title: Hemorrhage rates from brain arteriovenous malformation in patients with hereditary hemorrhagic telangiectasia.
abstract: OBJECTIVE
  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.
authors: 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.
authors: Helen
authors: H
authors: Kim
authors: 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.).
authors: Jeffrey
authors: J
authors: Nelson
authors: 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.).
authors: Timo
authors: T
authors: Krings
authors: 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.).
authors: Karel G
authors: KG
authors: terBrugge
authors: 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.).
authors: Charles E
authors: CE
authors: McCulloch
authors: 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.).
authors: Michael T
authors: MT
authors: Lawton
authors: 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.).
authors: William L
authors: WL
authors: Young
authors: 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.).
authors: Marie E
authors: ME
authors: Faughnan
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D017542:Aneurysm, Ruptured / Q000150:complications / Q000453:epidemiology; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D002538:Intracranial Arteriovenous Malformations / Q000150:complications* / Q000453:epidemiology*; D020300:Intracranial Hemorrhages / Q000453:epidemiology* / Q000209:etiology*; D008297:Male; D008875:Middle Aged; D012008:Recurrence; D012189:Retrospective Studies; D012959:Socioeconomic Factors; D016019:Survival Analysis; D013683:Telangiectasia, Hereditary Hemorrhagic / Q000150:complications* / Q000453:epidemiology* / Q000235:genetics; D055815:Young Adult
keywords: arteriovenous malformations; cerebral hemorrhage; natural history; telangiectasia, hereditary hemorrhagic