Volume 2024, Issue 1 7013576

Review Article

Open Access

Update in Noninvasive Home Mechanical Ventilation: A Narrative Review of Indications, Outcomes, and Monitoring

Laura Tregidgo,

Laura Tregidgo

orcid.org/0000-0003-1413-9356

Lane Fox Respiratory Unit , Guys and St Thomas’ NHS Foundation Trust , London , UK

Search for more papers by this author

Prasheena Naran,

Prasheena Naran

Department of Respiratory Medicine , Barts Health NHS Foundation Trust , London , UK

Search for more papers by this author

Eshrina Gosal,

Eshrina Gosal

Department of Respiratory Medicine , University College London Hospitals NHS Foundation Trust , London , UK , nhs.uk

Search for more papers by this author

Rebecca F. D’Cruz,

Corresponding Author

Rebecca F. D’Cruz

[email protected]

orcid.org/0000-0001-5245-9911

Lane Fox Respiratory Unit , Guys and St Thomas’ NHS Foundation Trust , London , UK

Centre for Human and Applied Physiological Sciences , King’s College London , London , UK , kcl.ac.uk

Search for more papers by this author

Laura Tregidgo,

Laura Tregidgo

orcid.org/0000-0003-1413-9356

Lane Fox Respiratory Unit , Guys and St Thomas’ NHS Foundation Trust , London , UK

Search for more papers by this author

Prasheena Naran,

Prasheena Naran

Department of Respiratory Medicine , Barts Health NHS Foundation Trust , London , UK

Search for more papers by this author

Eshrina Gosal,

Eshrina Gosal

Department of Respiratory Medicine , University College London Hospitals NHS Foundation Trust , London , UK , nhs.uk

Search for more papers by this author

Rebecca F. D’Cruz,

Corresponding Author

Rebecca F. D’Cruz

[email protected]

orcid.org/0000-0001-5245-9911

Lane Fox Respiratory Unit , Guys and St Thomas’ NHS Foundation Trust , London , UK

Centre for Human and Applied Physiological Sciences , King’s College London , London , UK , kcl.ac.uk

Search for more papers by this author

First published: 03 July 2024

https://doi.org/10.1155/2024/7013576

Academic Editor: Claudia Crimi

Share a link

Email
Wechat
Bluesky

Abstract

Hypercapnic respiratory failure arises due to an imbalance in the load-capacity-drive relationship of the respiratory muscle pump, typically arising in patients with chronic obstructive pulmonary disease, obesity-related respiratory failure, and neuromuscular disease. Patients at risk of developing chronic respiratory failure and those with established disease should be referred to a specialist ventilation unit for evaluation and consideration of home noninvasive ventilation (NIV) initiation. Clinical trials demonstrate that, following careful patient selection, home NIV can improve a range of clinical, patient-reported, and physiological outcomes. This narrative review provides an overview of the pathophysiology of chronic respiratory failure, evidence-based applications of home NIV, and monitoring of patients established on home ventilation and describes technological advances in ventilation devices, interfaces, and monitoring to enhance comfort, promote long-term adherence, and optimise gas exchange.

1. Introduction

Noninvasive ventilation (NIV) delivered in the home environment is an effective treatment for patients with chronic hypercapnic respiratory failure. Clinical trials evaluating its application in a range of disease groups demonstrate that, with careful patient selection, it can improve clinical, patient-reported, and physiological outcomes. This narrative review provides an overview of the pathophysiology of respiratory failure, principles of noninvasive ventilation, applications of home noninvasive ventilation, and the evidence supporting the application of home mechanical ventilation (HMV) for patients with chronic obstructive pulmonary disease (COPD), obesity-related respiratory failure, and neuromuscular disease (NMD), and methods of monitoring patients established on HMV.

2. Principles of Ventilatory Homeostasis

The primary function of the respiratory system is to facilitate gas exchange by supplying oxygen and removing carbon dioxide (CO₂) from the bloodstream to achieve ventilatory homeostasis. This process is achieved by two components: (1) the alveolar gas-exchange surface and (2) the respiratory muscle pump responsible for ventilation of the lungs [1]. The respiratory pump is comprised of inspiratory muscles, predominantly the diaphragm, and also intercostal and accessory muscles, airways, and lung parenchyma. Neural respiratory drive and ventilatory homeostasis are maintained by central respiratory control centres, which respond to feedback from central and peripheral chemoreceptors and mechano- and irritant-receptors in the chest wall and respiratory muscles, airway epithelium, and lung tissue [2].

Hypoxaemic respiratory failure occurs when there is an impairment of gas exchange at the alveolar-capillary level, resulting in inadequate oxygenation. This can be explained by four mechanisms: alveolar hypoventilation, ventilation-perfusion mismatch, diffusion defects, or right-to-left shunt [1]. Hypercapnic respiratory failure is caused by an imbalance in the loads and capacity of the respiratory muscle pump and inadequate neural respiratory drive to maintain ventilatory homeostasis [3] (Figure 1).

Details are in the caption following the image — **Figure 1**
Open in figure viewer PowerPoint

Schematic illustrating the pathophysiology of chronic respiratory failure caused by a load-capacity-drive imbalance of the respiratory muscle pump. COPD, chronic obstructive pulmonary disease; PEEPi, intrinsic positive end-expiratory pressure; ERV, expiratory reserve volume.

Excess respiratory muscle loads can be classified into resistive, elastic, or threshold loads. Resistive loads occur in COPD, asthma, and cystic fibrosis as a consequence of airway inflammation, bronchospasm, and sputum production [4]. Resistive loads arise in obesity, NMD, and chest wall disease due to upper airway obstruction and reduced lung volumes and chest wall compliance [5]. Alveolar destruction in COPD imposes an elastic load and intrinsic positive end-expiratory pressure (PEEP_i), caused by expiratory flow limitation in COPD and early airway closure in obesity, and creates a threshold load. Respiratory muscle capacity may be reduced due to hyperinflation and impaired force-generating capacity of the diaphragm, or intrinsic respiratory muscle weakness caused by NMD [6].

3. Principles of Noninvasive Ventilation

Chronic respiratory failure is managed with the optimisation of the underlying condition and with mechanical ventilation. The goal of the therapy is to achieve correction of gas exchange. This involves the delivery of positive airway pressure which may be achieved invasively, via endotracheal or tracheostomy tube, or using NIV, via the upper airway. Mechanical ventilation is used to control hypercapnia, and supplementary oxygen corrects hypoxaemia.

Inspiratory positive airway pressure (IPAP) is the peak airway pressure delivered during inspiration, and expiratory positive airway pressure (EPAP) is the pressure delivered during expiration and has the effects of maintaining airway patency and facilitating alveolar recruitment. In individuals with PEEP_i, appropriately titrated EPAP can overcome this threshold load and improve the work of breathing, ventilator triggering, and patient-ventilator synchrony [7]. The difference between IPAP and EPAP is termed pressure support, which actively supports respiratory muscles and decreases work of breathing, overall acting to increase tidal volume and minute ventilation. This “bilevel” support contrasts with the fixed pressure delivered throughout the respiratory cycle by continuous positive airway pressure (CPAP), which is thus not a form of assisted ventilation. Inspiratory time (Ti) is set based on the underlying cause of respiratory failure, with a shorter Ti preferred for those with obstructive lung disease to permit adequate lung emptying and a longer Ti for those with NMD and obesity-related respiratory failure. A backup rate maintains ventilation when the patient does not initiate a breath, and consideration should be given to patient comfort as well as to optimising ventilatory control when this is set.

