Hemorrhagic reversible cerebral vasoconstriction syndrome: A retrospective observational study

Background and purpose: Reversible cerebral vasoconstriction syndrome (RCVS) is characterized by recurrent thunderclap headaches associated with segmental vasoconstriction of cerebral arteries, which may result in intracranial hemorrhage (ICH). There is a lack of contemporary data available regarding the ICH burden in RCVS cohort. Our aim of the study is to assess the ICH burden, associated risk factors, and discharge outcome of ICH in patients with RCVS. Methods: All patients diagnosed with RCVS in the 2016 Nationwide Readmission Database were identified using ICD-10 code after excluding patients with the concurrent diagnosis of primary angiitis. ICH was defined as both intraparenchymal (IPH), subarachnoid hemorrhage (SAH), and subdural hematoma (SDH). Categorical and continuous variables were assessed by the Rao-Scott Chi-square test and the Wilcoxon signed-rank sum test respectively. We used a multivariable survey-weighted logistic model to determine the association between ICH and RCVS patient-level characteristics. Findings: A total of 799 patients were identified with RCVS. Total hospitalization of ICH was 43.4% [(95% CI 36.4–50.4%); (n = 346)] including SAH 35.9% [(95% CI 29.7–42.1%); (n = 287)], IPH 13.1% [(95% CI 9.5–16.7%); (n = 105)] and SDH 3.6% [(95% CI 1.5–5.6%); (n = 28)]. Patients with hemorrhagic RCVS (H-RCVS) had a mean age (years ± SE) of 47.4 ± 1.1 vs. 45.5 ± 1.2 years in R-RCVS (p = 0.247); and were predominantly female (84.0% vs. 68.8%; p = 0.001); with longer inpatient stays (10.9 vs. 6.8 days; p = 0.016); and a higher inpatient cost ($44,300 vs. $21,350; p < 0.001). On multivariable analyses, higher odds of ICH were female sex 2.57 (95% CI 1.45–4.55; p = 0.001), middle age-group (45–64 years) 1.87 (CI: 1.11–3.15; p = 0.018) and older age group (> 64 years) 3.72 (CI: 1.15–12.03; p = 0.029). About 67.0% of all H-RCVS patients were discharged home, with no observed inpatient mortality. Interpretation: Intracerebral hemorrhage is the most common vascular complication in hospitalized RCVS patients, resulting in longer hospitalizations with more invasive procedures and higher healthcare expenditure. However, overall outcomes are excellent regardless of types of ICH, with no inpatient mortality observed in patients with hemorrhagic RCVS. Female sex and middle to older age-group are associated with higher odds of ICH.