Anesthetic Management of the Patient with Mitochondrial Disease: A Review of Current Best Evidence

Headnote

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.

Keywords: Anesthesiology, mitochondrial disease, mitochondrial myopathy, perioperative management.

Objectives

Upon completion of this course, the learner will be able to:

1. Identify common symptomatology of mitochondrial disease.

2. Discuss the preanesthetic evaluation of the patient with mitochondrial disease.

3. Design an intraoperative management plan for the patient with mitochondrial disease.

4. Review the process of mitochondrial respiration.

5. Evaluate the impact of individual anesthetic agents on mitochondrial function.

Introduction

Mitochondrial diseases (MD) are a heterogenous group of genetic disorders causing dysfunction of mitochondrial respiration at the electron transport chain (ETC) and oxidative phosphorylation, impairing essential adenosine triphosphate (ATP) production. MD encompasses mitochondrial myopathy, mitochondrial cytopathy, mitochondrial encephalopathy, and various other named syndromes.1 It is conservatively estimated to affect 1 in 4,000 to 1 in 5,000 persons of all ages. 2-5 Organ systems with high metabolic requirements are most often affected.6 Symptoms primarily impact the central nervous system (CNS), cardiac, ocular, musculoskeletal, and gastrointestinal systems.7 Symptoms may develop at any age but presentations occurring in childhood typically reflect more severe pathology than those with adult...