Different ventilator modes can be considered which can deliver fixed pressures (pressure-targeted ventilation) or a target tidal volume (volume-targeted pressure support). Pressure-targeted ventilation provides a preset IPAP and EPAP. Volume-targeted pressure support involves IPAP autotitration to achieve a preset target tidal volume, based on subjects’ ideal body weight [8]. Gas exchange, patient tolerance, and sleep quality are comparable between pressure- and volume-targeted modes [8, 9]. Furthermore, volume-targeted pressure support can facilitate delivery of higher preset maximum IPAP without deleteriously impacting comfort [10]. Specialist ventilation units may consider a mode in which both IPAP and EPAP are autotitrated to abolish upper airway obstruction and control hypoventilation [11].

Consideration should be given to the interface to optimise comfort and minimise excess unintentional leaks. A wide range that covers the mouth, nose, or both are commercially available. Oronasal is the most commonly implemented interface amongst HMV users, and individual participant data meta-analyses indicate that there is no difference in gas exchange or adherence between oronasal and nasal [12].

4. Applications of Home Mechanical Ventilation

4.1. Chronic Obstructive Pulmonary Disease

4.1.1. Pathophysiology of Respiratory Failure in COPD

COPD is a common respiratory condition and the third leading cause of death worldwide [13]. It is caused by inhalation of noxious particles, most commonly cigarette smoking and air pollution, which causes airway inflammation and alveolar destruction. The pathophysiological hallmark of COPD is expiratory flow limitation, which arises due to airway inflammation, bronchospasm, and mucus hypersecretion, which causes hyperinflation [14]. The development of chronic respiratory failure in COPD can be challenging to predict and is thought to affect approximately one-third of patients with severe disease [15]. Hypercapnic respiratory failure may present acutely de novo with respiratory acidosis or develop over time with compensated respiratory failure.

Chronic hypercapnia has both cardiovascular and musculoskeletal implications in COPD [16, 17] and is independently associated with mortality risk [18]. The aim of home NIV initiation is to correct hypercapnia and improve patient-centred outcomes, including hospitalisation risk, mortality, symptom burden, and health-related quality of life (HRQoL). Clinical practice guidelines on the use of domiciliary NIV to manage chronic respiratory failure in COPD are based on contemporary randomised clinical trials (RCT) which evaluated its application in patients with (1) stable hypercapnia and (2) persistent hypercapnia following acute hypercapnic episode requiring NIV, detailed as follows [19, 20].

4.1.2. Stable Hypercapnic Respiratory Failure

Current international guidelines make a conditional recommendation for the use of domiciliary NIV in stable patients with COPD and chronic hypercapnia [21]. Early RCTs implementing low-pressure ventilation (average IPAP range: 12–14; EPAP range: 2–5) demonstrated no clear benefit on gas exchange, survival, hospitalisation, or exacerbation rate and could have a deleterious effect on HRQoL [22–24]. Kohnlein et al. then demonstrated that by targeting arterial CO₂ reduction of at least 20% or <6.5 kPa (49 mmHg) using a high-intensity approach of higher pressures and backup rate (BUR) (mean IPAP 22; EPAP 5; BUR 16), nocturnal HMV improves daytime gas exchange occurring as early as 14 days after treatment initiation, compared to control-arm participants receiving optimised COPD care alone with no NIV [19]. Furthermore, those patients receiving HMV had lower all-cause 1-year mortality with no associated impairment of HRQoL. Further research to identify patient characteristics related to treatment success and adherence will continue to help improve patient selection for HMV in stable COPD.

4.1.3. Persistent Hypercapnia following Acute Exacerbation

The benefits of HMV in patients requiring acute NIV for acute hypercapnic respiratory failure have been explored in two large RCTs. The RESCUE trial randomised inpatients admitted with life-threatening respiratory failure with persistent hypercapnia of >48 hours after liberation from mechanical ventilation to receive long-term oxygen therapy (LTOT) and NIV or LTOT alone [25]. In this study, gas exchange improved in those receiving HMV, however, there was no difference in time to readmission or death between the groups. It is possible that this was observed due to the inclusion of patients without chronic hypoxaemic respiratory failure and those in whom hypercapnia would naturally resolve during recovery from their acute illness and therefore had a better prognosis. Murphy et al. therefore designed the HOT-HMV to randomise patients with persistent hypercapnia 2–4 weeks following resolution of respiratory acidaemia to receive LTOT or LTOT and NIV [20]. This study demonstrated significant prolongation in time to readmission or death in the HMV + LTOT group compared to those receiving LTOT alone. In addition, the COPD exacerbation rate was lower in the HMV group. These trials highlight the importance of careful patient selection when considering HMV initiation for persistent postexacerbation hypercapnia.

Overall, there is robust evidence supporting the use of HMV in stable hypercapnic COPD patients and in those with persistent hypercapnia following an acute hypercapnic episode, which is reflected in international guideline recommendations [21]. Future research to investigate the optimal timing of NIV initiation may help to identify patients most likely to benefit from treatment. In addition, few trials have assessed NIV and its impact on long-term patient outcomes, which may provide further insight into patient selection.

4.2. Obesity-Related Respiratory Failure

Obesity-related respiratory failure (ORRF) is defined by the presence of obesity (BMI ≥30 kg/m²), sleep-disordered breathing (obstructive sleep apnoea (OSA) with an apnoea-hypopnea index of ≥5 events/hour, or nonobstructive hypoventilation with CO₂ rise, or deep desaturation without obstruction), and daytime hypercapnia PaCO₂ of ≥6 kPa (45 mmHg) in the absence of an alternative neuromuscular, mechanical, or metabolic explanation for hypoventilation [26]. It is common in the general population, with increasing prevalence and rising BMI, affecting >25% of those with a BMI of >40 kg/m² [27]. Patients with ORRF may initially present with respiratory failure during an acute hospital admission, in whom long-term mortality is more than double that of individuals admitted with simple obesity [28]. A proportion is identified in the sleep and bariatric service outpatient setting [29].

4.2.1. Pathophysiology of Obesity-Related Respiratory Failure

The pathophysiology of ORRF depends on changes to respiratory mechanics that arise as a result of fat deposition, and the subsequent impact this has on respiratory drive, alongside sleep-related changes noted in this cohort [30]. Work of breathing is impacted by the weight of the chest wall and surrounding abdomen, which is intensified in the supine position and thus during sleep [31]. Increased adipose deposition reduces lung compliance, diaphragmatic movement, and lung volumes (expiratory reserve volume and functional residual capacity) [30]. Early airway closure leads to gas trapping and PEEP_i, which further increases the work of breathing and ventilation-perfusion mismatch [32]. Concurrent OSA arises due to adipose tissue deposition around the upper airway causing increased collapsibility, leading to obstruction during sleep [30]. In ORRF, the neural respiratory drive response to hypercapnia is blunted. This is hypothesised to be secondary to leptin resistance [33]. Leptin acts as a ventilatory stimulant, and as such, resistance results in decreased response to hypercapnia, which manifests as hypoventilation.

