Chest
Special FeatureConsensus StatementAmerican College of Chest Physicians Consensus Statement on the Respiratory and Related Management of Patients With Duchenne Muscular Dystrophy Undergoing Anesthesia or Sedation
Section snippets
Executive Summary
Duchenne muscular dystrophy (DMD) is a progressive neuromuscular disease transmitted by X-linked inheritance with an incidence of approximately 1 in 3,500 live male births. DMD affects the muscles of respiration and is associated with dilated cardiomyopathy, which often leads to death from cardiopulmonary causes. Patients with DMD are especially vulnerable to the adverse physiologic effects of general anesthesia and procedural sedation, prompting the need for expert recommendations on this
Background
DMD is a progressive neuromuscular disease transmitted by X-linked inheritance with an incidence of approximately 1 in 3,500 live male births. DMD affects the muscles of respiration and is associated with dilated cardiomyopathy, which often leads to death from cardiopulmonary causes. With current medical management, including the use of nocturnally assisted ventilation, mean survival now approximates 25 years.1 Patients with DMD are especially vulnerable to the adverse physiologic effects of
A. Pulmonary Assessment
DMD is characterized by weakness of the diaphragm, intercostal muscles, and the accessory muscles of respiration, resulting in restrictive pulmonary impairment and a progressive decrease in total lung capacity and vital capacity.1011 These abnormalities lead to hypoventilation and impaired cough, which predisposes to atelectasis and respiratory failure. Before patients with DMD receive general anesthesia or procedural sedation, they should undergo measurement of Spo2 in room air, and
A. Choice of Pharmacologic Agents for General Anesthesia
Patients with DMD are at increased risk for extreme hyperthermic events and rhabdomyolysis when they are exposed to certain anesthetics, especially inhaled agents such as halothane, isofluorane, and servoflurane. Such episodes can cause hyperkalemia and sudden death from cardiac arrest.234 These events mimic malignant hyperthermia, but DMD and malignant hyperthermia are genetically distinct diseases. Succinylcholine, a depolarizing muscle relaxant that can disrupt unstable cell membranes, has
A. Respiratory Support
Extubation directly to NPPV should be considered for DMD patients with baseline FVC < 50% of predicted, and should be strongly considered for those with FVC < 30% of predicted who have been endotracheally intubated for general anesthesia or procedural sedation. Extubation directly to NPPV should also be considered for any patient using NPPV preoperatively (see Section II, D, above).2744 Continuous use of NPPV can then be weaned as tolerated,18 except in patients who require NPPV 24 h/d at
IV. Areas in Need of Further Study
The area explored in this consensus statement is characterized by a lack of prospective, randomized studies. Examples of specific studies needed to develop evidence-based guidelines for the care of DMD patients undergoing general anesthesia or procedural sedation include the following: prospective studies that identify the baseline pulmonary function parameters that predict an increased risk of postoperative respiratory complications for DMD patients undergoing different types of procedures;
Acknowledgments
We thank the leadership and staff of the ACCP and the Muscular Dystrophy Association for their support of this project.
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Dr. Cwik is a full-time employee of the Muscular Dystrophy Association. Dr. Schroth is a recipient of grant monies from and membership on the advisory committee of Families of Spinal Muscular Atrophy. Dr. Sussman is a recipient of grant monies from the Shriner's Hospital Research Fund.