title: Posterior Circulation Ischemia or Occlusion in Five Adults With Failing Fontan Circulation.
abstract: BACKGROUND
  Palliative procedures performed before the Fontan procedure may require ligation of the subclavian arteries, thereby affecting flow to the vertebral arteries. In adults with functionally univentricular heart disease, the implications of altered brainstem vascular anatomy for perioperative management of failing Fontan circulation are not known.
  
  
  METHODS
  We identified abnormal posterior circulation anatomy in an adult patient with failing Fontan circulation who experienced a brainstem stroke after Fontan conversion. We then changed our clinical practice to include detailed preoperative neurologic evaluation of adults with univentricular heart disease and failing Fontan circulation. Here, we report the clinical and neuroimaging findings in 5 consecutive patients before and after this change in practice.
  
  
  RESULTS
  Five patients ages 28 to 42 years had Fontan procedures performed in childhood, and underwent either Fontan conversion or cardiac transplantation. Patient 1 experienced an episode of decreased cerebral perfusion pressure on postoperative day 3, and experienced an ischemic brainstem stroke causing transient locked-in syndrome. A change in practice was made, and patients 2, 3, and 4 were evaluated preoperatively by the neurocritical care service. These patients then had higher target blood pressures perioperatively and no neurologic injury. Patient 5 was evaluated for symptoms consistent with subclavian steal. Neuroimaging in 3 patients was abnormal, with atrophic vertebral arteries, an occluded vertebral artery, and retrograde perfusion of a vertebral artery.
  
  
  CONCLUSIONS
  In adults with failing Fontan circulation there is a potential for neurologic complications as a result of venous congestion with elevated central venous pressures, and aberrant posterior circulation. The patient's history and brain imaging may be used to identify at-risk patients and to tailor perioperative management during Fontan conversion or heart transplantation to mitigate the risk for brainstem ischemia.
authors.affiliation: Division of Neurology, Department of Pediatrics, Ann & Robert H. Lurie Children's Hospital of Chicago, Northwestern University Feinberg School of Medicine, Chicago, Illinois; Ruth D. & Ken M. Davee Pediatric Neurocritical Care Program, Ann & Robert H. Lurie Children's Hospital of Chicago, Northwestern University Feinberg School of Medicine, Chicago, Illinois.
authors.forename: Eileen
authors.initials: E
authors.lastname: Broomall
authors.affiliation: Division of Cardiology, Department of Pediatrics, Ann & Robert H. Lurie Children's Hospital of Chicago, Northwestern University Feinberg School of Medicine, Chicago, Illinois.
authors.forename: Mary E
authors.initials: ME
authors.lastname: McBride
authors.affiliation: Division of Cardiology, Department of Pediatrics, Ann & Robert H. Lurie Children's Hospital of Chicago, Northwestern University Feinberg School of Medicine, Chicago, Illinois.
authors.forename: Barbara J
authors.initials: BJ
authors.lastname: Deal
authors.affiliation: Division of Neurology, Department of Pediatrics, Ann & Robert H. Lurie Children's Hospital of Chicago, Northwestern University Feinberg School of Medicine, Chicago, Illinois; Ruth D. & Ken M. Davee Pediatric Neurocritical Care Program, Ann & Robert H. Lurie Children's Hospital of Chicago, Northwestern University Feinberg School of Medicine, Chicago, Illinois.
authors.forename: Laurence
authors.initials: L
authors.lastname: Ducharme-Crevier
authors.affiliation: Division of Neurology, Department of Pediatrics, Ann & Robert H. Lurie Children's Hospital of Chicago, Northwestern University Feinberg School of Medicine, Chicago, Illinois.
authors.forename: Alexandra
authors.initials: A
authors.lastname: Shaw
authors.affiliation: Division of Cardiology, Department of Pediatrics, Ann & Robert H. Lurie Children's Hospital of Chicago, Northwestern University Feinberg School of Medicine, Chicago, Illinois.
authors.forename: Mjaye
authors.initials: M
authors.lastname: Mazwi
authors.affiliation: Department of Cardiovascular Thoracic Surgery, Ann & Robert H. Lurie Children's Hospital of Chicago, Northwestern University Feinberg School of Medicine, Chicago, Illinois.
authors.forename: Carl L
authors.initials: CL
authors.lastname: Backer
authors.affiliation: Department of Cardiovascular Thoracic Surgery, Ann & Robert H. Lurie Children's Hospital of Chicago, Northwestern University Feinberg School of Medicine, Chicago, Illinois.
authors.forename: Michael C
authors.initials: MC
authors.lastname: Mongé
authors.affiliation: Division of Cardiology, Department of Pediatrics, Ann & Robert H. Lurie Children's Hospital of Chicago, Northwestern University Feinberg School of Medicine, Chicago, Illinois.
authors.forename: John
authors.initials: J
authors.lastname: Costello
authors.affiliation: Division of Cardiology, Department of Pediatrics, Ann & Robert H. Lurie Children's Hospital of Chicago, Northwestern University Feinberg School of Medicine, Chicago, Illinois.
authors.forename: Bradley S
authors.initials: BS
authors.lastname: Marino
authors.affiliation: Division of Cardiology, Department of Pediatrics, Ann & Robert H. Lurie Children's Hospital of Chicago, Northwestern University Feinberg School of Medicine, Chicago, Illinois.
authors.forename: Andrew
authors.initials: A
authors.lastname: DeFreitas
authors.affiliation: Division of Neurology, Department of Pediatrics, Ann & Robert H. Lurie Children's Hospital of Chicago, Northwestern University Feinberg School of Medicine, Chicago, Illinois; Ruth D. & Ken M. Davee Pediatric Neurocritical Care Program, Ann & Robert H. Lurie Children's Hospital of Chicago, Northwestern University Feinberg School of Medicine, Chicago, Illinois. Electronic address: m-wainwright@northwestern.edu.
authors.forename: Mark S
authors.initials: MS
authors.lastname: Wainwright
mesh_terms: D000328:Adult; D002545:Brain Ischemia / Q000000981:diagnostic imaging / Q000209:etiology* / Q000503:physiopathology / Q000517:prevention & control; D001933:Brain Stem / Q000098:blood supply / Q000000981:diagnostic imaging; D002496:Central Venous Pressure; D000072226:Computed Tomography Angiography; D017809:Fatal Outcome; D005260:Female; D018729:Fontan Procedure; D006330:Heart Defects, Congenital / Q000601:surgery; D006530:Hepatorenal Syndrome / Q000209:etiology; D006801:Humans; D006973:Hypertension / Q000209:etiology; D008026:Ligation / Q000009:adverse effects; D008297:Male; D009460:Neurologic Examination; D010166:Palliative Care; D011183:Postoperative Complications / Q000000981:diagnostic imaging / Q000209:etiology* / Q000503:physiopathology; D011300:Preoperative Care / Q000379:methods*; D011782:Quadriplegia / Q000209:etiology / Q000503:physiopathology; D012189:Retrospective Studies; D013348:Subclavian Artery / Q000601:surgery*; D013349:Subclavian Steal Syndrome / Q000209:etiology; D014711:Vertebral Artery / Q000000981:diagnostic imaging / Q000503:physiopathology; D014715:Vertebrobasilar Insufficiency / Q000000981:diagnostic imaging / Q000209:etiology* / Q000503:physiopathology