Defining a phenotype of severe COPD patients who develop chronic hypercapnia.

Introduction: Chronic hypercapnia, defined by elevated blood CO levels, is a serious complication most prevalent in severe COPD. It negatively impacts quality of life, increases hospitalization rates, and elevates mortality risks. However, not all severe COPD patients develop chronic hypercapnia, and its underlying mechanisms remain unclear.

Deventilation Syndrome in Patients with Chronic Obstructive Pulmonary Disease Using Nocturnal Noninvasive Ventilation: What Are the Underlying Mechanisms?

Introduction: Patients with chronic obstructive pulmonary disease (COPD) commonly experience severe dyspnea after discontinuation of nocturnal noninvasive ventilation (NIV), known as deventilation syndrome (DVS), which negatively affects quality of life. Despite various hypotheses, the precise mechanisms of DVS remain unknown.

Methods: An observational pilot study was performed monitoring 16 stable COPD patients before, during, and after an afternoon nap on NIV.

Initiation of Chronic Non-invasive Ventilation.

Initiation of home non-invasive ventilation (NIV) requires careful consideration of the patient's condition, motivation, expectations, wishes, and social circumstances. The decision to start NIV depends on a combination of factors including patient symptoms and objective evidence of nocturnal hypoventilation. A solid understanding of the underlying pathophysiology is key to a systematic and well-balanced clinical approach to titrating NIV.

Validity and reliability of the Dutch version of the S3-NIV questionnaire to evaluate long-term noninvasive ventilation.

Objectives: Noninvasive ventilation (NIV) is an effective treatment for chronic respiratory failure (CRF). Patient-centered outcomes need to be evaluated regularly and the S-NIV questionnaire seems an applicable tool. We translated this short, self-administered questionnaire into a Dutch version and tested its construct validity and reliability.

On the relation between tidal and forced spirometry.

Spirometry is a lung function test involving deep inhalation and forceful deep exhalation. It is widely used to obtain objective information about airflow limitation and to diagnose lung diseases. In contrast, tidal spirometry is based on normal breathing and therefore much more convenient, but it is hardly used in medical care and its relation with conventional (forced) spirometry is largely unknown.

Home noninvasive ventilation in severe COPD: in whom does it work and how?

Background: Not all hypercapnic COPD patients benefit from home noninvasive ventilation (NIV), and mechanisms through which NIV improves clinical outcomes remain uncertain. We aimed to identify "responders" to home NIV, denoted by a beneficial effect of NIV on arterial partial pressure of carbon dioxide (), health-related quality of life (HRQoL) and survival, and investigated whether NIV achieves its beneficial effect through an improved .

Methods: We used individual patient data from previous published trials collated for a systematic review.

Respiratory function in LAMA2-related muscular dystrophy and SELENON-related congenital myopathy, a 1.5-year natural history study.

Introduction: LAMA2-related muscular dystrophy (LAMA2-MD) and SELENON(SEPN1)-related congenital myopathy (SELENON-RM) are rare neuromuscular diseases with respiratory impairment from a young age. Prospective natural history studies are needed for prevalence estimations, respiratory characterization, optimizing clinical care and selecting outcome measures for trial readiness.

Methods: Our prospective 1.

Role of respiratory characteristics in treatment adherence with noninvasive home mechanical ventilation in myotonic dystrophy type 1, a retrospective study.

Chronic respiratory insufficiency is common in patients with myotonic dystrophy type 1 (DM1) and can be treated with noninvasive home mechanical ventilation (HMV). HMV is not always tolerated well resulting in low treatment adherence. We aimed to analyze if baseline respiratory characteristics such as pulmonary function, level of pCO and presence of sleep apnea are associated with HMV treatment adherence in DM1 patients.

Clinical practice of non-invasive ventilation for acute exacerbations of chronic obstructive pulmonary disease.

Background: Non-invasive ventilation (NIV) is an evidence-based treatment for acute respiratory failure in chronic obstructive pulmonary disease (COPD). However, suboptimal application of NIV in clinical practice, possibly due to poor guideline adherence, can impact patient outcomes. This study aims to evaluate guideline adherence to NIV for acute COPD exacerbations and explore its impact on mortality.

Effect of Intensity of Home Noninvasive Ventilation in Individuals With Neuromuscular and Chest Wall Disorders: A Systematic Review and Meta-Analysis of Individual Participant Data.

Introduction: Home noninvasive ventilation (NIV), targeting a reduction of carbon dioxide with a combination of sufficient inspiratory support and backup-rate improves outcomes in patients with chronic obstructive pulmonary disease. The aim of this systematic review with individual participant data (IPD) meta-analysis was to evaluate the effects of intensity of home NIV on respiratory outcomes in individuals with slowly progressive neuromuscular (NMD) or chest-wall disorders (CWD).

