Myopathies are a heterogeneous group of diseases characterized by a primary structural or functional impairment of the skeletal muscle cell. The most common symptom is muscle weakness with possible respiratory and cardiovascular impact and also other systemic manifestations. Among the inherited myopathies, distinction is made between muscular dystrophies, congenital myopathies, metabolic and mitochondrial myopathies, and channelopathies. Multidisciplinary care in early pregnancy is essential to ensure a good outcome to a woman with myopathy and her newborn. Several specific aspects have to be considered: cardiac and pulmonary involvements, potential scoliosis and its respiratory impact, anesthetic agents’ hypersensibility, risk of rhabdomyolysis, and severe hyperkalemia, as well as malignant hyperthermia in some myopathies with all the precautions it implies. Early epidural analgesia is recommended during labor. If caesarean delivery is performed, neuraxial technique is preferred. When general anesthesia is unavoidable, succinylcholine is strictly forbidden in muscular diseases, and halogenated agents are better avoided.
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