is a practicing CRNA in the Washington, D.C. metro area. This Journal course was written during the author's doctoral candidacy at Virginia Commonwealth University, Richmond, Virginia. Email:
AANA J. 2022 Apr;90(2):148-154.
Anesthetic management of the patient with mitochondrial disease (MD) requires thoughtful preoperative planning and hypervigilant perioperative monitoring. MD affects 1 in 4,000 persons and is often an unfamiliar topic to the anesthesia provider. This review aims to inform the anesthetist on important considerations in perioperative management of MD. Patients with MD have impaired mitochondrial energy formation pathways affecting function of cardiac, central nervous, and musculoskeletal systems. All general anesthetics interfere with these mitochondrial bioenergetic pathways. MD patients exhibit hypersensitivity to volatile anesthetics. Propofol interferes with mitochondrial function via multiple pathways thus its use should be limited. MD is not at increased risk for malignant hyperthermia and should not be managed with prolonged propofol infusion. Succinylcholine is contraindicated due to hyperkalemia and myotonic risks. Nondepolarizing agents should be used with caution given unpredictable effects. No single anesthetic plan has been found to be safer than another in patients with MD. Intravenous and volatile anesthetics should be titrated incrementally while monitoring anesthetic depth clinically or via processed electroencephalogram (EEG). All MD patients should be optimized by minimizing fasting times, careful fluid selection to avoid lactate, and hypervigilant temperature management aimed at reducing the detrimental effects of catabolic stress during the perioperative period.
线粒体疾病 (MD) 患者的麻醉管理需要深思熟虑的术前规划和高度警惕的围手术期监测。MD 影响每 4000 人中的 1 人，并且通常是麻醉提供者不熟悉的主题。本综述旨在为麻醉师提供 MD 围手术期管理的重要注意事项。MD 患者的线粒体能量形成途径受损，影响心脏、中枢神经系统和肌肉骨骼系统的功能。所有全身麻醉药都会干扰这些线粒体生物能途径。MD 患者对挥发性麻醉剂表现出过敏反应。丙泊酚通过多种途径干扰线粒体功能，因此应限制其使用。MD 发生恶性高热的风险不增加，不应使用长时间的丙泊酚输注来进行管理。由于高钾血症和肌强直性风险，琥珀酰胆碱禁忌使用。应谨慎使用非去极化剂，因为其作用不可预测。在 MD 患者中，尚未发现某种麻醉方案比另一种更安全。应逐渐滴定静脉和挥发性麻醉剂，同时通过临床监测或经处理的脑电图 (EEG) 监测麻醉深度。所有 MD 患者都应通过最小化禁食时间、谨慎选择避免乳酸的液体以及高度警惕的体温管理来优化，以减少围手术期分解代谢应激的有害影响。
