastic left heart syndrome with intact or restrictive atrial septum (HLHS-RAS) is an extremely high-risk subtype with near 50% mortality despite prenatal diagnosis and successful intervention. To enhance fetal counseling in this lesion beyond survival, we reviewed long-term neurodevelopmental outcomes in HLHS-RAS survivors.Methods Institutional IRB approval was obtained to perform a retrospective chart review on all patients with prenatally diagnosed HLHS-RAS who underwent emergent catheter-based intervention at UT Southwestern-Children’s Medical Center Dallas between 2009–2016.Results 6/14 patients with HLHS-RAS survived (43%). Demographics are presented in Table 1. Excluding the patients who received heart transplantation, available neurodevelopmental screening and qualitative remarks are presented in Table 2.Conclusions Survival in HLHS-RAS is poor despite prenatal diagnosis. Survivors may have average neurodevelopment in early childhood but are at increased risk for neurodevelopmental delays which may manifest at variable intervals, underscoring the importance of serial assessment to maximize potential in this high-risk patient population. Qualitative remarks implicate reasonable activity tolerance and socialization in survivors to later childhood.