REINVENT: ERS International survey on REstrictive thoracic diseases IN long term home noninvasive VENTilation

Abstract

Background and aim Little is known about the current use of long-term home non-invasive ventilation (LTHNIV) in restrictive thoracic diseases (RTD), including chest wall and neuromuscular disorders (CWD, NMD). This study aimed to capture the pattern of LTHNIV in RTD patients via a web-based international survey. Methods The survey involved ERS Assembly 2.02 (NIV dedicated group) October-December 2019. Results 166/748 (22.2%) members from 41 countries responded; 80% were physicians, of whom 43% worked in a respiratory intermediate intensive care unit (RIICU). The NMD:CWD ratio was 5:1, Amyotrophic lateral sclerosis (ALS) being 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, pressure support ventilation spontaneous-timed (PSV-ST) 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 RTD patients. NMD and, specifically, ALS were the main indications for LTHNIV. NIV was started mostly because of diurnal hypoventilation with a primary goal of patient-centred benefits. Bi-level PSV-ST and oro-nasal masks were more likely to be chosen for providing NIV. LTHNIV efficacy was assessed mainly in an outpatient setting.