NICE did a rapid review of the published literature on the efficacy and safety of this procedure. This comprised a comprehensive literature search and detailed review of the evidence from 5 sources, which was discussed by the committee. The evidence included 4 case series and 1 analysis of the Avery Biomedical Devices database. It is presented in the summary of key evidence section in the overview. Other relevant literature is in the appendix of the overview.
The professional experts and the committee considered the key efficacy outcomes to be: quality of life, ventilator-free hours per day, tracheostomy decannulation, survival, respiratory infections, and hospital admissions.
The professional experts and the committee considered the key safety outcomes to be: device failure, revision surgery, phrenic nerve palsy, and infections.
One patient organisation submission was received and discussed by the committee. Patient commentary was sought but none was received.
The committee was pleased to hear from a patient organisation and its representative. It heard about the impact of congenital central hypoventilation syndrome on quality of life for people who are ventilator or mask dependent.
People who have this procedure should be followed up long term, with routine collection of safety and outcome data, technology failures and reoperation rates, possibly through a registry.
There are a small number of people who have had phrenic nerve pacing for over 40 years, but replacement electrodes or receivers might be needed over time.
The committee was informed that having a backup mode of ventilation is essential, particularly in previously ventilator-dependent people.
The committee was informed that the condition varies in severity, meaning that there may be people with undetected daytime hypoventilation whose condition is undiagnosed and who may need pacing in adulthood.
The committee was informed that phrenic nerve pacing may be used in other forms of central hypoventilation.
The committee was informed that pacing can be used 24 hours a day if needed.