Abstract

Background: Pregnancy is associated with increased stroke risk in young women. Idiopathic moyamoya syndrome (IMMS) is a rare condition characterized by progressive narrowing of large cerebral arteries resulting in flimsy collaterals prone to rupture or thrombosis. Data are limited on pregnancy outcomes in women with IMMS. We hypothesized that IMMS would be associated with increased pregnancy morbidity, including stroke. Methods: Using the New York State Department of Health Statewide Planning and Research Cooperative System data from 2000-2014 we identified all women aged 18 and older with diagnoses of IMMS (ICD-9 437.5) who had hospitalizations for delivery at any time either prior, concomitant or subsequent to IMMS diagnosis. We considered a pregnancy to be IMMS-exposed if it occurred ≤ 3 years prior to, or any time after, IMMS diagnosis. We defined pregnancy morbidity as admission within 1 year of delivery for any of the Center for Disease Control and Prevention’s severe maternal morbidity indicators, including stroke. We compared the morbidity of IMMS-exposed pregnancies to unexposed pregnancies occurring in the same cohort >3 years prior to IMMS diagnosis. We used conditional logistic regression to calculate odds ratio (OR) and 95% confidence intervals (95%CI), adjusting for women with both exposed and unexposed pregnancies. Results: We identified 134 patients with IMMS, with 117 exposed pregnancies and 147 unexposed pregnancies. Severe maternal morbidity was higher for exposed pregnancies (33.3% vs 19.0%, OR: 2.1, 95%CI: 1.2-3.7). After adjusting for maternal morbidity, exposed pregnancies had more caesarean sections (44.4% vs 23.0%, OR: 4.2, 95%CI: 2.3-7.6). Complications occurring during and after exposed pregnancies included stroke, acute myocardial infarction, cardiac arrest, cardiac arrhythmia requiring conversion, eclampsia, pulmonary edema, and adult respiratory distress syndrome. Conclusion: Pregnancies within 3 years prior or any time after IMMS diagnosis had increased maternal morbidity within 1 year of delivery. Prospective studies are needed to better characterize increased maternal risks for women with moyamoya syndrome and develop preventive strategies.

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