TY - JOUR
T1 - Intracranial aneurysms in sickle cell disease are associated with hemodynamic stress and anemia
AU - Wang, Yan
AU - Garland, Jared S.
AU - Fellah, Slim
AU - Reis, Martin N.
AU - Parsons, Matthew S.
AU - Guilliams, Kristin P.
AU - Fields, Melanie E.
AU - Mirro, Amy E.
AU - Lewis, Josiah B.
AU - Ying, Chunwei
AU - Cohen, Rachel A.
AU - Hulbert, Monica L.
AU - King, Allison A.
AU - Chen, Yasheng
AU - Lee, Jin Moo
AU - An, Hongyu
AU - Ford, Andria L.
N1 - Publisher Copyright:
© 2024 by The American Society of Hematology.
PY - 2024/9/24
Y1 - 2024/9/24
N2 - Although hemodynamic stress plays a key role in aneurysm formation outside of sickle cell disease (SCD), its role is understudied in patients with SCD. We hypothesized that tissue-based markers of hemodynamic stress are associated with aneurysm presence in a prospective SCD cohort. Children and adults with SCD, with and without aneurysms, underwent longitudinal brain magnetic resonance imaging/magnetic resonance angiography (MRA) to assess cerebral blood flow (CBF) and oxygen extraction fraction (OEF). Baseline characteristics were recorded. In the subgroup of adults, stepwise mixed-effect logistic regression examined clinical variables, CBF, and OEF as predictors of aneurysm presence. Cumulative rates of new aneurysm formation were estimated using Kaplan-Meier analyses. Forty-three aneurysms were found in 27 of 155 patients (17%). Most aneurysms were ≤3 mm and in the intracranial internal carotid artery. On univariate analysis, older age (P = .07), lower hemoglobin (P = .002), higher CBF (P = .03), and higher OEF (P = .02) were associated with aneurysm presence. On multivariable analysis, age and CBF remained independently associated with aneurysm presence. Seventy-six patients (49% of enrollment) received follow-up MRAs (median, 3.5 years). No aneurysm grew or ruptured, however, 7 new aneurysms developed in 6 patients. The 3-year cumulative rate of aneurysm formation was 3.5%. In 155 patients with SCD, 17% had intracranial aneurysms. Three-year aneurysm formation rate was 3.5%, although limited by small longitudinal sample size and short follow-up duration. Aneurysm presence was associated with elevated CBF in adults, as a tissue-based marker of cerebral hemodynamic stress. Future studies may examine the predictive role of CBF in aneurysm development in SCD.
AB - Although hemodynamic stress plays a key role in aneurysm formation outside of sickle cell disease (SCD), its role is understudied in patients with SCD. We hypothesized that tissue-based markers of hemodynamic stress are associated with aneurysm presence in a prospective SCD cohort. Children and adults with SCD, with and without aneurysms, underwent longitudinal brain magnetic resonance imaging/magnetic resonance angiography (MRA) to assess cerebral blood flow (CBF) and oxygen extraction fraction (OEF). Baseline characteristics were recorded. In the subgroup of adults, stepwise mixed-effect logistic regression examined clinical variables, CBF, and OEF as predictors of aneurysm presence. Cumulative rates of new aneurysm formation were estimated using Kaplan-Meier analyses. Forty-three aneurysms were found in 27 of 155 patients (17%). Most aneurysms were ≤3 mm and in the intracranial internal carotid artery. On univariate analysis, older age (P = .07), lower hemoglobin (P = .002), higher CBF (P = .03), and higher OEF (P = .02) were associated with aneurysm presence. On multivariable analysis, age and CBF remained independently associated with aneurysm presence. Seventy-six patients (49% of enrollment) received follow-up MRAs (median, 3.5 years). No aneurysm grew or ruptured, however, 7 new aneurysms developed in 6 patients. The 3-year cumulative rate of aneurysm formation was 3.5%. In 155 patients with SCD, 17% had intracranial aneurysms. Three-year aneurysm formation rate was 3.5%, although limited by small longitudinal sample size and short follow-up duration. Aneurysm presence was associated with elevated CBF in adults, as a tissue-based marker of cerebral hemodynamic stress. Future studies may examine the predictive role of CBF in aneurysm development in SCD.
UR - http://www.scopus.com/inward/record.url?scp=85205140285&partnerID=8YFLogxK
U2 - 10.1182/bloodadvances.2024013928
DO - 10.1182/bloodadvances.2024013928
M3 - Article
C2 - 39093929
AN - SCOPUS:85205140285
SN - 2473-9529
VL - 8
SP - 4823
EP - 4831
JO - Blood Advances
JF - Blood Advances
IS - 18
ER -