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Vienna phrenic pacemaker--experience with diaphragm pacing in children
1Department of Plastic and Reconstructive Surgery, Surgical University Clinic, Vienna, Austria.
Summary
Diaphragm pacing using the Vienna phrenic pacemaker effectively enabled electrophrenic respiration (EPR) in children with ventilatory insufficiency. This treatment improved quality of life, though not necessarily lifespan, for these young patients.
Area of Science:
- Pediatric Pulmonology
- Biomedical Engineering
- Neurology
Background:
- Central alveolar hypoventilation syndrome (CAH) and spinal cord/brain stem lesions (SCI) can cause severe ventilatory insufficiency in children.
- Traditional mechanical ventilation may have limitations for long-term management in pediatric patients.
- Phrenic nerve pacing offers a potential alternative for restoring diaphragmatic function.
Purpose of the Study:
- To evaluate the efficacy and outcomes of diaphragm pacing in pediatric patients with ventilatory insufficiency.
- To assess the feasibility of achieving continuous electrophrenic respiration (EPR) in children.
- To compare diaphragm pacing with positive pressure mechanical ventilation for quality of life improvement.
Main Methods:
- Eight children (5 boys, 3 girls; ages 2-13) with CAH or SCI underwent phrenic pacemaker implantation.
- Four electrodes were placed on each phrenic nerve via sternotomy.
- Synchronous pacing with increasing duty cycles was used to condition diaphragms for EPR.
Main Results:
- Successful electrophrenic respiration (EPR) was achieved in seven out of eight children.
- Four children achieved chronic EPR, with one undergoing conditioning.
- Six children were discharged, with survival ranging from 1 to 4 years post-discharge; one tracheotomy was closed.
- Two patients died within 2-3 years due to reasons unrelated to pacing, linked to parental neglect.
Conclusions:
- Diaphragm pacing is a viable treatment for pediatric ventilatory insufficiency due to CAH and SCI.
- Careful patient selection, considering medical and social factors, is crucial for successful implantation.
- While not extending lifespan in severely injured children, diaphragm pacing significantly enhances quality of life compared to mechanical ventilation.