Methods: Controlled, non-controlled and cohort studies indexed between January-2000 and December-2020 were sought from Medline, Embase and the Cochrane Central Register.

Building a home ventilation programme: population, equipment, delivery and cost.

Home mechanical ventilation (HMV) improves quality of life and survival in patients with neuromuscular disorders (NMD). Developing countries may benefit from published evidence regarding the prevalence, cost of equipment, technical issues and organisation of HMV in NMD, facilitating the development of local turn-key HMV programmes. Unfortunately, such evidence is scattered in the existing literature.

Upper airway pressure distribution during nasal high-flow therapy.

Two working mechanisms of Nasal High-Flow Therapy (NHFT) are washout of anatomical dead space and provision of positive end-expiratory pressure (PEEP). The extent of both mechanisms depends on the respiration aerodynamics and the corresponding pressure distribution: at end-expiration the onset of uniform pressure indicates the jet penetration length, and the level of the uniform pressure is the PEEP. The clinical problem is that adequate measurements in patients are presently impossible.

Long-term follow-up of respiratory function in facioscapulohumeral muscular dystrophy.

Objective: To evaluate the 5-year change in respiratory function in patients with facioscapulohumeral muscular dystrophy (FSHD).

Methods: Genetically confirmed patients with FSHD aged ≥ 18 years were examined twice over five years. Forced vital capacity (FVC) and forced expiratory volume in 1 s (FEV1) were measured using hand-held spirometry with a face mask.

Cardiovascular and metabolic effects of a mandibular advancement device and continuous positive airway pressure in moderate obstructive sleep apnea: a randomized controlled trial.

Study Objectives: It has been suggested that treatment for obstructive sleep apnea (OSA) reduces cardiovascular risk. So far, knowledge is limited about the difference in the reduction of this risk between mandibular advancement device (MAD) and continuous positive airway pressure (CPAP) therapy. The aim of this study was to compare the cardiovascular effects of MAD vs CPAP therapy in patients with moderate OSA.

Chronic non-invasive ventilation for chronic obstructive pulmonary disease.

Background: Chronic non-invasive ventilation (NIV) is increasingly being used to treat people with COPD who have respiratory failure, but the evidence supporting this treatment has been conflicting.

Objectives: To assess the effects of chronic non-invasive ventilation at home via a facial mask in people with COPD, using a pooled analysis of IPD and meta-analysis.

Search Methods: We searched the Cochrane Airways Register of Trials, MEDLINE, Embase, PsycINFO, CINAHL, AMED, proceedings of respiratory conferences, clinical trial registries and bibliographies of relevant studies.

Diaphragm Pacing in Patients with Spinal Cord Injury: A European Experience.

Background: Patients with high spinal cord injury (SCI) are unable to breathe on their own and require mechanical ventilation (MV). The long-term use of MV is associated with increased morbidity and mortality. In patients with intact phrenic nerve function, patients can be partially or completely removed from MV by directly stimulating the diaphragm motor points with a diaphragm pacing system (DPS).

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

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 a web-based international survey.

Methods: The survey involved European Respiratory Society (ERS) Assembly 2.

Nasal versus oronasal masks for home non-invasive ventilation in patients with chronic hypercapnia: a systematic review and individual participant data meta-analysis.

Background: The optimal interface for the delivery of home non-invasive ventilation (NIV) to treat chronic respiratory failure has not yet been determined. The aim of this individual participant data (IPD) meta-analysis was to compare the effect of nasal and oronasal masks on treatment efficacy and adherence in patients with COPD and obesity hypoventilation syndrome (OHS).

Methods: We searched Medline and Cochrane Central Register of Controlled Trials for prospective randomised controlled trials (RCTs) of at least 1 month's duration, published between January 1994 and April 2019, that assessed NIV efficacy in patients with OHS and COPD.

Health outcomes of continuous positive airway pressure versus mandibular advancement device for the treatment of severe obstructive sleep apnea: an individual participant data meta-analysis.

Study Objectives: The impact of therapy with continuous positive airway pressure (CPAP) and mandibular advancement device (MAD) has not been directly compared in patients with severe obstructive sleep apnea (OSA). The purpose of this individual participant data meta-analysis was to compare the treatment effects of CPAP and titratable MAD on sleepiness, quality of life, sleep-disordered breathing severity, and sleep structure in patients with severe OSA.

Methods: Randomized controlled trials (RCTs) that included severe OSA patients were identified in order to compare the impact of the two treatments.