Sleep apnoea is more common in those with hypervolaemic states such as congestive cardiac failure and end-stage renal failure. This occurs due to chronic nocturnal hypoxaemia, which potentiates vascular damage, eventually leading to organ dysfunction and interstitial fluid retention [34]. When in supine position, fluid shifts rostrally to the thorax and neck as hydrostatic pressures within lower limb capillaries are reduced due to the reduced effect of gravity. This allows fluid to easily move from the extravascular (interstitial) to the intravascular compartment and is redistributed around the body [35]. In ORRF, where the respiratory drive is already diminished, the patient cannot compensate, resulting in further CO₂ retention and progressive worsening of daytime hypercapnia [30].

4.2.2. Ventilatory Management of Obesity-Related Respiratory Failure

Positive airway pressure therapy, alongside lifestyle modification (dietetic measures and weight management), is first-line treatments in patients with ORRF, with the overall goal of correcting sleep-disordered breathing, arterial hypoxaemia, and daytime hypercapnia [30, 36]. Weight management is the optimal treatment and can be curative, but this is often difficult to achieve independently [37]. Depending on local services, some patients may be considered for bariatric surgery, which is an effective medium-term weight loss strategy although weight gain can recur in subsequent years [38]. Furthermore, the limited evidence on the effect of bariatric surgery on sleep-disordered breathing indicates an ongoing need for positive airway pressure therapy postoperatively [39]. Both CPAP and NIV have been identified as effective treatment modalities for sleep-disordered breathing in obese subjects. Several factors should be considered when selecting which therapy to apply, including patient phenotype (simple OSA, hypoventilation with OSA, or lone hypoventilation), device cost, and device complexity.

In the short term (3 months), there appear to be no significant differences in physiological or patient-reported outcomes between CPAP and NIV in patients with ORRF, excluding those with persistent hypercapnia or significant oxygen desaturation despite CPAP. Piper et al. observed no differences in daytime PaCO₂ reduction, weight loss, daytime somnolence, or treatment adherence between CPAP and NIV [40]. Using a primary outcome of treatment failure, as defined by nonadherence, hospital admission, or residual ventilatory failure (failure of PaCO2 to fall below 8 kPa (60 mmHg) or a rise of ≥1.3 kPa (10 mmHg), Howard et al. observed comparable improvements in daytime PaCO₂, HRQoL, and treatment adherence [41]. Persistent ventilatory failure was seen in both cohorts, and this was related to the severity of ventilatory failure at presentation (measured by PaCO2 at initial presentation). Patients in the highest quartile (PaCO2 >8.3 kPa, 62 mmHg) had an eightfold increased risk of persistent ventilatory failure. In addition, patients with more severe OSA were less likely to have persistent ventilatory failure, supporting the hypothesis that correcting upper airway obstruction during sleep (using CPAP or NIV) results in treatment response. Notably, this study excluded patients with evidence of clinical hypoventilation (persistent hypoxaemia) and severe hypercapnia and participants’ BMI was lower than in comparable trials [40, 42].

Medium- and long-term outcomes have been evaluated by Masa et al. in the Pickwick Project [42, 43]. This was a large, multicentre RCT comparing CPAP, NIV, and lifestyle measures in the treatment of ORRF with severe OSA (AHI >30 events/hour), initially using daytime hypercapnia as the primary outcome [42]. In the medium-term, both CPAP and NIV improved daytime hypercapnia in adherent (>4 hours usage/night) subjects compared to lifestyle measures alone. In the long term, NIV and CPAP were compared over a median follow-up of 5.4 years [43]. No differences were observed in the number of hospitalisation days per year (primary outcome), physiological outcomes (daytime PaCO₂, PaO₂, pH, or bicarbonate), weight loss, daytime somnolence, treatment adherence, incidence of cardiovascular events, or survival.

The Pickwick Project included only patients with severe OSA (AHI >30 events/hour), based on the hypothesis that those with more frequent obstructive nocturnal events would be more likely to respond to CPAP [42]. Therefore, the results for the efficacy of CPAP in this trial are not generalizable to all ORRF patients. Masa et al. have also evaluated ORRF patients without severe OSA (AHI<30 events/hour), comparing NIV to lifestyle modifications, and observed a significant reduction in daytime hypercapnia, serum bicarbonate, daytime somnolence, and HRQoL in those receiving NIV [44].

It is thus crucial to evaluate the patient’s phenotype to guide the selection of positive airway pressure therapy. The aim of the therapy is to effectively control sleep-disordered breathing using an intervention that can be supported by local services and that the patient can adhere to. Patients with predominant OSA and normal or mildly elevated PaCO₂ (<7 kPa, 53 mmHg) may be considered for CPAP, and those with hypoventilation and severe OSA and those with hypoventilation and mild or moderate OSA should be initiated on NIV as first-line therapy [45]. Consideration should also be given to device expense and availability, staff training and expertise, and setup facilities, with CPAP devices being less costly than NIV and can readily be setup in the outpatient department [45, 46].

4.3. Neuromuscular and Restrictive Thoracic Disease

4.3.1. Pathophysiology of Respiratory Failure in Neuromuscular and Restrictive Thoracic Disease

Chronic respiratory failure develops in a range of hereditary and acquired neuromuscular disorders, which may be classified into slowly or rapidly progressive, due to respiratory muscle weakness. Understanding the rate of disease progression is important when determining the need for and timing of initiation of NIV. Duration of daily ventilator usage following HMV setup, rather than ventilation intensity, is key to optimising gas exchange in this cohort [47]. The most common rapidly progressive NMD is amyotrophic lateral sclerosis (ALS). Respiratory muscle involvement is typical and inevitably leads to respiratory failure, which is the leading cause of death [48]. Timely NIV initiation following the development of respiratory muscle weakness can improve quality of life and increase survival in patients without significant bulbar disease [49]. Patients with NMD may experience difficulty in effectively clearing respiratory secretions, which can lead to recurrent infection. Following evaluation of cough strength, for example, with peak cough flow [50], manual techniques including breath stacking and manually assisted cough and mechanical insufflation-exsufflation (“cough assist”) may be implemented [51]. Slowly progressive NMD includes postpolio syndrome, spinal muscular atrophy, myotonic dystrophy, and muscular dystrophies (including Duchenne muscular dystrophy and limb-girdle muscular dystrophy) [52]. Patients with these disorders are at variable risk of respiratory failure.

Restrictive thoracic disorders (RTDs) include pathologies which affect the architecture of the spine, sternum, or ribs. This includes congenital or acquired chest wall deformity and severe kyphoscoliosis. Ventilatory failure in RTD occurs due to decreased compliance of the chest wall, leading to a reduction in functional residual capacity. As a result, there is a compensatory increase in the work of breathing to achieve the same tidal volume, which can lead to hypoventilation.

4.3.2. HMV in Neuromuscular Disease

Patients should ideally be referred to a ventilation unit prior to the development of symptoms or signs of respiratory muscle weakness to enable close monitoring and timely initiation of HMV. Symptoms of respiratory muscle weakness include breathlessness, orthopnea, recurrent respiratory infection, sleep disturbance with unrefreshing sleep, daytime somnolence and fatigue, and persistent morning headache. Respiratory muscle surveillance can be performed by measuring vital capacity (VC), sniff nasal inspiratory pressure (SNIP), maximal inspiratory and expiratory pressures (MIP and MEP), peak cough flow (PCF), and overnight oximetry or capnography to detect nocturnal hypoventilation (Table 1) [50, 53, 54]. The aims of NIV in this cohort are to improve survival, symptoms, and HRQoL [55].

