Diaphragm pacing: clinical and experimental results.

Over the last 26 years diaphragm pacing has been used in over 400 adults and 70 children to support ventilation and oxygenation. Diaphragm pacing can be useful for conditions in which the brain stem respiratory centers provide little or no stimulation to the respiratory muscles, i.e. central hypoventilation syndrome, Arnold-Chiari malformation/brain stem dysfunction, and high quadriplegia. Because the pacing systems are so portable, the greatest advantages accrue to those patients who require ventilatory support both while awake and asleep. Infants and children require tracheostomy to avoid upper airway obstruction and bilateral pacing to meet higher metabolic demands. The stimulus parameters most appropriate for pediatric patients have been characterized as low stimulus frequency, short inspiratory time, and moderate respiratory rate. Use of similar stimulus parameters in an immature animal model has resulted in preservation of diaphragmatic structure and function but transformation of the diaphragm from a mixed muscle to one with a uniform population of type 1, fatigue-resistant fibers (physiologic, histochemical, myosin isoform, and ultrastructural evidence). In 33 pediatric patients, representing 96 patient-years of use, there were 26 failures of the pacing systems requiring removal and/or replacement of the internal components. Mean time to failure was 56 months. Of our 36 patients who had diaphragm pacing systems implanted, 26 are alive and 22 are currently using the pacing systems. wo recent advances may further improve the long-term outcome of patients using diaphragm pacing. Smaller, better encapsulated receivers may improve system longevity and a new stimulus electrode may reduce the risk of diaphragmatic damage.