Outcomes and Causes of Death in Children on Home Mechanical Ventilation via Tracheostomy: An Institutional and Literature Review

Objective To describe outcomes and causes of death in children on chronic positive-pressure ventilation via tracheostomy. Study design We conducted a retrospective observational cohort analysis of 228 children enrolled in an university-affiliated home mechanical ventilation (HMV) program over 22 years (990 person-years). Cumulative incidences of survival and liberation from HMV are presented. Time-to-events were compared by reason for chronic respiratory failure (CRF) and age and date of HMV initiation with Kaplan-Meier and Cox regression analyses. Circumstances of death are described. Results Of our cohort, 47 of 228 children died, and 41 children were liberated from HMV. The 5-year cumulative incidences of survival and liberation were 80% and 24%, respectively. Being placed on HMV for chronic pulmonary disease was independently associated with liberation from HMV (hazard ratio, 7.38; 95% CI, 3.0-18.2; P < .001). Neither age nor reasons for CRF were associated with shortened survival. Progression of underlying condition accounted for only 34% of deaths; 49% of deaths were unexpected. Conclusion Most children on HMV survive or were weaned off. However, a sizable number of children in our cohort died, and many deaths were unexpected and from causes not directly related to their primary reason for CRF.