Intermittent abdominal pressure ventilation: feasibility and efficacy in neuromuscular disease. A case report

• Main Authors: Cristina Puricelli, Eleonora Volpato, Salvatore Sciurello, Antonello Nicolini, Paolo Banfi
• Format: Article
• Language: English
• Published: PAGEPress Publications 2021-08-01
• Series: Monaldi Archives for Chest Disease
• Subjects: Neuromuscular disease; daytime ventilation; non-invasive ventilation
• Online Access: https://monaldi-archives.org/index.php/macd/article/view/1828

he standard treatment for patients with neuromuscular respiratory failure is non-invasive ventilation (NIV) as non-invasive ventilation support-setting (NVS). NVS is administered through a nasal or face mask and/or mouthpiece with the potential to cause nasal ulcers, discomfort, and/or aesthetic issues, resulting in poor compliance. We reported the observation of a 45-year-old woman with limb-girdle muscular dystrophy (LGMD), secondary to Dysferlin deficiency, who was on NVS since 2017 for nocturnal hypoventilation. In 2018, despite nocturnal ventilation, due to weight gain and daytime hypoventilation, a nasal mask was introduced. We initiated daytime intermittent abdominal pressure ventilation (IAPV) to mitigate cosmetic problems, improving in pO2 and decreasing in pCO2 versus baseline (52>84 mmHg, 46>33 mmHg respectively) at 6 (85 mmHg, 42 mmHg) and 18 months (93 mmHg, 38 mmHg), respectively. IAPV was effective, safe, and well-tolerated in our patients who did not tolerate standard daytime NVS with the known interface.