Introduction: Home tracheotomy mechanical ventilation (HTMV) can prolong survival in patients with amyotrophic lateral sclerosis (ALS) when noninvasive ventilation (NIV) and mechanical assisted coughing (MIE) fail. The aim of this study was to determine the causes of tracheotomy and the long-term survival in a cohort of patients with ALS.

Methods: A prospective study including ALS patients attendedin a referral respiratory care unit (RCU) during a period of 5 years. Patients?informed decisions about HTMV were fully respected. Caregivers were trained in patientŽs care.

Results: A total of 171 patients were managed at our RCU during the period of the study. In 40 (23,4%) of them a tracheotomy was performed mainly due to severe bulbar dysfunction. The main causes for the need of a tracheotomy were NIV failure to mantain an adequate ventilation in 21 (52,5%) patients, MIE failure to remove secretions in 13 (32,5%) and both ineffective NIV and MIE in 6 (15%) of them. No patient needed a tracheotomy at the time of ALS diagnosis. The 5-year mean survival was 53,302 +/- 10,528 (95% CI 32,668 to 73,936).

Conclusions: Both NIV inadequacy and ineffectiveness of MIE appears to be a relevant cause of tracheotomy for patients with ALS with severe bulbar dysfunction. Patients choosing HTMV provided by a referral RCU could have a long-term survival rate.