Table 1. Objective assessment of respiratory muscle weakness [50, 53, 54, 56].

Indicator of respiratory muscle weakness
Peripheral oxygen saturation	SpO₂ <94% OR SpO₂ <92% (if known lung disease) OR subjective symptoms (especially orthopnea) If any present, urgent evaluation with arterial blood gas required
Arterial blood gas	PaCO₂ >6 kPa (45 mmHg), urgent referral to ventilation centre PaCO₂ ≤6 kPa (45 mmHg) with subjective symptoms (especially orthopnea), refer to ventilation centre for evaluation with sleep study and respiratory muscle testing

Respiratory muscle weakness indicating the need for NIV initiation

FVC or VC	FVC or VC <50% predicted OR FVC or VC <80% predicted with subjective symptoms (especially orthopnea) and a fall of >10% between seated and supine
SNIP	SNIP <40 cm H₂O OR SNIP <65 cm H₂O in males, or <55 cm H₂O in females plus subjective symptoms (especially orthopnoea) OR decrease by >10 cm H₂O over 3 months
PCF	PCF <160 L/min

FVC, forced vital capacity; PaCO₂, arterial partial pressure of carbon dioxide; PCF, peak cough flow; SNIP, sniff nasal inspiratory pressure; SpO₂, peripheral oxygen saturation; VC, vital capacity.

It is challenging to perform prospective clinical trials by recruiting patients with rare and/or rapidly progressive disease; therefore, data on optimal timing of HMV setup and associated prospective data on short-, medium-, and long-term outcomes are limited. Current clinical practice is therefore largely guided by case series, observational studies, and consensus expert opinion. Current guidelines recommend that NIV should be initiated in patients with NMD presenting with symptoms of sleep-disordered breathing and either daytime hypercapnia (PaCO₂ >6 kPa, 45 mmHg), respiratory muscle weakness, or evidence of sleep-disordered breathing on overnight oximetry [57]. This practice is supported by data from Ward et al., who aimed to evaluate whether nocturnal hypercapnia in the presence of daytime normocapnia was a valid indication for NIV [58]. Subjects included those with congenital NMD and chest wall disease with a VC of less than 50% predicted, or symptoms suggestive of nocturnal hypoventilation. Subjects were daytime eucapnic and overnight transcutaneous CO2 monitoring confirmed nocturnal hypercapnia (peak transcutaneous CO₂ > 6.5 kPa, 49 mmHg). At 12 months, 70% of patients in the control group developed ventilatory failure requiring NIV.

The landmark trial of home NIV in ALS was undertaken by Bourke et al.. Subjects with symptomatic daytime hypercapnia or orthopnea with MIP of <60% predicted were randomised to receive usual care or additional home NIV [49]. Symptoms, HRQoL, and respiratory muscle tests were monitored for up to 3 years. In the NIV group, quality of life remained above 75% baseline for the majority of the study, with improvements mainly related to sleep quality. Survival benefits were observed in the NIV group compared to standard care amongst those with normal or moderate bulbar dysfunction (median survival 216 days in the NIV group compared to 11 days in the control group). Whilst this survival benefit was not conferred in those with severe bulbar dysfunction, comparable improvements in HRQoL were suggested potential benefits of NIV in this cohort despite continual physical decline.

It has also been shown that NIV confers survival and HRQoL advantages amongst patients with other types of NMD. A small observational study undertaken by Simonds et al. evaluated the survival benefit of NIV in patients with Duchenne muscular dystrophy (DMD) [59]. Subjects were established on HMV based on symptomatic/proven daytime hypercapnia (PaCO₂ >7 kPa, 53 mmHg) and confirmed nocturnal hypercapnia. Of the subjects established on NIV, 73% remained alive at 5 years. Despite functional limitations, HRQoL was comparable to that of age-matched male controls with regard to mental health and social factors.

Based on a range of evidence sources, including observational, RCT, and consensus expert guidance, patients with NMD should be referred for assessment at a specialist ventilation unit early in their disease trajectory to evaluate their risk of developing respiratory failure. Patients with symptoms and objective evidence of respiratory muscle weakness and/or sleep-disordered breathing should be established on HMV, and in certain cohorts, this may improve survival and HRQoL.

4.3.3. HMV in Restrictive Thoracic Disease

Respiratory failure is the leading cause of death in patients with RTD [60]. Initiation of HMV in this cohort is largely guided by clinical expertise and small clinical trials’ datasets. Owing to the relatively small patient cohort, the evidence for NIV is largely a combination of retrospective and prospective observational and uncontrolled clinical trials, which indicate that home NIV improves physiological and clinical outcomes, including gas exchange, HRQoL, and risk of acute hospitalisation [61, 62].

4.4. Location of HMV Setup

Conventionally, HMV is initiated during an inpatient hospital admission. As increasing numbers of patients are being established on HMV following recent clinical trials and increasing numbers of regional ventilation units and expertise, outpatient or home setups have been evaluated to explore clinically- and cost-effective alternatives to inpatient initiation.

In patients with COPD, Duiverman et al. conducted an RCT which demonstrated noninferiority of home versus hospital initiation for patients with stable hypercapnic respiratory failure [63]. This trial showed comparable exacerbation and hospitalisation rates between home and hospital setups, as well as a significant reduction in cost associated with home setup. This trial points towards home initiation as a noninferior, cost-effective option for NIV initiation in the COPD cohort.

The recent OPIP trial explored the safety and cost-effectiveness of outpatient HMV setup in stable patients with ORRF [64]. Murphy et al. identified no significant difference in medium-term cost-effectiveness between outpatient and inpatient setup, and comparable PaCO₂ control between both the groups. Whilst long-term outcomes remain under investigation, current evidence indicates that HMV setup should be guided by local team resources. Irrespective of setup location, all patients should be reviewed on a regular basis to monitor adherence, disease control, and interface fit (particularly relevant in the context of intentional weight loss) and offer ongoing lifestyle modifications, including dietetic support and referral to bariatric surgical services where appropriate and available.

Patients with NMD may present with advanced disease with marked disability, and as such may prefer to minimise time spent in hospital to reduce the associated emotional burden and risk of nosocomial infection [65]. Hazenberg et al. evaluated home NIV initiation in patients with NMD or chest wall deformity and identified noninferiority of a home compared to in-hospital setup regarding PaCO₂ reduction and HRQoL, as well as significant cost saving in favour of home setup.

4.5. Monitoring HMV Users

4.5.1. Clinical Evaluation

Patients established on home NIV are monitored on an outpatient basis at a specialist ventilation unit, typically every 3–6 months depending on local protocols. Some services may offer telephone or video consultations in combination with home visits for patients with advanced disease, for whom transport may be challenging. Enquiry is made into the aforementioned symptoms of chronic respiratory failure and sleep-disordered breathing, unscheduled healthcare utilization, including treatment of respiratory infections or exacerbation of airways’ disease, and hospital admissions.

