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Myotonic dystrophy type 1 (DM1) frequently leads to chronic respiratory failure, yet the effectiveness of noninvasive home mechanical ventilation (HMV) remains understudied. Our objective was to assess the effects of HMV on gas exchange, health-related quality of life (HRQL), and daily functioning, and to explore baseline predictors of treatment response. A prospective multicenter study was conducted in which clinical data, pulmonary function tests, blood gas analysis, polysomnography and overnight pulse oximetry with transcutaneous CO₂ monitoring, and validated questionnaires were collected at baseline and after six months of treatment. Paired t-tests and correlation analyses assessed treatment outcomes and predictive factors. Forty participants (mean age 46.6 years, 63% male) were enrolled, and follow-up data of 38 participants were available for analysis. After six months of treatment, significant improvements in gas exchange were observed with a reduction in daytime pCO₂ of 0.48 ± 0.81 kPa (p = 0.001) and nocturnal mean pCO₂ of 0.92 ± 0.81 kPa (p < 0.001). HRQL, assessed with the Severe Respiratory Insufficiency (SRI) questionnaire, improved significantly, with a mean increase of 9.9 ± 8.9 points (p < 0.001).No significant changes were seen in daily functioning measured with the DM1-Activ^C questionnaire. Improvements in nocturnal pCO₂ correlated with HRQL gains (r = 0.427, p < 0.021), while baseline characteristics were not predictive of response. This prospective multicenter study demonstrates that nocturnal HMV is associated with significant improvements in gas exchange and HRQL in patients with DM1 over six months.