Diaphragm pacing improves survival in a select group of amyotrophic lateral sclerosis

Introduction: Respiratory insufficiency in amyotrophic lateral sclerosis (ALS)patients is treated by NIMV. Objectives: In select ALS patients we aim to treat respiratory insufficiency via diaphragm pacing. Methods: Between April 2014 and December 2016, we prospectively collected data of 10 ALS patients (6 females, median age 59)who underwent diaphragm pacing and 15 ALS-matched patients who did not. In pacing group, 1 patient had trachestomy, 4 were using BIPAP. In the non-pacing group 1 patient had tracheostomy, 2 were using BIPAP. Patients who were diagnosed to have chronic hypoventilation(FVC Results: Morbidity was 10%(n=1, pneumothorax) in pacing group.30-day mortality was 0% and 90-day mortality was 10%(n=1).In acute period, patients had better sleep cycles. The patient with tracheostomy was off the ventilator at the end of the 3rd month. During the follow-up two patients underwent tracheostomy, one due to excessive salivation and one due to respiratory insufficiency. None of the patients had pneumonia in the first 12 month period. Average follow-up of patients was 20.4 months (range, 3-32 months) after implantation. Diaphragmatic thickness calculated by diaphragm ultrasound showed an average of 23% increase in the thickness at the 3rd month post-implant. While the survival was 80% in the pacing group, survival was 0% in the non-pacing group. Conclusion: Diaphragm pacing improves survival in a select group of ALS patients with chronic hypoventilation.