Risk of Future Hemorrhage From Unruptured Brain Arteriovenous Malformations: The Multicenter Arteriovenous Malformation Research Study (MARS)

Authors

Helen Kim, Center for Cerebrovascular Research, Department of Anesthesia and Perioperative Care, University of California, San Francisco.
Jeffrey Nelson, Center for Cerebrovascular Research, Department of Anesthesia and Perioperative Care, University of California, San Francisco.
Charles E. McCulloch, Department of Epidemiology and Biostatistics, University of California, San Francisco.
Christopher Hess, Department of Radiology and Biomedical Imaging, University of California, San Francisco.
Steven W. Hetts, Department of Radiology and Biomedical Imaging, University of California, San Francisco.
Kelly Flemming, Department of Neurology, Mayo Clinic, Rochester, Minnesota.
Giuseppe S. Lanzino, Department of Neurosurgery, Mayo Clinic, Rochester, Minnesota.
Päivi Koroknay-Pál, Department of Neurosurgery, Helsinki University Hospital, Helsinki, Finland.
Elias Oulasvirta, Department of Neurosurgery, Helsinki University Hospital, Helsinki, Finland.
Aki Laakso, Department of Neurosurgery, Helsinki University Hospital, Helsinki, Finland.
Michael T. Lawton, Department of Neurosurgery, Barrow Neurological Institute, Phoenix, Arizona.
Jay P. Mohr, Department of Neurology, Columbia University, New York, New York.
Michael K. Morgan, Macquarie University, Sydney, New South Wales, Australia.
Nicole Moayeri, Santa Barbara Cottage Hospital, Santa Barbara, California.
Jonathan G. Zaroff, Kaiser Permanente San Francisco Medical Center, San Francisco, California.
Marco A. Stefani, Department of Neurology and Neurosurgery, Hospital de Clinicas de Porto Alegre, Porto Alegre, Brazil.
Xiaolin Chen, Beijing Tiantan Hospital, Capital Medical University, Beijing, China.
Yuanli Zhao, Beijing Tiantan Hospital, Capital Medical University, Beijing, China.
Rustam Al-Shahi Salman, Institute for Neuroscience and Cardiovascular Research, The University of Edinburgh, Edinburgh, Great Britain.

Document Type

Article

Abstract

IMPORTANCE: Annual rates of first intracranial hemorrhage (ICH) from unruptured brain arteriovenous malformations (AVMs) are often quoted as 2% to 4% in clinical practice. Precise estimates and risk factors are unavailable to inform treatment decisions. OBJECTIVE: To provide estimates of rates and risk factors for first ICH in a large cohort study of unruptured brain AVMs. DESIGN, SETTING, AND PARTICIPANTS: The Multicenter Arteriovenous Malformation Research Study (MARS) included data from 9 cohorts, each contributing 100 or more unruptured brain AVMs. The study was conducted from 2017 to 2023 with retrospective and prospective data collection for existing cohorts and/or new recruitment. This was an international study (2 population-based and 7 referral-based cohorts) that included participants diagnosed with an unruptured brain AVM. EXPOSURES: Demographic, clinical, and angiographic characteristics. MAIN OUTCOME AND MEASURE: The primary outcome was time to first ICH after diagnosis of unruptured brain AVM. Data were collected using standardized definitions; missing data were imputed. Cox regression analysis was performed, censoring at first brain AVM treatment, death, or last visit, and allowing baseline hazards to vary by cohort. RESULTS: A total of 3225 individuals were eligible for participation in this study. After 195 exclusions, 3030 participants (median [IQR] age, 38 [25-50] years; 1524 female [50.3%]) were included. Among 2989 participants with unruptured brain AVMs, 1333 (45%) presented with seizure. The median (IQR) maximal brain AVM diameter was 3.1 (2.2-4.4) cm, 248 of 2466 AVMs (10%) had exclusively deep venous drainage, 297 of 2690 (11%) were in supratentorial deep or cerebellar locations, and 457 of 2440 (19%) had associated arterial aneurysms. First ICH occurred in 159 participants over 11 339 person-years of follow-up for an ICH rate of 1.40 (95% CI, 1.20-1.64) per 100 person-years. Significant independent risk factors included (1) increasing age category at diagnosis (hazard ratio [HR], 0.87; 95% CI, 0.53-1.41 for those aged 20 to 39 years; HR, 1.23; 95% CI, 0.74-2.04 for those aged 40 to 59 years; and HR, 2.01; 95% CI, 1.14-3.57 for those aged 60 years vs younger than 20 years; P = .008), (2) presence of associated aneurysms (HR, 1.66; 95% CI, 1.06-2.59; P = .03), and (3) cerebellar or supratentorial deep location (HR, 1.87; 95% CI, 1.16-3.00; P = .01). CONCLUSIONS AND RELEVANCE: The annual ICH rate from unruptured brain AVM was lower than that commonly cited in clinical practice. Increasing age, associated arterial aneurysms, and cerebellar or supratentorial deep brain AVM location were associated with risk of first ICH. These results may be used to counsel patients about the natural history of unruptured brain AVMs.

Medical Subject Headings

Humans; Intracranial Arteriovenous Malformations (complications, epidemiology, diagnostic imaging); Female; Male; Adult; Middle Aged; Risk Factors; Intracranial Hemorrhages (epidemiology, etiology); Cohort Studies; Retrospective Studies; Young Adult; Aged

Publication Date

12-1-2025

Publication Title

JAMA neurology

E-ISSN

2168-6157

Volume

82

Issue

12

First Page

1274

Last Page

1281

PubMed ID

41051760

Digital Object Identifier (DOI)

10.1001/jamaneurol.2025.3581

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