From the Guidelines
Diaphragmatic pacing stimulators are primarily indicated for patients with congenital central hypoventilation syndrome (CCHS), as they provide optimal ventilation during wakefulness and allow for participation in age-appropriate activities. The indications for diaphragmatic pacing stimulators include patients with CCHS, as well as those with other conditions such as high cervical spinal cord injuries and select cases of amyotrophic lateral sclerosis (ALS) 1. These devices are recommended for patients who require ventilatory support but have intact phrenic nerves and diaphragm function.
Key Considerations
- The most established indication is for ventilator-dependent patients with CCHS, where pacing can provide freedom from mechanical ventilation during daytime use 1.
- Current outcomes data shows that diaphragmatic pacing can provide optimal ventilation in children with CCHS, allowing for participation in supervised age-appropriate activities in moderation while receiving assisted ventilation 1.
- The procedure involves surgical implantation of electrodes on the phrenic nerves or directly into the diaphragm, connected to an external stimulator, and requires careful patient selection to ensure intact phrenic nerve function and adequate diaphragm response to stimulation 1.
- Complications of diaphragmatic pacing include obstructive apnea during sleep, which can be overcome by adjusting settings on the pacers to lengthen inspiratory time and/or decrease the force of inspiration 1.
Patient Selection and Outcomes
- Patient selection is critical, requiring intact phrenic nerve function, adequate diaphragm response to stimulation, and stable respiratory mechanics 1.
- The benefit of diaphragm pacing is freedom from the mechanical ventilator during daytime use, and for those older patients who use the diaphragm pacers during sleep time only, the aim is to minimize the need for the mechanical ventilator and potentially remove the tracheostomy 1.
- The use of diaphragmatic pacing stimulators can significantly improve quality of life for patients with CCHS, allowing for greater mobility and participation in daily activities, and should be considered as a treatment option for these patients.
From the Research
Indications for Diaphragmatic Pacing
- Diaphragmatic pacing is indicated for patients with spinal cord injury (SCI) who are ventilator-dependent 2, 3, 4
- It is also indicated for patients with amyotrophic lateral sclerosis (ALS) who are experiencing respiratory failure 2, 5
- Additionally, diaphragmatic pacing can be used for patients with central hypoventilation syndrome 4
- Pediatric patients with respiratory failure may also be candidates for diaphragmatic pacing, although the indications and techniques may differ from those used in adults 6
Summary of Current Outcomes/Data
- Studies have shown that diaphragmatic pacing can be safely and effectively used to provide ventilation for patients with SCI and ALS 2, 3
- In patients with SCI, diaphragmatic pacing has been shown to allow for freedom from ventilator use in up to 96% of patients 2
- In patients with ALS, diaphragmatic pacing has been shown to delay the need for mechanical ventilation by up to 24 months 2
- However, other studies have raised concerns about the safety and efficacy of diaphragmatic pacing in patients with ALS and cervical spine injury, citing a higher risk of complications and adverse events 5
- Overall, the outcomes and data on diaphragmatic pacing suggest that it can be a useful treatment option for patients with respiratory failure, but careful patient selection and monitoring are necessary to minimize risks and optimize benefits 2, 3, 4