Impact of tracheostomy invasive ventilation on survival in Japanese patients with multiple system atrophy

Introduction: Tracheostomy invasive ventilation (TIV) is therapeutic intervention to prolong survival. However, few reports have addressed TIV in multiple system atrophy (MSA). This study sought to evaluate the impact of TIV on survival in MSA patients.

Methods: This retrospective cohort study examined medical records of probable or definite MSA for patients in Hyogo-Chuo National Hospital from January 2000 to September 2021 to investigate overall survival and cause of death in those with tracheostomy and TIV.

Results: The study enrolled 12 definite and 127 probable MSA patients. Mean age at onset was 61.3 ± 9.8 years, and median survival time was 9.0 years. Tracheostomy was performed in 53 patients, 21 of whom were ventilated. Mean time from onset to tracheostomy and TIV was 7.0 ± 3.0 and 8.4 ± 4.4 years, respectively. After propensity score matching, tracheostomy showed a significant prolongation of median survival compared with no tracheostomy (10.1 vs. 7.5 years, p = 0.001) and TIV significantly prolonged survival compared with tracheostomy alone (17.8 vs. 9.2 years, p = 0.023). On Cox regression analysis, the hazard ratio for tracheostomy was 0.35 (95% confidence interval [CI] 0.17-0.68, p = 0.002) and TIV was 0.22 (95% CI 0.07-0.89, p = 0.032). In MSA with TIV, sudden death was significantly lower compared with tracheostomy alone, and infection was the most common cause of death.

Conclusion: Results showed that TIV prolonged survival and reduced sudden death compared with tracheostomy alone in MSA, although sudden death can never be completely prevented.