Abstract
Cystic fibrosis (CF) is a genetic disorder caused by mutations in the CFTR gene, leading to multi-system impairment. Sleep respiratory disorders (SRDs) are frequent in individuals with CF-even in those with normal or mildly impaired lung function-and may adversely affect overall health. The triple combination of elexacaftor, tezacaftor, and ivacaftor (ETI) has markedly improved clinical outcomes in CF; however, its long-term impact on SRDs remains unclear. This study aimed to assess the effects of ETI on nocturnal cardiorespiratory parameters in individuals with CF over a two-year period. Thirty-five clinically stable patients aged ≥13 years, eligible for ETI therapy, were enrolled. Nocturnal cardiorespiratory polygraphy and spirometry were performed at baseline (T0), one year (T1), and two years (T2) after ETI initiation. After one year, significant improvements were observed in mean oxygen saturation (mSpO(2)), time with SpO(2) ≤ 90% (t ≤ 90%), and respiratory rate. Spirometric indices (FEV(1), FVC, FEF) also significantly increased (p < 0.05). Correlation analysis revealed positive associations between mSpO(2) and FEV(1) (ρ = 0.515, p = 0.002) and between FEV(1) and FVC (ρ = 0.894, p < 0.001), while t ≤ 90% negatively correlated with FEV(1) (ρ = -0.404, p = 0.016). No additional significant changes were found at T2. ETI therapy resulted in sustained improvements in nocturnal oxygenation and lung function, supporting the importance of nocturnal respiratory monitoring during follow-up.