Impact of Built-In Software Monitoring on Survival in Amyotrophic Lateral Sclerosis Patients Receiving Home Mechanical Ventilation: A Cohort Study
Abstract
1. Introduction
2. Materials and Methods
2.1. Study Design and Patient Selection
2.2. Ventilator Adjustments and Clinical Management
2.3. Statistical Analysis
2.4. Ethical Approval
3. Results
3.1. Functional and Ventilatory Progression
3.2. Overall Survival and Prognostic Factors
3.3. Association Between BVS Monitoring and Survival

3.4. Prognostic Value of BVS-Derived Ventilatory Parameters

4. Discussion
5. Conclusions
Supplementary Materials
Author Contributions
Funding
Institutional Review Board Statement
Informed Consent Statement
Data Availability Statement
Acknowledgments
Conflicts of Interest
Abbreviations
| ALS | Amyotrophic lateral sclerosis |
| BVS | Built-in ventilator software |
| MV | Mechanical ventilation |
References
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| Variable | BVS Group (n = 57) | Non-BVS Group (n = 63) | p | |
|---|---|---|---|---|
| Sex | Male (n = 85) Female (n = 35) | 38 (66.7%) 17 (33.3%) | 47 (74.6%) 16 (25.4%) | 0.34 |
| Site of onset | Bulbar (n = 35) Spinal (n = 85) | 15 (26.3%) 42 (73.3%) | 20 (31.7%) 43 (68.3%) | 0.51 |
| Age at diagnosis (years) | Mean (SD) | 60.1 (11.3) | 64.9 (11.1) | 0.15 |
| Mechanical ventilation | Noninvasive (n = 117) Invasive (n = 3) | 54 (94.7%) 3 (100%) | 63 (100%) 0 (0%) | 0.06 |
| Ventilator brand | Philips® (n = 87) | 45 (78.9%) | 42 (66.7%) | 0.13 |
| ResMed® (n = 15) | 8 (14%) | 7 (11.1%) | 0.63 | |
| BREAS® (n = 13) | 4 (7%) | 9 (14.3%) | 0.20 | |
| Other (n = 5) | 0 (0%) | 5 (7.9%) | ||
| Interface model | Oronasal (n = 111) | 51 (89.5%) | 60 (95.2%) | 0.23 |
| Nasal (n = 6) | 3 (5.3%) | 3 (4.8%) | 0.90 | |
| Tracheostomy (n = 3) | 3 (5.3%) | 0 (0%) | ||
| Mechanical insuflation-exuflation device | Indication Infectiveness | 48 (84.2%) 15 (26.3%) | 47 (74.6%) 18 (28.6%) | 0.20 0.78 |
| Visits | 1 (n = 57) | 2 (n = 57) | 3 (n = 56) | 4 (n = 43) | 5 (n = 32) | 6 (n = 23) | 7 (n = 10) | 8 (n = 6) |
|---|---|---|---|---|---|---|---|---|
| ALSFRS-R scale (points), mean ± SD | 26.5 ± 7.4 | 24.3 ± 8 | 21.5 ± 9.1 | 21.9 ± 8.5 | 20.0 ± 8.8 | 20.8 ± 8.8 | 20.2 ± 9.2 | 14.5 ± 6.8 |
| Hours of MV use (hours) mean ± SD | 7.50 ± 3.07 | 8.87 ± 4.21 | 11.02 ± 4.84 | 11.53 ± 5.15 | 11.87 ± 6.14 | 12.56 ± 6.87 | 13.60 ± 6.51 | 16.17 ± 7.10 |
| Spontaneous inspirations (%), mean ± SD | 70.17 ± 24.61 | 64.29 ± 26.08 | 61.63 ± 27.25 | 56.65 ± 29.89 | 53.84 ± 32.88 | 46.75 ± 34.60 | 50.82 ± 38.42 | 38.40 ± 37.29 |
| Unintentional leak > 20 L/min (%) | 33 | 23 | 39 | 26 | 34 | 22 | 33 | 33 |
| Asynchronies (%) | 53 | 51 | 43 | 42 | 44 | 26 | 42 | 33 |
| Variable | Hazard Ratio | Std. Error | z | p-Value | 95% CI |
|---|---|---|---|---|---|
| BVS group (vs. non-BVS) | 0.18 | 0.04 | −7.21 | <0.001 | 0.11–0.27 |
| Male (vs. female) | 1.15 | 0.27 | 0.60 | 0.546 | 0.73–1.82 |
| Mechanical insufflation–exsufflation (vs. no) | 0.99 | 0.21 | −0.05 | 0.957 | 0.65–1.51 |
| Bulbar onset (vs. spinal) | 1.07 | 0.25 | 0.32 | 0.687 | 0.69–1.68 |
| ≥64 years old at diagnosis (vs. <64 years) | 0.83 | 0.19 | −0.79 | 0.428 | 0.53–1.30 |
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Hernández-Voth, A.; Sayas-Catalán, J.; Corral-Blanco, M.; Jiménez-Gómez, M.; Carvajal-Cuesta, G.; Luján-Torné, M.; Lalmolda-Puyol, C.; Florez-Solarana, P.; Villena-Garrido, V. Impact of Built-In Software Monitoring on Survival in Amyotrophic Lateral Sclerosis Patients Receiving Home Mechanical Ventilation: A Cohort Study. J. Clin. Med. 2026, 15, 1513. https://doi.org/10.3390/jcm15041513
Hernández-Voth A, Sayas-Catalán J, Corral-Blanco M, Jiménez-Gómez M, Carvajal-Cuesta G, Luján-Torné M, Lalmolda-Puyol C, Florez-Solarana P, Villena-Garrido V. Impact of Built-In Software Monitoring on Survival in Amyotrophic Lateral Sclerosis Patients Receiving Home Mechanical Ventilation: A Cohort Study. Journal of Clinical Medicine. 2026; 15(4):1513. https://doi.org/10.3390/jcm15041513
Chicago/Turabian StyleHernández-Voth, Ana, Javier Sayas-Catalán, Marta Corral-Blanco, Miguel Jiménez-Gómez, Gema Carvajal-Cuesta, Manel Luján-Torné, Cristina Lalmolda-Puyol, Pablo Florez-Solarana, and Victoria Villena-Garrido. 2026. "Impact of Built-In Software Monitoring on Survival in Amyotrophic Lateral Sclerosis Patients Receiving Home Mechanical Ventilation: A Cohort Study" Journal of Clinical Medicine 15, no. 4: 1513. https://doi.org/10.3390/jcm15041513
APA StyleHernández-Voth, A., Sayas-Catalán, J., Corral-Blanco, M., Jiménez-Gómez, M., Carvajal-Cuesta, G., Luján-Torné, M., Lalmolda-Puyol, C., Florez-Solarana, P., & Villena-Garrido, V. (2026). Impact of Built-In Software Monitoring on Survival in Amyotrophic Lateral Sclerosis Patients Receiving Home Mechanical Ventilation: A Cohort Study. Journal of Clinical Medicine, 15(4), 1513. https://doi.org/10.3390/jcm15041513

