Broomall Eileen, McBride Mary E, Deal Barbara J, Ducharme-Crevier Laurence, Shaw Alexandra, Mazwi Mjaye, Backer Carl L, Mongé Michael C, Costello John, Marino Bradley S, DeFreitas Andrew, Wainwright Mark S
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.
Division of Cardiology, Department of Pediatrics, Ann & Robert H. Lurie Children's Hospital of Chicago, Northwestern University Feinberg School of Medicine, Chicago, Illinois.
Ann Thorac Surg. 2016 Jun;101(6):2315-20. doi: 10.1016/j.athoracsur.2016.01.001. Epub 2016 Mar 24.
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.
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.
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.
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.
在Fontan手术前进行的姑息性手术可能需要结扎锁骨下动脉，从而影响椎动脉的血流。在患有功能性单心室心脏病的成年人中，脑干血管解剖结构改变对Fontan循环衰竭围手术期管理的影响尚不清楚。
我们在一名Fontan循环衰竭的成年患者中发现了异常的后循环解剖结构，该患者在Fontan转换术后发生了脑干卒中。然后我们改变了临床实践，将对患有单心室心脏病且Fontan循环衰竭的成年人进行详细的术前神经学评估纳入其中。在此，我们报告了在实践改变前后连续5例患者的临床和神经影像学检查结果。
5例年龄在28至42岁之间的患者在儿童期接受了Fontan手术，并接受了Fontan转换或心脏移植。患者1在术后第3天出现脑灌注压降低，发生缺血性脑干卒中，导致短暂性闭锁综合征。我们改变了实践方式，患者2、3和4在术前接受了神经重症监护服务的评估。这些患者围手术期的目标血压更高，且没有神经损伤。患者5因出现与锁骨下动脉盗血相符的症状而接受评估。3例患者的神经影像学检查异常，表现为椎动脉萎缩、椎动脉闭塞和椎动脉逆行灌注。
在Fontan循环衰竭的成年人中，由于中心静脉压升高导致的静脉充血和异常的后循环，存在发生神经并发症的可能性。患者的病史和脑部影像学检查可用于识别高危患者，并在Fontan转换或心脏移植期间调整围手术期管理，以降低脑干缺血的风险。