4.5.2. Software Analysis and Telemonitoring

Most newer devices can be setup to continuously record a wide range of technical parameters and store data in a cloud-based system to enable remote monitoring. Older devices store data on SD cards, which can be downloaded at outpatient appointments [66]. These data can supplement patients’ descriptions of adherence and interface fit, as well as optimise ventilator settings using data on tidal volume for pressure-targeted ventilation and mean and 90^th percentile delivered pressures for volume-targeted ventilation. Disease control for patients with sleep-disordered breathing can be evaluated using the residual AHI. These data can thus be used as a form of telemonitoring, which yields potential opportunities to improve compliance and reduce hospitalisations and exacerbations [67, 68], and may represent cost-saving opportunities for health services [69]. These data may also be used to predict respiratory deterioration, as highlighted by Blouet et al. who observed an increased respiratory rate in the days preceding an exacerbation in HMV users with COPD [70].

4.5.3. NIV Troubleshooting

Patients may report noisy air leaks, eye dryness or infection, or pressure-related skin damage, which must prompt an interface review. Physical examination for erythema or pressure sores should routinely be undertaken and encouraged by patients at home. Mask cushions or pressure relieving dressings and warning against overtightening straps can help reduce the risk of developing pressure sores [71]. A range of interface designs and sizes are available and should be selected to suit patients’ face shape and comfort. A nasal or minimal contact interface can be used in instances where patients are claustrophobic [72]. A humidifier may relieve discomfort caused by upper airway dryness. A ramp, whereby pressures are increased over a preset time, can be added for comfort. Accurate assessment of leak is also important for device optimisation; significant leak can result in inaccurate tidal volume estimation and patient-ventilator asynchrony, ultimately impacting on gas exchange and patient tolerance [73]. Inpatient review with overnight evaluation may be offered if troubleshooting cannot rectify issues in the outpatient setting.

4.5.4. Patient-Reported Outcomes

Breathlessness is common in patients with chronic respiratory failure and can be distressing and disabling [74]. Patient-reported outcome (PRO) tools can help with symptom reporting, and these may be general or disease-specific (Table 2) [75]. Psychosocial aspects including the effects of treatment burden especially in patients with multimorbidity can impact adherence [76]. Patients who are younger, have a mental health diagnosis, and are from socioeconomically deprived backgrounds may need additional support to promote adequate HMV compliance [77].

Table 2. Examples of available generic and disease-specific questionnaires.

Questionnaire	Type	Description
Modified Borg Scale [78]	Generic	Quantifies breathlessness intensity at the current point in time. Scored from 0 to 10
Medical Research Council (MRC) Dyspnoea Scale [79]	Generic	Categorises functional limitation. Grades 1 to 5
Severe Respiratory Insufficiency Questionnaire (SRI) [80]	Disease-specific: Respiratory failure	49 items covering respiratory complaints, physical functioning, attendant symptoms and sleep, social relationships, anxiety, psychological well-being, and social functioning. Scored from 1 to 5 for each item
The Maugeri Foundation Respiratory Failure Questionnaire (MF-28) [81]	Disease-specific: Respiratory failure	28-item questionnaire assessing 3 domains: Daily activities, cognitive function, and invalidity Scores 1 for positive responses
S³-NIV Questionnaire	Disease-specific: Respiratory failure	11 items assessing symptoms related to chronic respiratory failure and HMV use. Scored from 0 to 4 on each item with the average score multiplied by 2.5
COPD Assessment Test (CAT) [82]	Disease-specific: COPD	8 items covering symptoms, sleep disturbance, and disease impact on daily activities. Scored from 0 to 5
Individualised Neuromuscular Quality of Life Questionnaire (INQoL) [83]	Disease-specific: NMD	45 items covering muscle disease symptoms, impact on areas of life, and positive and negative effects of treatment
Sleep Apnea Quality of Life Index (SAQLI) [84]	Disease-specific: Sleep-disordered breathing	35 items assessing daily functioning, social interactions, emotional functioning, and symptoms Scaled scoring system
Epworth Sleep Score (ESS) [85]	Disease-specific: Sleep-disordered breathing	8 items requiring participants to rate the likelihood of sleeping during activity. Scored from 0 to 24

COPD, chronic obstructive pulmonary disease; HMV, home mechanical ventilation; NMD, neuromuscular disease.

4.5.5. Physiological Measurements

PaCO₂ measurement is required to diagnose chronic respiratory failure. For patients who are established on NIV, physiological response should be regularly monitored. This may be achieved using arterial, capillary, or transcutaneous sampling. Transcutaneous monitoring involves a sensor warmed to a temperature of 40–44°C to cause local arterialisation, which when applied with an algorithm, provides measures of CO₂, SpO₂, and heart rate. Transcutaneous CO₂ (TcCO₂) values have been shown to correspond with PaCO₂ within a clinically acceptable range [86]. Whilst it should not be used to diagnose respiratory failure, TcCO₂ has a valuable role in monitoring following HMV setup, can be performed in the outpatient and home settings, and can limit the need for painful arterial sampling [63].

Home overnight oximetry measurements can be used to evaluate mean SpO₂ and time-spent SpO₂ below 90% (T<90%), with acceptable thresholds based on individuals’ pathophysiology, and the SpO₂ trace is used to identify rapid deep desaturations (indicative of upper airway obstruction) or periods of prolonged desaturation (indicative of hypoventilation) [87]. Persistently abnormal findings despite HMV adherence should prompt a clinical review and reevaluation of device settings and interface.

The aforementioned tests of respiratory muscle strength are used to diagnose and monitor patients with neuromuscular disorders (Table 1). Measurement of PCF is valuable in the assessment of expiratory muscle strength and effective secretion clearance; a reduced value may predispose to chest infections and increased mortality risk [88]. Values of >250–270 L/min have been proposed as a threshold for risk of recurrent chest infections and for consideration of a daily secretion management schedule including manual techniques and mechanical insufflation/exsufflation [89].

4.5.6. Physiological Techniques under Evaluation

Patients with chronic respiratory failure are at high risk of respiratory infection, hospitalisation, and death. Advanced physiological markers that can predict respiratory deterioration could facilitate timely clinical review and treatment initiation, and thus prevent hospitalisation. Emerging techniques are as follows:

(1)
Parasternal electromyography (EMG_para): Neural respiratory drive (NRD) cannot be measured directly in living subjects; therefore, surrogate measures are required. Respiratory muscle electromyography can be used, with both invasive and surface techniques applied in patients with airways’ disease and obesity [90, 91]. During severe COPD exacerbations, EMG_para is closely associated with patient-reported and physician-defined clinical deterioration and can be used to predict COPD patients who are safe for discharge and at lower risk of early hospital readmission [92, 93]. Overall, EMG_para is a feasible and acceptable home-based technique, which tracks patient-reported and physiological recovery from severe COPD exacerbation [94].
(2)
Forced oscillation technique (FOT): It involves the application of small-amplitude sonic oscillations during tidal breathing via a mouthpiece to quantify respiratory system reactance and resistance [95]. Application in the home setting has shown it to be an acceptable measurement for COPD patients, with good daily adherence, as observed by Walker et al. [96]. It may also be used to identify expiratory flow limitation in COPD and optimise HMV pressure to abolish it by using a novel auto-titrating ventilatory mode [7]. The clinical utility of FOT in optimising gas exchange, predicting respiratory events, and reducing risk of hospitalisation remains under investigation.
(3)
Physical activity monitoring: It is a simple and noninvasive technique that can be achieved by using commercially available or advanced accelerometer devices typically worn on the nondominant wrist. Such devices can quantify physical movement and actigraphy indices can be used to evaluate sleep quality in the home setting. Physical activity monitoring can identify changes during periods of acute illness and has been used extensively in clinical trials of COPD patients [97–99]. Further data are needed to evaluate the long-term effect of HMV on functional limitation in patients with chronic respiratory failure.

