TY - JOUR
T1 - Posterior Circulation Ischemia or Occlusion in Five Adults with Failing Fontan Circulation
AU - Broomall, Eileen
AU - McBride, Mary E.
AU - Deal, Barbara J.
AU - Ducharme-Crevier, Laurence
AU - Shaw, Alexandra
AU - Mazwi, Mjaye
AU - Backer, Carl L.
AU - Mongé, Michael C.
AU - Costello, John
AU - Marino, Bradley S.
AU - Defreitas, Andrew
AU - Wainwright, Mark S.
N1 - Publisher Copyright:
© 2016 The Society of Thoracic Surgeons.
Copyright:
Copyright 2017 Elsevier B.V., All rights reserved.
PY - 2016/6/1
Y1 - 2016/6/1
N2 - 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.
AB - 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.
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U2 - 10.1016/j.athoracsur.2016.01.001
DO - 10.1016/j.athoracsur.2016.01.001
M3 - Article
C2 - 27016841
AN - SCOPUS:84969849715
VL - 101
SP - 2315
EP - 2320
JO - Annals of Thoracic Surgery
JF - Annals of Thoracic Surgery
SN - 0003-4975
IS - 6
ER -