RationaleThe conventional approach of low-intensity non-invasive positive pressure ventilation (NPPV) produces only minimal physiological and clinical benefits in patients with stable hypercapnic chronic obstructive pulmonary disease (COPD). Objectives To determine whether the novel approach of high-intensity NPPV is superior to low-intensity NPPV in controlling nocturnal hypoventilation. Methods A randomised controlled crossover trial comparing 6 weeks of high-intensity NPPV (using controlled ventilation with mean inspiratory pressures of 28.661.9 mbar) with low-intensity NPPV (using assisted ventilation with mean inspiratory pressures of 14.660.8 mbar) was performed in 17 patients with severe stable hypercapnic COPD. Results Two patients refused low-intensity NPPV and two patients dropped out while on low-intensity NPPV. Thirteen patients (mean forced expiratory volume in 1 s (FEV 1 ) 0.7660.29 l) completed the trial. High-intensity NPPV produced higher pneumotachographicallymeasured expiratory volumes, with a mean treatment effect of 96 ml (95% CI 23 to 169) (p¼0.015). This resulted in a mean treatment effect on nocturnal arterial carbon dioxide tension (Paco 2 ) of À9.2 mm Hg (95% CI À13.7 to À4.6) (p¼0.001) in favour of high-intensity NPPV. Daily use of NPPV was increased in high-intensity NPPV compared with low-intensity NPPV, with a mean difference of 3.6 h/day (95% CI 0.6 to 6.7) (p¼0.024). In addition, compared with baseline, only high-intensity NPPV resulted in significant improvements in exerciserelated dyspnoea, daytime Paco 2 , FEV 1 , vital capacity and the Severe Respiratory Insufficiency Questionnaire Summary Score. Conclusions High-intensity NPPV is better tolerated by patients with severe chronic hypercapnic COPD and has been shown to be superior to the conventional and widely-used form of low-intensity NPPV in controlling nocturnal hypoventilation. High-intensity NPPV therefore offers a new promising therapeutic option for these patients.

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BackgroundWhile the role of acute non-invasive ventilation (NIV) has been shown to improve outcome in acute life-threatening hypercapnic respiratory failure in COPD, the evidence of clinical efficacy of long-term home NIV (LTH-NIV) for management of COPD is less. This document provides evidence-based recommendations for the clinical application of LTH-NIV in chronic hypercapnic COPD patients.Materials and methodsThe European Respiratory Society task force committee was composed of clinicians, methodologists and experts in the field of LTH-NIV. The committee developed recommendations based on the GRADE (Grading, Recommendation, Assessment, Development and Evaluation) methodology. The GRADE Evidence to Decision framework was used to formulate recommendations. A number of topics were addressed under a narrative format which provides a useful context for clinicians and patients.ResultsThe task force committee delivered conditional recommendations for four actionable PICO (target population-intervention-comparator-outcome) questions, 1) suggesting for the use of LTH-NIV in stable hypercapnic COPD; 2) suggesting for the use of LTH-NIV in COPD patients following a COPD exacerbation requiring acute NIV 3) suggesting for the use of NIV settings targeting a reduction in carbon dioxide and 4) suggesting for using fixed pressure support as first choice ventilator mode.ConclusionsManaging hypercapnia may be an important intervention for improving the health outcome of COPD patients with chronic respiratory failure. The task force conditionally supports the application of LTH-NIV to improve health outcome by targeting a reduction in carbon dioxide in COPD patients with persistent hypercapnic respiratory failure. These recommendations should be applied in clinical practice by practitioners that routinely care for chronic hypercapnic COPD patients.

Background: The objective of the present analysis is to describe the outcomes of high-intensity non-invasive positive pressure ventilation (NPPV) aimed at maximally decreasing PaCO2 as an alternative to conventional NPPV with lower ventilator settings in stable hypercapnic COPD patients.Methods: Physiological parameters, exacerbation rates and long-term survival were assessed in 73 COPD patients (mean FEV1 30±12 %predicted) who were established on high-intensity NPPV due to chronic hypercapnic respiratory failure between March 1997 and May 2006.Results: Controlled NPPV with breathing frequencies of 21±3 breath/min and mean inspiratory/expiratory positive airway pressures of 28±5/5±1 cmH2O led to significant improvements in blood gases, lung function and hematocrit after two months. Only sixteen patients (22%) required hospitalisation due to exacerbation during the first year, with anaemia increasing the risk for exacerbation. Two- and five-year survival rates of all patients were 82% and 58%, respectively. The five year survival rate was 32% and 83% in patients with low (≤39%) and high (≥55%) hematocrit, respectively.Conclusion: High-intensity NPPV improves blood gases, lung function and hematocrit, and is also associated with low exacerbation rates and a favourable long-term outcome. The current report strongly emphasises the need for randomised controlled trials evaluating the role of high-intensity NPPV in stable hypercapnic COPD patients.

