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Stroke. 2015 May;46(5):1362-4. doi: 10.1161/STROKEAHA.114.007367. Epub 2015 Apr 9.

Hemorrhage rates from brain arteriovenous malformation in patients with hereditary hemorrhagic telangiectasia.

Author information

1
From the Departments of Anesthesia and Perioperative Care (H.K., J.N., W.L.Y.), Epidemiology and Biostatistics (H.K., C.E.M.), and Neurological Surgery (M.T.L., W.L.Y.), University of California, San Francisco; Division of Neuroradiology, Department of Medical Imaging (T.K., K.G.T.), Toronto Western Hospital, University of Toronto, Toronto, Ontario, Canada; and Division of Respirology, Keenan Research Centre, and Li Ka Shing Knowledge Institute, St. Michael's Hospital, University of Toronto, Toronto, Ontario, Canada (M.E.F.). kimhel@anesthesia.ucsf.edu.
2
From the Departments of Anesthesia and Perioperative Care (H.K., J.N., W.L.Y.), Epidemiology and Biostatistics (H.K., C.E.M.), and Neurological Surgery (M.T.L., W.L.Y.), University of California, San Francisco; Division of Neuroradiology, Department of Medical Imaging (T.K., K.G.T.), Toronto Western Hospital, University of Toronto, Toronto, Ontario, Canada; and Division of Respirology, Keenan Research Centre, and Li Ka Shing Knowledge Institute, St. Michael's Hospital, University of Toronto, Toronto, Ontario, Canada (M.E.F.).

Abstract

BACKGROUND AND PURPOSE:

Hereditary hemorrhagic telangiectasia (HHT) is a systemic disease characterized by mucocutaneous telangiectasias, epistaxis, and arteriovenous malformations (AVMs). Intracranial hemorrhage (ICH) rates in this population are not well described. We report ICH rates and characteristics in HHT patients with brain AVMs (HHT-BAVMs).

METHODS:

We studied the first 153 HHT-BAVM patients with follow-up data enrolled in the Brain Vascular Malformation Consortium HHT Project. We estimated ICH rates after BAVM diagnosis.

RESULTS:

The majority of patients were women (58%) and white (98%). The mean age at BAVM diagnosis was 31±19 years (range, 0-70), with 61% of cases diagnosed on asymptomatic screening. Overall, 14% presented with ICH; among symptomatic cases, 37% presented ruptured. During 493 patient-years of follow-up, 5 ICH events occurred yielding a rate of 1.02% per year (95% confidence interval, 0.42-2.44%). ICH-free survival differed significantly by ICH presentation (P=0.003); ruptured cases had a higher ICH rate (10.07%; 95% confidence interval, 3.25-31.21%) than unruptured cases (0.43%; 95% confidence interval, 0.11-1.73%).

CONCLUSIONS:

Patients with HHT-BAVM who present with hemorrhage are at a higher risk for rehemorrhage compared with patients with BAVM detected presymptomatically.

KEYWORDS:

arteriovenous malformations; cerebral hemorrhage; natural history; telangiectasia, hereditary hemorrhagic

PMID:
25858236
PMCID:
PMC4415515
DOI:
10.1161/STROKEAHA.114.007367
[Indexed for MEDLINE]
Free PMC Article

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