Liberation and mortality outcomes in pediatric long-term ventilation: A qualitative systematic review

Objective: To provide a systematic review of liberation from positive pressure ventilation and mortality of children with chronic respiratory failure who used long-term invasive and noninvasive ventilation (LTV). Methods: Papers published from 1980 to 2018 were identified using Pubmed MEDLINE, Ovid MEDLINE, Embase, and Cochrane databases. Search results were limited to English-language papers with (a) patients less than 22 years at initiation, (b) patients who used invasive ventilation (IV) via tracheostomy or noninvasive ventilation (NIV), and (c) data on mortality or liberation from LTV. Data were presented using descriptive statistics; changes in outcomes over time were explored using linear regression. Follow-up variability, cohort heterogeneity, and insufficient data precluded combining data to estimate incidences or rates. Results: One hundred and thirty papers with 12 704 patients were included. The median number of patients was 37 (interquartile range [IQR] 17-74, range 6-3802). Twenty-five percent of patients were initiated on IV; 75% on NIV. The maximum follow-up ranged from 0.5 to 31.8 years (median 8.8 years). The median proportion of patients liberated in these papers was 3% (IQR 0%-21%). The median proportion of mortality was 18% (IQR 8%-27%). Proportions of liberation and mortality did not significantly change over time. Progression of underlying disease (44%), respiratory illness (19%), and LTV accident (11%) were the most common causes of death. Conclusions: These papers collectively show most patients survive for many years using LTV; in many subgroups, death is a more common outcome than liberation. However, the limitations of these papers preclude robust prognostication.