It was hypothesised that noninvasive positive-pressure ventilation (NPPV) applied during walking prevents exercise-induced hypoxaemia and improves exercise performance in severe chronic obstructive pulmonary disease (COPD) patients already receiving long-term NPPV.A total of 20 COPD patients (mean¡SD age 65.1¡8.7 yrs, forced expiratory volume in one second 27¡8% predicted and total lung capacity 116¡27% pred) reporting dyspnoea, even during mild exertion, underwent two 6-min walking tests with a rollator and supplemental oxygen (2.1¡0.9 L?min -1 ) in a randomised cross-over design: with and without pressure-limited NPPV as used at home (inspiratory:expiratory pressure 2.9¡0.44:0.4¡0.1 kPa (29¡4:4¡1 mbar), respiratory frequency 20¡2 breaths?min -1 ).The arterial oxygen tension significantly increased by 1.39¡1.43 kPa (95% confidence interval (CI) 0.71-2.07 kPa) after walking with NPPV, but significantly decreased by 1.43¡1.06 kPa (95% CI -1.92 --0.94 kPa) without NPPV. Dyspnoea, as assessed by the Borg dyspnoea scale, significantly decreased from 6 (interquartile range (IQR) 4.5-10) to 4 (1.5-4.5) and walking distance significantly increased from 209 (IQR 178-279) to 252 (203-314) m when walking was NPPV-aided.In chronic hypercapnic chronic obstructive pulmonary disease, high-intensity noninvasive positive-pressure ventilation can also be administered during walking with unchanged ventilator settings compared with settings used at rest, thus resulting in improved oxygenation, decreased dyspnoea and increased walking distance. Therefore, noninvasive positive-pressure ventilation during walking could prevent hypoxia-induced complications and could, in future, play a role in palliative care.

DRKS00000433 at http://apps.who.int/trialsearch/default.aspx.

Today, invasive and non-invasive home mechanical ventilation have become a well-established treatment option. Consequently, in 2010 the German Society of Pneumology and Mechanical Ventilation (DGP) has leadingly published the guidelines on "Non-Invasive and Invasive Mechanical Ventilation for Treatment of Chronic Respiratory Failure". However, continuing technical evolutions, new scientific insights, and health care developments require an extensive revision of the guidelines.For this reason, the updated guidelines are now published. Thereby, the existing chapters, namely technical issues, organizational structures in Germany, qualification criteria, disease specific recommendations including special features in pediatrics as well as ethical aspects and palliative care, have been updated according to the current literature and the health care developments in Germany. New chapters added to the guidelines include the topics of home mechanical ventilation in paraplegic patients and in those with failure of prolonged weaning.In the current guidelines different societies as well as professional and expert associations have been involved when compared to the 2010 guidelines. Importantly, disease-specific aspects are now covered by the German Interdisciplinary Society of Home Mechanical Ventilation (DIGAB). In addition, societies and associations directly involved in the care of patients receiving home mechanical ventilation have been included in the current process. Importantly, associations responsible for decisions on costs in the health care system and patient organizations have now been involved.The currently updated guidelines are valid for the next three years, following their first online publication on the home page of the Association of the Scientific Medical Societies in German (AWMF) in the beginning of July 2017. A subsequent revision of the guidelines remains the aim for the future.

Background: The number of patients with invasive home mechanical ventilation (HMV) following unsuccessful weaning is steadily increasing, but little is known about the living conditions and health-related quality of life (HRQL) in these patients. Objectives: To establish detailed information on living conditions and HRQL in patients with invasive HMV. Methods: The Severe Respiratory Insufficiency Questionnaire (SRI) was used to measure specific HRQL aspects in addition to patient interviews on individual living conditions during home visits. Results: Thirty-two patients with lung disease, most prominently COPD (n = 18), and neuromuscular disorders (n = 14) were included. The overall mean SRI summary scale score (range 0-100) was 53 ± 16, with a broad range amongst individuals (23-86). Neuromuscular patients were younger than those with lung diseases (49 ± 18 vs. 67 ± 11 years; p < 0.005), and although they had a higher nursing dependency and fewer comorbidities, they tended to have higher (better) SRI summary scale scores (58 ± 16 vs. 48 ± 15; p = 0.092). Living in a private home compared to living in nursing facilities did not influence the SRI scores. Conclusions: Patients undergoing invasive HMV primarily following unsuccessful weaning reported an individual HRQL which, when taken together, was highly heterogeneous and ranged from very good to extremely bad. Older patients with COPD and more comorbidities are likely to have a worse HRQL than neuromuscular patients, while the living situation does not influence the HRQL.