Intracranial aneurysms in sickle cell disease are associated with hemodynamic stress and anemia.
Yan WangJared Sebastian GarlandSlim FellahMartin ReisMatthew S ParsonsKristin P GuilliamsMelanie E FieldsAmy E MirroJosiah B LewisChunwei YingRachel CohenMonica L HulbertAllison Ann KingYasheng ChenJin-Moo LeeHongyu AnAndria L FordPublished in: Blood advances (2024)
While hemodynamic stress plays a key role in aneurysm formation outside of 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 MRI/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=0.07), lower hemoglobin (p=0.002), higher CBF (p=0.03), and higher OEF (p=0.02) 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, seven new aneurysms developed in six patients. The three-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.
Keyphrases
- coronary artery
- end stage renal disease
- abdominal aortic aneurysm
- chronic kidney disease
- ejection fraction
- newly diagnosed
- peritoneal dialysis
- prognostic factors
- internal carotid artery
- subarachnoid hemorrhage
- clinical trial
- computed tomography
- contrast enhanced
- multiple sclerosis
- white matter
- brain injury
- current status
- case control
- resting state
- phase iii
- placebo controlled