Final analysis of the pilot trial of diaphragm pacing in amyotrophic lateral sclerosis with long-term follow-up: diaphragm pacing positively affects diaphragm respiration.

BACKGROUND: Respiratory insufficiency is the major cause of mortality in patients with amyotrophic lateral sclerosis or Lou Gehrig's disease. This is the final report of the diaphragm pacing (DP) pilot trial.

METHODS: Patients underwent laparoscopic diaphragm electrode implantations and subsequent conditioning of diaphragms. Serial respiratory function tests were performed in the initial year and followed until death.

RESULTS: Sixteen patients were implanted with no perioperative or unanticipated device-related adverse events. There were 452 implant-months of follow-up. DP allowed greater movement of the diaphragm under fluoroscopy, increased muscle thickness, and decreased the decline in forced vital capacity. Median survival from implant was 19.7 months with the cause of death respiratory in only 31%.

CONCLUSIONS: Long-term analysis of DP in amyotrophic lateral sclerosis showed no safety issues and can positively influence diaphragm physiology and survival. This formed the initial basis for subsequent US Food and Drug Administration approval.