AANA April 2022 Journal Course: Anesthetic Management of the Patient with Mitochondrial Disease: A Review of Current Best Evidence-Article

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Mitochondrial diseases are genetic disorders that affect the function of the mitochondria, resulting in impaired ATP production. These diseases can present with a wide range of symptoms, including neurological, cardiac, ocular, musculoskeletal, and gastrointestinal symptoms. While patients with mitochondrial disease generally tolerate anesthesia well, they are at increased risk for perioperative complications. Anesthesia providers should be knowledgeable about the implications of mitochondrial dysfunction and individualized anesthetic needs of patients with mitochondrial disease.<br /><br />The management of patients with mitochondrial disease during anesthesia requires careful preoperative evaluation and planning. An interdisciplinary approach involving specialists in genetics, neurology, and cardiology can help develop an individualized anesthetic plan. Patients should be informed about the risk of postoperative ventilation, especially if surgery involves the thorax or upper abdomen. Baseline neurologic, cardiac, and respiratory function should be assessed, and patients should be screened for baseline oxygen levels. Patients with cardiomyopathy may require immediate access to external pacing and defibrillation.<br /><br />During the intraoperative period, the goals of management include minimizing metabolic stress, maintaining hemodynamic stability, and optimizing respiratory function. Patients should receive appropriate fluid and glucose supplementation. Temperature control is essential, as both hypo- and hyperthermia are poorly tolerated in patients with mitochondrial disease. Anesthetic agents should be titrated slowly and carefully, with consideration given to their impact on mitochondrial function. Some agents, such as propofol, should be used with caution, while others, like volatile anesthetics, may be preferred. Careful monitoring of neuromuscular function is necessary, and succinylcholine should be avoided due to the risk of hyperkalemia and myotonic crisis.<br /><br />Postoperatively, close monitoring should continue until the patient returns to baseline. Pulmonary hygiene, early mobilization, and appropriate pain management should be implemented to promote recovery.<br /><br />In conclusion, the management of patients with mitochondrial disease during anesthesia requires careful planning, monitoring, and individualized care. Anesthesia providers should be familiar with the impact of mitochondrial dysfunction on anesthesia and take appropriate measures to mitigate the risks associated with these conditions.

Keywords

mitochondrial diseases

genetic disorders

mitochondria

ATP production

perioperative complications

anesthesia management

baseline neurologic function

baseline cardiac function

baseline respiratory function

mitochondrial dysfunction