Clinical benefit of chronic non-invasive ventilation in severe stable COPD: a matter of persistent hypercapnia improvement.

Purpose: In patients with chronic obstructive pulmonary disease (COPD) treated with chronic non-invasive ventilation (NIV), the relation between improvements in nocturnal transcutaneous partial pressure of CO (PtcCO) and daytime arterial partial pressure of CO (PaCO) remains uncertain. Also, to what extent improvements in nocturnal PtcCO result in better health-related quality of life (HRQL), exercise capacity, lung function and survival has not been investigated.

Patients And Methods: Patients with COPD who were initiated on chronic NIV were prospectively followed for 6 months.

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.

Future Directions for Respiratory Muscle Training in Neuromuscular Disorders: A Scoping Review.

Background: Respiratory muscle training (RMT) aims to improve inspiratory and/or expiratory muscle function in neuromuscular disorders (NMDs). A comprehensive overview of the available literature is lacking. This scoping review explores methodological characteristics, (adverse) effects, and adherence of RMT studies in NMDs.

Continuous and bilevel positive airway pressure may improve radiotherapy delivery in patients with intra-thoracic tumors.

Background: Minimizing tumor motion in radiotherapy for intra-thoracic tumors reduces side-effects by limiting radiation exposure to healthy tissue. Continuous or Bilevel Positive Airway Pressure (CPAP/BiPAP) could achieve this, since it could increase lung inflation and decrease tidal volume variability. We aim to identify the better CPAP/BiPAP setting for minimizing tumor motion.

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.

Characteristics and outcomes in children with congenital central hypoventilation syndrome on long-term mechanical ventilation in the Netherlands.

Unlabelled: Congenital central hypoventilation syndrome (CCHS) is a rare condition characterized by central hypoventilation, leading to the majority of patients being dependent on ventilatory support during sleep. This condition is often accompanied by various associated symptoms, due to a PHOX2B gene variant involved in neuronal crest cell migration. This study is the first to review the characteristics and outcomes in children with CCHS on long-term mechanical ventilation in the Netherlands.

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.

Respiratory features of centronuclear myopathy in the Netherlands.

Centronuclear myopathy (CNM) is a heterogeneous group of muscle disorders primarily characterized by muscle weakness and variable degrees of respiratory dysfunction caused by mutations in MTM1, DNM2, RYR1, TTN and BIN1. X-linked myotubular myopathy has been the focus of recent natural history studies and clinical trials. Data on respiratory function for other genotypes is limited.

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.

Starting HMV at home: a reasonable option for many patients?

Background And Objective: In the current study, we undertook a more detailed exploration of the reasons why patients undergoing HMV were screened out of a recently published study in order to better understand how applicable home initiation of HMV is under real life conditions.

Methods: All referred patients who had an indication for starting HMV were screened to participate in the Homerun study. In this trial 512 patients were screened out of the study.

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.

Home mechanical ventilation: the Dutch approach.

In the Netherlands we have an unique organisation of only 4 centres being responsible for all patients who need Home Mechanical ventilation(HMV). Nationwide criteria for referral and initiation of HMV are stated in our national guideline and recently a unique national learning management system (LMS) for all caregivers and professionals was developed. A nationwide multi-centric research program is running and every centre is participating.