Abstract WMP70: Neuroradiological Markers of Cerebral Small Vessel Disease in Adults With Congenital Heart Disease After Fontan Surgery

Abstract

Introduction: Fontan surgery performed during childhood enhances the lifespan and quality of life of patients with critical congenital heart disease. Post-Fontan circulation may impact function in multiple organ systems, but its long term effects on the brain microvasculature are not well characterized. We investigated brain MRI markers of cerebral small vessel disease (SVD) after exposure to Fontan circulation. Methods: We reviewed medical records and MRI images of 34 adults followed in an academic medical center after Fontan surgery, who had brain MRI for any reason from 08/01/1995-03/01/2022. We characterized Fontan physiological stage (a standardized measure of disease severity) at time of MRI, and identified SVD markers, including deep silent brain infarcts, small perivascular spaces in the basal ganglia, microhemorrhages, and white matter hyperintensities. We calculated SVD scores using a standardized scale of 0-4, where scores of ≥2 indicate moderate to severe SVD. We created ordinal logistic regression models to test the association between physiological stage and SVD score. Results: Characteristics of the patients are summarized in the Table, and distribution of SVD scores in the Figure. Adjusting for age at MRI, there was no association between physiological stage of disease and higher SVD score. Conclusion: In our sample of 34 young adult patients with long term exposure to Fontan circulation, the majority had evidence of moderate to severe SVD, which was not predicted by physiological stage of disease at time of MRI. Exposure to Fontan circulation may be associated with early onset of cerebral SVD, suggesting increased long term risk of stroke and dementia.