Abstract WP218: Risk Factors for Cerebral Aneurysms in Sickle Cell Disease

Abstract

Introduction: Cerebral aneurysm frequency in sickle cell disease (SCD) is higher than in the general population. Specific risk factors explaining this difference have not been previously explored. We hypothesize that increased cerebral blood flow (CBF), low hemoglobin (Hb) levels, and documented risk factors for aneurysms in the general population would be associated with aneurysm development in SCD. Methods: Children and young adults with SCD defined as HbSS and HbSβ0 thalassemia were recruited from SCD clinics at an academic medical center and a community health clinic from 2014-2022 along with age and race-matched controls. Written consent and assent were required. Inclusion: ages 6-45 years, study MRI and MRA of the brain, risk factor data collected. MRAs were reviewed and aneurysms were confirmed by consensus of 2 neuroradiologists. CBF was assessed via MRI with pseudo-continuous arterial spin labeling. Results: The study population included 183 SCD participants, including 13 (7.1%) with aneurysms. The healthy control group included 77 participants, 3 (3.9%) of these participants had aneurysms, all women. In the SCD sample, 10 of 13 (76.9%) aneurysms occurred in males while 76 of 170 (44.7%) without aneurysms were male (p=0.025). In the aneurysm group, 3 of 13 (23.1%) were smokers versus 8 of 170 (4.7%) without aneurysms (p=0.035). In the SCD aneurysm group, median age was older, 30.9 years versus 17.2 years without aneurysm, (p<0.001). Systolic blood pressure and Hb levels were not different in SCD participants with and without aneurysms, p=0.074 and p=0.358, respectively. However, SBP was 119.7±14.9 with aneurysms vs 113.2±12.3mmHg without aneurysms. Mean gray matter CBF for the SCD group with aneurysms was higher at 86.7± 25.9ml/100g/min compared to 80.8 ± 20.3ml/100g/min in the SCD group without aneurysms, though not significantly (p=0.325). Discussion: Increased CBF and low hemoglobin levels were not risk factors for cerebral aneurysms in SCD in this relatively small cohort. Like the general population, age and smoking are risk factors for aneurysm development in SCD. In contrast to the general population, male sex rather than female sex in SCD is an aneurysm risk factor. Based on these data, a larger consortium to study aneurysms in SCD is needed.