5. Conclusions

Hypercapnic respiratory failure is a complication of commonly encountered diseases and arises due to an imbalance in the loads and capacity of the respiratory muscle pump. Clinicians should be aware of indications to refer patients to a specialist ventilation centre for assessment and consideration of home noninvasive ventilation, considering risk factors, symptoms, signs, and physiological evaluation. Robust clinical trials demonstrate that home NIV can improve clinical, patient-reported, and physiological outcomes in selected patients with COPD, obesity-related respiratory failure, and neuromuscular disease. Technological advances have seen developments in novel ventilator modes, interfaces to optimise comfort, and remote monitoring of detailed technical parameters, all of which serve to support clinicians in delivering personalised, patient-centred care.

Conflicts of Interest

RFD has received consulting fees from Astra Zeneca, ResMed, Aerogen, and Fisher and Paykel. LT, PN, and EG declare that they have no conflicts of interest.

Authors’ Contributions

LT, PN, EG, and RFD contributed to writing (original draft, review, and editing) of this manuscript. All authors agree to be accountable for the content and conclusions of the article.

References

1 D’Cruz R. F. and Hart N., Mechanisms of hypoxaemia and hypercapnia, ERS Practical Handbook of Invasive Mechanical Ventilation, 2019, 1–7, European Respiratory Society, Lausanne, Switzerland, 1–7, https://doi.org/10.1183/9781849841221.017919.
10.1183/9781849841221.017919
Google Scholar
2 Jonkman A. H., de Vries H. J., and Heunks L. M. A., Physiology of the respiratory drive in ICU patients: implications for diagnosis and treatment, Critical Care. (2020) 24, no. 1, https://doi.org/10.1186/s13054-020-2776-z.
10.1186/s13054-020-2776-z
Web of Science® Google Scholar
3 Adler D. and Janssens J. P., The pathophysiology of respiratory failure: control of breathing, respiratory load, and muscle capacity, Respiration. (2019) 97, no. 2, 93–104, https://doi.org/10.1159/000494063, 2-s2.0-85057021592.
10.1159/000494063
PubMed Web of Science® Google Scholar
4 Moxham J. and Jolley C., Breathlessness, fatigue and the respiratory muscles, Clinical Medicine. (2009) 9, no. 5, 448–452, https://doi.org/10.7861/clinmedicine.9-5-448, 2-s2.0-72849149134.
10.7861/clinmedicine.9-5-448
PubMed Web of Science® Google Scholar
5 Zerah F., Harf A., Perlemuter L., Lorino H., Lorino A. M., and Atlan G., Effects of obesity on respiratory resistance, Chest. (1993) 103, no. 5, 1470–1476, https://doi.org/10.1378/chest.103.5.1470, 2-s2.0-0027295109.
10.1378/chest.103.5.1470
CAS PubMed Web of Science® Google Scholar
6 Jolley C. J. and Moxham J., A physiological model of patient-reported breathlessness during daily activities in COPD, European Respiratory Review. (2009) 18, no. 112, 66–79, https://doi.org/10.1183/09059180.00000809, 2-s2.0-66549120040.
10.1183/09059180.00000809
CAS PubMed Google Scholar
7 Suh E. S., Pompilio P., Mandal S., Hill P., Kaltsakas G., Murphy P. B., Romano R., Moxham J., Dellaca R., and Hart N., Autotitrating external positive end-expiratory airway pressure to abolish expiratory flow limitation during tidal breathing in patients with severe COPD: a physiological study, European Respiratory Journal. (2020) 56, no. 3, https://doi.org/10.1183/13993003.02234-2019.
10.1183/13993003.02234-2019
PubMed Web of Science® Google Scholar
8 Murphy P. B., Davidson C., Hind M. D., Simonds A., Williams A. J., Hopkinson N. S., Moxham J., Polkey M., and Hart N., Volume targeted versus pressure support non-invasive ventilation in patients with super obesity and chronic respiratory failure: a randomised controlled trial, Thorax. (2012) 67, no. 8, 727–734, https://doi.org/10.1136/thoraxjnl-2011-201081, 2-s2.0-84863992648.
10.1136/thoraxjnl-2011-201081
PubMed Web of Science® Google Scholar
9 Crisafulli E., Manni G., Kidonias M., Trianni L., and Clini E. M., Subjective sleep quality during average volume assured pressure support (AVAPS) ventilation in patients with hypercapnic COPD: a physiological pilot study, Lung. (2009) 187, no. 5, 299–305, https://doi.org/10.1007/s00408-009-9167-1, 2-s2.0-70349243091.
10.1007/s00408-009-9167-1
PubMed Web of Science® Google Scholar
10 Nilius G., Katamadze N., Domanski U., Schroeder M., and Franke K. J., Non-invasive ventilation with intelligent volume-assured pressure support versus pressure-controlled ventilation: effects on the respiratory event rate and sleep quality in COPD with chronic hypercapnia, International Journal of Chronic Obstructive Pulmonary Disease. (2017) 12, 1039–1045, https://doi.org/10.2147/copd.s126970, 2-s2.0-85017296678.
10.2147/COPD.S126970
PubMed Web of Science® Google Scholar
11 McArdle N., Rea C., King S., Maddison K., Ramanan D., Ketheeswaran S., Erikli L., Baker V., Armitstead J., Richards G., Singh B., Hillman D., and Eastwood P., Treating chronic hypoventilation with automatic adjustable versus fixed EPAP intelligent volume-assured positive airway pressure support (iVAPS): a randomized controlled trial, Sleep. (2017) 40, no. 10, https://doi.org/10.1093/sleep/zsx136, 2-s2.0-85043250598.
10.1093/sleep/zsx136
PubMed Web of Science® Google Scholar
12 Lebret M., Leotard A., Pepin J. L., Windisch W., Ekkernkamp E., Pallero M., Sanchez-Quiroga M. A., Hart N., Kelly J. L., Patout M., Funk G. C., Duiverman M. L., Masa J. F., Simonds A., Murphy P. B., Wijkstra P. J., Dreher M., Storre J., Khouri C., and Borel J. C., Nasal versus oronasal masks for home non-invasive ventilation in patients with chronic hypercapnia: a systematic review and individual participant data meta-analysis, Thorax. (2021) 76, no. 11, 1108–1116, https://doi.org/10.1136/thoraxjnl-2020-215613.
10.1136/thoraxjnl-2020-215613
PubMed Web of Science® Google Scholar
13 GBD Diseases and Injuries Collaborators, Global burden of 369 diseases and injuries in 204 countries and territories, 1990-2019: a systematic analysis for the Global Burden of Disease Study 2019, The Lancet. (2020) 396, no. 10258, 1204–1222, https://doi.org/10.1016/s0140-6736(20)30925-9.
