Establishing polysomnographic criteria for initiation of non-invasive ventilation in children with neuromuscular diseases

STUDY OBJECTIVES: This study aims to (1) assess agreement for polysomnography (PSG)-based criteria for non-invasive ventilation (NIV) initiation between the American College of Chest Physicians (CHEST) neuromuscular guidelines, American Academy of Sleep Medicine (AASM) definitions for nocturnal hypoventilation and pediatric obstructive sleep apnea (OSA), and authors' proposed rapid eye movement (REM)-related criteria and (2) compare clinical outcomes and NIV adherence within 12 months in children with neuromuscular disorders (NMD). METHODS: Multicenter retrospective study of children with NMD recommended NIV between 2010 and 2023. We classified participants in 3 groups based on described criteria. Kappa and Wilcoxon signed-rank were used to assess agreement and compare groups. RESULTS: Of 115 participants (88 male, median age 11.8 years [IQR 7-15]), 71% met at least one NIV initiation criterion across all groups. Twenty-five percent had nocturnal hypoventilation by CHEST guidelines, 16% by REM-related criteria and 9% AASM-based criteria. Nocturnal hypoxemia was diagnosed in 22% (CHEST) and 19% (REM-related). Overall agreement was moderate between REM-related and CHEST criteria (k = 0.71; CI95% 0.47-0.95; p < 0.001). Apnea-hypopnea index agreement was strong between CHEST and AASM-based criteria (k = 0.86, CI95% 0.74-0.98; p < 0.001). Nocturnal hypoxemia agreement was moderate between CHEST and REM-related criteria (k = 0.69, CI95% 0.49-0.89; p < 0.001). From the 96% (n = 111) of children that started NIV, 73% (n = 84) maintained its use for 6-12 months, with no differences in adherence or clinical outcomes across groups. CONCLUSIONS: Findings support new CHEST guidelines integrate disease and REM-specific criteria to enhance timely NIV initiation for early signs of nocturnal hypoventilation or hypoxemia in children with NMD. CURRENT KNOWLEDGE/STUDY RATIONALE: The American College of Chest Physicians (CHEST) guidelines recommend starting non-invasive ventilation (NIV) before advanced sleep-disordered breathing (SDB) develops. However, it remains unclear whether these criteria fully capture the progressive nature of SDB in children and youth with neuromuscular disorders (NMD), which often begins in rapid eye movement (REM) sleep and later affects all stages. STUDY IMPACT: retention or hypoxemia not captured by American Academy of Sleep Medicine (AASM) standards. These findings support continued exploration of inclusive and physiologically grounded criteria for NIV initiation in pediatric NMD populations and align with growing recognition that current standard thresholds may be overly restrictive, potentially excluding children who experience progressive SDB, particularly those with nocturnal hypoventilation or hypoxemia.