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A Multidisciplinary Approach to the Anesthetic Management of Patients With Duchenne Muscular Dystrophy
Fabrizio Racca1, Elena Conti1, Giulia Racca2
1Division of Anesthesia and Intensive Care, AO Ordine Mauriziano, Torino, Italy.
Abstract:
Duchenne muscular dystrophy (DMD) is an inherited neuromuscular disorder with multisystem involvement that causes progressive muscular weakness and cardiorespiratory dysfunction. The first comprehensive recommendations regarding anesthetic management are now almost 20 years old and, in the interval, DMD care has evolved. Patients with DMD are experiencing prolongation of survival and, with widespread use of assisted ventilation, cardiac function has become the main determinant of their survival. Newer procedures, such as advanced cardiac interventions, are now performed, in a population of older, medically fragile individuals. Regional anesthetic techniques have advanced, allowing more patients to avoid the risks of inhalational anesthesia, and newer techniques for reversing neuromuscular blockade and minimizing cardiorespiratory depression have become available. Additionally, new medications are being used that have anesthetic implications, including newer guideline-directed heart failure medications. Overall, it has become increasingly important for perioperative management to be addressed in a multidisciplinary way, with coordination between medical, surgical, and anesthetic clinicians. In this narrative review, we provide information on how DMD's multi-system involvement impacts perioperative assessment and management, and we advocate for a multidisciplinary approach to perioperative care.
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