10.1016/S0140-6736(20)30925-9
PubMed Web of Science® Google Scholar
14 O′Donnell D. E. and Parker C. M., COPD exacerbations. 3: Pathophysiology, Thorax. (2006) 61, no. 4, 354–361, https://doi.org/10.1136/thx.2005.041830, 2-s2.0-33645573320.
10.1136/thx.2005.041830
CAS PubMed Web of Science® Google Scholar
15 Dreher M., Neuzeret P. C., Windisch W., Martens D., Hoheisel G., Groschel A., Woehrle H., Fetsch T., Graml A., and Kohnlein T., Prevalence of chronic hypercapnia in severe chronic obstructive pulmonary disease: data from the HOmeVent registry, International Journal of Chronic Obstructive Pulmonary Disease. (2019) 14, 2377–2384, https://doi.org/10.2147/copd.s222803.
10.2147/COPD.S222803
PubMed Web of Science® Google Scholar
16 Held M., Walthelm J., Baron S., Roth C., and Jany B., Functional impact of pulmonary hypertension due to hypoventilation and changes under noninvasive ventilation, European Respiratory Journal. (2014) 43, no. 1, 156–165, https://doi.org/10.1183/09031936.00147712, 2-s2.0-84891938301.
10.1183/09031936.00147712
PubMed Web of Science® Google Scholar
17 Balnis J., Korponay T. C., and Jaitovich A., AMP-activated protein kinase (AMPK) at the crossroads between CO(2) retention and skeletal muscle dysfunction in chronic obstructive pulmonary disease (COPD), International Journal of Molecular Sciences. (2020) 21, no. 3, https://doi.org/10.3390/ijms21030955.
10.3390/ijms21030955
PubMed Web of Science® Google Scholar
18 Yang H., Xiang P., Zhang E., Guo W., Shi Y., Zhang S., and Tong Z., Is hypercapnia associated with poor prognosis in chronic obstructive pulmonary disease? A long-term follow-up cohort study, BMJ Open. (2015) 5, no. 12, e008909, https://doi.org/10.1136/bmjopen-2015-008909, 2-s2.0-84960360205.
10.1136/bmjopen-2015-008909
PubMed Web of Science® Google Scholar
19 Kohnlein T., Windisch W., Kohler D., Drabik A., Geiseler J., Hartl S., Karg O., Laier-Groeneveld G., Nava S., Schonhofer B., Schucher B., Wegscheider K., Criee C. P., and Welte T., Non-invasive positive pressure ventilation for the treatment of severe stable chronic obstructive pulmonary disease: a prospective, multicentre, randomised, controlled clinical trial, The Lancet Respiratory Medicine. (2014) 2, no. 9, 698–705, https://doi.org/10.1016/s2213-2600(14)70153-5, 2-s2.0-84908047257.
10.1016/S2213-2600(14)70153-5
PubMed Web of Science® Google Scholar
20 Murphy P. B., Rehal S., Arbane G., Bourke S., Calverley P. M. A., Crook A. M., Dowson L., Duffy N., Gibson G. J., Hughes P. D., Hurst J. R., Lewis K. E., Mukherjee R., Nickol A., Oscroft N., Patout M., Pepperell J., Smith I., Stradling J. R., Wedzicha J. A., Polkey M. I., Elliott M. W., and Hart N., Effect of home noninvasive ventilation with oxygen therapy vs oxygen therapy alone on hospital readmission or death after an acute COPD exacerbation: A randomized clinical trial, JAMA. (2017) 317, no. 21, 2177–2186, https://doi.org/10.1001/jama.2017.4451, 2-s2.0-85020387471.
10.1001/jama.2017.4451
PubMed Web of Science® Google Scholar
21 Ergan B., Oczkowski S., Rochwerg B., Carlucci A., Chatwin M., Clini E., Elliott M., Gonzalez-Bermejo J., Hart N., Lujan M., Nasilowski J., Nava S., Pepin J. L., Pisani L., Storre J. H., Wijkstra P., Tonia T., Boyd J., Scala R., and Windisch W., European Respiratory Society guidelines on long-term home non-invasive ventilation for management of COPD, European Respiratory Journal. (2019) 54, no. 3, 1901003, https://doi.org/10.1183/13993003.01003-2019, 2-s2.0-85072746961.
10.1183/13993003.01003-2019
PubMed Web of Science® Google Scholar
22 Casanova C., Celli B. R., Tost L., Soriano E., Abreu J., Velasco V., and Santolaria F., Long-term controlled trial of nocturnal nasal positive pressure ventilation in patients with severe COPD, Chest. (2000) 118, no. 6, 1582–1590, https://doi.org/10.1378/chest.118.6.1582, 2-s2.0-0034548358.
10.1378/chest.118.6.1582
CAS PubMed Web of Science® Google Scholar
23 Clini E., Sturani C., Rossi A., Viaggi S., Corrado A., Donner C. F., Ambrosino N., and Rehabilitation Chronic Care Study Group IaoHP, The Italian multicentre study on noninvasive ventilation in chronic obstructive pulmonary disease patients, European Respiratory Journal. (2002) 20, no. 3, 529–538, https://doi.org/10.1183/09031936.02.02162001, 2-s2.0-18544366064.
10.1183/09031936.02.02162001
CAS PubMed Web of Science® Google Scholar
24 McEvoy R. D., Pierce R. J., Hillman D., Esterman A., Ellis E. E., Catcheside P. G., O’Donoghue F. J., Barnes D. J., Grunstein R. R., and Australian trial of non-invasive Ventilation in Chronic Airflow Limitation Study G, Nocturnal non-invasive nasal ventilation in stable hypercapnic COPD: a randomised controlled trial, Thorax. (2009) 64, no. 7, 561–566, https://doi.org/10.1136/thx.2008.108274, 2-s2.0-67650245610.
10.1136/thx.2008.108274
CAS PubMed Web of Science® Google Scholar
25 Struik F. M., Sprooten R. T., Kerstjens H. A., Bladder G., Zijnen M., Asin J., Cobben N. A., Vonk J. M., and Wijkstra P. J., Nocturnal non-invasive ventilation in COPD patients with prolonged hypercapnia after ventilatory support for acute respiratory failure: a randomised, controlled, parallel-group study, Thorax. (2014) 69, no. 9, 826–834, https://doi.org/10.1136/thoraxjnl-2014-205126, 2-s2.0-84905975996.
10.1136/thoraxjnl-2014-205126
CAS PubMed Web of Science® Google Scholar
26 American Academy of Sleep Medicine Task Force, Sleep-related breathing disorders in adults: recommendations for syndrome definition and measurement techniques in clinical research. The Report of an American Academy of Sleep Medicine Task Force, Sleep. (1999) 22, 667–689.
10.1093/sleep/22.5.667
PubMed Web of Science® Google Scholar
27 Mokhlesi B., Obesity hypoventilation syndrome: a state-of-the-art review, Respiratory Care. (2010) 55, no. 10, 1347–1365.
PubMed Web of Science® Google Scholar
