Background and aim: Little is known about the current use of long-term home noninvasive ventilation (LTHNIV) in restrictive thoracic diseases, including chest wall disorders and neuromuscular disorders (NMD). This study aimed to capture the pattern of LTHNIV in patients with restrictive thoracic diseases via a web-based international survey.

Methods: The survey involved European Respiratory Society (ERS) Assembly 2.02 (NIV-dedicated group), from October to December 2019.

Results: 166 (22.2%) out of 748 members from 41 countries responded; 80% were physicians, of whom 43% worked in a respiratory intermediate intensive care unit. The ratio of NMD to chest wall disorders was 5:1, with amyotrophic lateral sclerosis the most frequent indication within NMD (78%). The main reason to initiate LTHNIV was diurnal hypercapnia (71%). Quality of life/sleep was the most important goal to achieve. In 25% of cases, clinicians based their choice of the ventilator on patients' feedback. Among NIV modes, spontaneous-timed pressure support ventilation (ST-PSV) was the most frequently prescribed for day- and night-time. Mouthpieces were the preferred daytime NIV interface, whereas oro-nasal masks the first choice overnight. Heated humidification was frequently added to LTHNIV (72%). Single-limb circuits with intentional leaks (79%) were the most frequently prescribed. Follow-up was most often provided in an outpatient setting.

Conclusions: This ERS survey illustrates physicians' practices of LTHNIV in patients with restrictive thoracic diseases. NMD and, specifically, amyotrophic lateral sclerosis were the main indications for LTHNIV. NIV was started mostly because of diurnal hypoventilation with a primary goal of patient-centred benefits. Bi-level ST-PSV and oro-nasal masks were more likely to be chosen for providing NIV. LTHNIV efficacy was assessed mainly in an outpatient setting.