{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,5]],"date-time":"2026-05-05T23:08:46Z","timestamp":1778022526624,"version":"3.51.4"},"reference-count":37,"publisher":"MDPI AG","issue":"22","license":[{"start":{"date-parts":[[2025,11,18]],"date-time":"2025-11-18T00:00:00Z","timestamp":1763424000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JCM"],"abstract":"<jats:p>Background: The impact of Long-Term Non-Invasive Ventilation (LT-NIV) on patient-relevant outcomes has yet to be clearly established in types 2 and 3 Spinal Muscular Atrophy (SMA). Objectives: The current study aimed to assess LT-NIV effect(s) on tracheostomy-free survival and the need for hospitalization. Its secondary aim was to identify patient characteristics that can be considered risk factors for an unsatisfactory response to treatment. Design: This study is a retrospective analysis of long-term outcomes in types 2 and 3 SMA patients receiving home LT-NIV. Methods:Patients and interventions: Eighteen types 2 and 3 SMA patients who were receiving home LT-NIV between 1 January 1990 and 31 March 2025 were included. Primary study endpoint: The endpoint comprised a composite of tracheostomy-free survival time and hospitalization rate. Results: Twelve patients (66.7%) had a positive response to LT-NIV (group A); the response was unsatisfactory in the remaining 6 (33.3%) (group B). Tracheostomy-free survival time was significantly reduced in the latter compared to the former [66 (range: 2\u2013172) vs. 280 (range: 67\u2013407) months; p = 0.010] and the hospitalization rate was significantly higher [0.35 (range: 0.06\u20131.44) vs. 0.07 (range: 0.00\u20130.18) episodes\/yr; p = 0.007]. A correlation was found between an unsatisfactory response to LT-NIV and treatment initiation following an episode of acute respiratory failure (OR: 7.90; 95% CI, 0.99\u2013123.2; p = 0.051). Conclusions: LT-NIV has a positive impact on tracheostomy-free survival and hospitalizations in types 2 and 3 SMA patients. The risk of an unsatisfactory response appears to be higher when the treatment is initiated in clinically unstable patients.<\/jats:p>","DOI":"10.3390\/jcm14228171","type":"journal-article","created":{"date-parts":[[2025,11,19]],"date-time":"2025-11-19T12:55:31Z","timestamp":1763556931000},"page":"8171","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["The Effect of Long-Term Non-Invasive Ventilation on Tracheostomy-Free Survival and Hospitalizations in Types 2 and 3 Spinal Muscular Atrophy Patients"],"prefix":"10.3390","volume":"14","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-8790-6029","authenticated-orcid":false,"given":"Andrea","family":"Vianello","sequence":"first","affiliation":[{"name":"Department of Cardiac, Thoracic, Vascular Sciences and Public Health, University of Padova, Via Giustiniani 2, 35128 Padova, Italy"},{"name":"Fisiopatologia Respiratoria, Ospedale-Universit\u00e0 di Padova, Via Giustiniani 2, 35128 Padova, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3664-2024","authenticated-orcid":false,"given":"Gabriella","family":"Guarnieri","sequence":"additional","affiliation":[{"name":"Department of Cardiac, Thoracic, Vascular Sciences and Public Health, University of Padova, Via Giustiniani 2, 35128 Padova, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3855-0916","authenticated-orcid":false,"given":"Leonardo","family":"Bertagna De Marchi","sequence":"additional","affiliation":[{"name":"Department of Cardiac, Thoracic, Vascular Sciences and Public Health, University of Padova, Via Giustiniani 2, 35128 Padova, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8891-7061","authenticated-orcid":false,"given":"Beatrice","family":"Molena","sequence":"additional","affiliation":[{"name":"Department of Cardiac, Thoracic, Vascular Sciences and Public Health, University of Padova, Via Giustiniani 2, 35128 Padova, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Giovanna","family":"Arcaro","sequence":"additional","affiliation":[{"name":"Department of Cardiac, Thoracic, Vascular Sciences and Public Health, University of Padova, Via Giustiniani 2, 35128 Padova, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0009-0003-0454-2464","authenticated-orcid":false,"given":"Giuliana","family":"Capece","sequence":"additional","affiliation":[{"name":"Department of Neuroscience, University of Padova, Via Giustiniani 2, 35128 Padova, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Elena","family":"Sogus","sequence":"additional","affiliation":[{"name":"Department of Neuroscience, University of Padova, Via Giustiniani 2, 35128 Padova, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0009-0003-3945-7622","authenticated-orcid":false,"given":"Federico","family":"Lionello","sequence":"additional","affiliation":[{"name":"Department of Cardiac, Thoracic, Vascular Sciences and Public Health, University of Padova, Via Giustiniani 2, 35128 Padova, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7740-4156","authenticated-orcid":false,"given":"Elena","family":"Pegoraro","sequence":"additional","affiliation":[{"name":"Department of Neuroscience, University of Padova, Via Giustiniani 2, 35128 Padova, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2025,11,18]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"71","DOI":"10.1186\/1750-1172-6-71","article-title":"Spinal muscular atrophy","volume":"6","author":"Mercuri","year":"2011","journal-title":"Orphanet J. 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