Department of Neurological Surgery, Mayo Clinic, Rochester, Minnesota, USA.
Department of Neurological Surgery, Mayo Clinic, Rochester, Minnesota, USA; Department of Neurology, Mayo Clinic, Rochester, Minnesota, USA.
World Neurosurg. 2019 Oct;130:133-137. doi: 10.1016/j.wneu.2019.07.010. Epub 2019 Jul 8.
Eisenmenger syndrome is a rare sequela of uncorrected congenital heart disease complicated by pulmonary hypertension, from which reversal of the pathologic left-to-right cardiovascular shunt and cyanosis follow. Right-to-left shunting can lead to paradoxical cerebral emboli-increasing the risk of spontaneous or iatrogenic stroke and cerebral abscess.
A 38-year-old man presented with new focal seizures due to a brain abscess. Ventricular septal defect and pulmonary hypertension were identified. Despite dexamethasone and broad-spectrum antibiotics, he developed hemiparesis and altered mental status and required emergent stereotactic abscess drainage. Despite the anesthetic hazards of Eisenmenger syndrome, the procedure was successful and the patient recovered completely.
Noncardiac perioperative mortality in Eisenmenger syndrome is historically reported up to 19%, and risks are further increased with prolonged case duration or hypotension, mandating vigilant attention to volume status. Correspondingly, shorter- or lower-risk procedures such as stereotactic drainage are recommended. Procedures should be performed only at centers with expertise in management of Eisenmenger syndrome and cardiac-specialized anesthesiologists whenever possible. Although a conservative approach with early, aggressive medical management is preferred, operative intervention may be required in the setting of progressive deterioration and excellent postoperative outcomes are achievable.
艾森曼格综合征是一种罕见的先天性心脏病未经矫正的后遗症，伴有肺动脉高压，随后出现病理性左向右心血管分流和发绀逆转。右向左分流可导致反常性脑栓塞——增加自发性或医源性中风和脑脓肿的风险。
一名 38 岁男性因脑脓肿出现新发局灶性癫痫发作。发现室间隔缺损和肺动脉高压。尽管使用地塞米松和广谱抗生素，他仍出现偏瘫和精神状态改变，需要紧急立体定向脓肿引流。尽管存在艾森曼格综合征的麻醉风险，但该手术取得成功，患者完全康复。
艾森曼格综合征的非心脏围手术期死亡率历史上报告高达 19%，手术持续时间延长或低血压会进一步增加风险，因此需要密切关注容量状态。相应地，推荐采用立体定向引流等较短或风险较低的手术。只要有可能，应在具有艾森曼格综合征管理专业知识的中心并由心脏专科麻醉师进行手术。虽然早期积极的药物治疗是首选的保守方法，但在病情逐渐恶化的情况下可能需要手术干预，并且可以获得良好的术后效果。
