Posterior Circulation Ischemia or Occlusion in Five Adults with Failing Fontan Circulation

Eileen Broomall, Mary Eileen McBride, Barbara Deal, Laurence Ducharme-Crevier, Alexandra Shaw, Mjaye Leslie Mazwi, Carl L Backer, Michael C Monge, John M Costello, Bradley Scott Marino, Roger deFreitas, Mark Wainwright*

*Corresponding author for this work

Research output: Contribution to journalArticle

2 Citations (Scopus)

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.

Original languageEnglish (US)
Pages (from-to)2315-2320
Number of pages6
JournalAnnals of Thoracic Surgery
Volume101
Issue number6
DOIs
StatePublished - Jun 1 2016

Fingerprint

Ischemia
Vertebral Artery
Neuroimaging
Brain Stem Infarctions
Fontan Procedure
Heart Transplantation
Nervous System
Brain Stem
Heart Diseases
Anatomy
Cerebrovascular Circulation
Subclavian Steal Syndrome
Nervous System Trauma
Central Venous Pressure
Quadriplegia
Subclavian Artery
Hyperemia
Ligation
Blood Vessels
Perfusion

ASJC Scopus subject areas

  • Surgery
  • Pulmonary and Respiratory Medicine
  • Cardiology and Cardiovascular Medicine

Cite this

Broomall, Eileen ; McBride, Mary Eileen ; Deal, Barbara ; Ducharme-Crevier, Laurence ; Shaw, Alexandra ; Mazwi, Mjaye Leslie ; Backer, Carl L ; Monge, Michael C ; Costello, John M ; Marino, Bradley Scott ; deFreitas, Roger ; Wainwright, Mark. / Posterior Circulation Ischemia or Occlusion in Five Adults with Failing Fontan Circulation. In: Annals of Thoracic Surgery. 2016 ; Vol. 101, No. 6. pp. 2315-2320.
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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.",
author = "Eileen Broomall and McBride, {Mary Eileen} and Barbara Deal and Laurence Ducharme-Crevier and Alexandra Shaw and Mazwi, {Mjaye Leslie} and Backer, {Carl L} and Monge, {Michael C} and Costello, {John M} and Marino, {Bradley Scott} and Roger deFreitas and Mark Wainwright",
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Posterior Circulation Ischemia or Occlusion in Five Adults with Failing Fontan Circulation. / Broomall, Eileen; McBride, Mary Eileen; Deal, Barbara; Ducharme-Crevier, Laurence; Shaw, Alexandra; Mazwi, Mjaye Leslie; Backer, Carl L; Monge, Michael C; Costello, John M; Marino, Bradley Scott; deFreitas, Roger; Wainwright, Mark.

In: Annals of Thoracic Surgery, Vol. 101, No. 6, 01.06.2016, p. 2315-2320.

Research output: Contribution to journalArticle

TY - JOUR

T1 - Posterior Circulation Ischemia or Occlusion in Five Adults with Failing Fontan Circulation

AU - Broomall, Eileen

AU - McBride, Mary Eileen

AU - Deal, Barbara

AU - Ducharme-Crevier, Laurence

AU - Shaw, Alexandra

AU - Mazwi, Mjaye Leslie

AU - Backer, Carl L

AU - Monge, Michael C

AU - Costello, John M

AU - Marino, Bradley Scott

AU - deFreitas, Roger

AU - Wainwright, Mark

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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