28 Nowbar S., Burkart K. M., Gonzales R., Fedorowicz A., Gozansky W. S., Gaudio J. C., Taylor M. R., and Zwillich C. W., Obesity-associated hypoventilation in hospitalized patients: prevalence, effects, and outcome, The American Journal of Medicine. (2004) 116, 1–7, https://doi.org/10.1016/j.amjmed.2003.08.022, 2-s2.0-0346969989.
10.1016/j.amjmed.2003.08.022
PubMed Web of Science® Google Scholar
29 Macavei V. M., Spurling K. J., Loft J., and Makker H. K., Diagnostic predictors of obesity-hypoventilation syndrome in patients suspected of having sleep disordered breathing, Journal of Clinical Sleep Medicine. (2013) 9, no. 9, 879–884, https://doi.org/10.5664/jcsm.2986, 2-s2.0-84888148627.
10.5664/jcsm.2986
PubMed Web of Science® Google Scholar
30 Masa J. F., Pepin J. L., Borel J. C., Mokhlesi B., Murphy P. B., and Sanchez-Quiroga M. A., Obesity hypoventilation syndrome, European Respiratory Review. (2019) 28, no. 151, 180097, https://doi.org/10.1183/16000617.0097-2018, 2-s2.0-85062962527.
10.1183/16000617.0097-2018
PubMed Web of Science® Google Scholar
31 Mokhlesi B., Masa J. F., Brozek J. L., Gurubhagavatula I., Murphy P. B., Piper A. J., Tulaimat A., Afshar M., Balachandran J. S., Dweik R. A., Grunstein R. R., Hart N., Kaw R., Lorenzi-Filho G., Pamidi S., Patel B. K., Patil S. P., Pepin J. L., Soghier I., Tamae Kakazu M., and Teodorescu M., Evaluation and management of obesity hypoventilation syndrome. An official American thoracic society clinical practice guideline, American Journal of Respiratory and Critical Care Medicine. (2019) 200, no. 3, e6–e24, https://doi.org/10.1164/rccm.201905-1071st, 2-s2.0-85070877856.
10.1164/rccm.201905-1071ST
PubMed Web of Science® Google Scholar
32 Pankow W., Podszus T., Gutheil T., Penzel T., Peter J., and Von Wichert P., Expiratory flow limitation and intrinsic positive end-expiratory pressure in obesity, Journal of Applied Physiology. (1998) 85, no. 4, 1236–1243, https://doi.org/10.1152/jappl.1998.85.4.1236.
10.1152/jappl.1998.85.4.1236
CAS PubMed Web of Science® Google Scholar
33 Piper A. J. and Grunstein R. R., Obesity hypoventilation syndrome: Mechanisms and management, American Journal of Respiratory and Critical Care Medicine. (2011) 183, no. 3, 292–298, https://doi.org/10.1164/rccm.201008-1280ci, 2-s2.0-79551519254.
10.1164/rccm.201008-1280CI
PubMed Web of Science® Google Scholar
34 Sakaguchi Y., Hatta T., Hayashi T., Shoji T., Suzuki A., Tomida K., Okada N., Rakugi H., Isaka Y., and Tsubakihara Y., Association of nocturnal hypoxemia with progression of CKD, Clinical Journal of the American Society of Nephrology. (2013) 8, no. 9, 1502–1507, https://doi.org/10.2215/cjn.11931112, 2-s2.0-84883661787.
10.2215/CJN.11931112
CAS PubMed Web of Science® Google Scholar
35 White L. H. and Bradley T. D., Role of nocturnal rostral fluid shift in the pathogenesis of obstructive and central sleep apnoea, The Journal of Physiology. (2013) 591, no. 5, 1179–1193, https://doi.org/10.1113/jphysiol.2012.245159, 2-s2.0-84874404262.
10.1113/jphysiol.2012.245159
PubMed Web of Science® Google Scholar
36 National Institute for Health and Care Excellence, Obstructive Sleep Apnoea/hypopnoea Syndrome and Obesity Hypoventilation Syndrome in over 16s, 2021, Nice, London, UK.
Google Scholar
37 Kakazu M. T., Soghier I., Afshar M., Brozek J. L., Wilson K. C., Masa J. F., and Mokhlesi B., Weight loss interventions as treatment of obesity hypoventilation syndrome. A systematic review, Annals of the American Thoracic Society. (2020) 17, no. 4, 492–502, https://doi.org/10.1513/annalsats.201907-554oc.
10.1513/AnnalsATS.201907-554OC
PubMed Web of Science® Google Scholar
38 Cooper T. C., Simmons E. B., Webb K., Burns J. L., and Kushner R. F., Trends in weight regain following Roux-en-Y gastric bypass (RYGB) bariatric surgery, Obesity Surgery. (2015) 25, no. 8, 1474–1481, https://doi.org/10.1007/s11695-014-1560-z, 2-s2.0-84939418077.
10.1007/s11695-014-1560-z
PubMed Web of Science® Google Scholar
39 Marti-Valeri C., Sabate A., Masdevall C., and Dalmau A., Improvement of associated respiratory problems in morbidly obese patients after open Roux-en-Y gastric bypass, Obesity Surgery. (2007) 17, no. 8, 1102–1110, https://doi.org/10.1007/s11695-007-9186-z, 2-s2.0-35748967296.
10.1007/s11695-007-9186-z
CAS PubMed Web of Science® Google Scholar
40 Piper A. J., Wang D., Yee B. J., Barnes D. J., and Grunstein R. R., Randomised trial of CPAP vs bilevel support in the treatment of obesity hypoventilation syndrome without severe nocturnal desaturation, Thorax. (2008) 63, no. 5, 395–401, https://doi.org/10.1136/thx.2007.081315, 2-s2.0-43749095671.
10.1136/thx.2007.081315
CAS PubMed Web of Science® Google Scholar
41 Howard M. E., Piper A. J., Stevens B., Holland A. E., Yee B. J., Dabscheck E., Mortimer D., Burge A. T., Flunt D., Buchan C., Rautela L., Sheers N., Hillman D., and Berlowitz D. J., A randomised controlled trial of CPAP versus non-invasive ventilation for initial treatment of obesity hypoventilation syndrome, Thorax. (2017) 72, no. 5, 437–444, https://doi.org/10.1136/thoraxjnl-2016-208559, 2-s2.0-85019558511.
10.1136/thoraxjnl-2016-208559
PubMed Web of Science® Google Scholar
42 Masa J. F., Corral J., Alonso M. L., Ordax E., Troncoso M. F., Gonzalez M., Lopez-Martinez S., Marin J. M., Marti S., Diaz-Cambriles T., Chiner E., Aizpuru F., Egea C., and Spanish S. N., Efficacy of different treatment alternatives for obesity hypoventilation syndrome. Pickwick study, American Journal of Respiratory and Critical Care Medicine. (2015) 192, no. 1, 86–95, https://doi.org/10.1164/rccm.201410-1900oc, 2-s2.0-84937239225.
10.1164/rccm.201410-1900OC
PubMed Web of Science® Google Scholar
43 Masa J. F., Mokhlesi B., Benitez I., Gomez de Terreros F. J., Sanchez-Quiroga M. A., Romero A., Caballero-Eraso C., Teran-Santos J., Alonso-Alvarez M. L., Troncoso M. F., Gonzalez M., Lopez-Martin S., Marin J. M., Marti S., Diaz-Cambriles T., Chiner E., Egea C., Barca J., Vazquez-Polo F. J., Negrin M. A., Martel-Escobar M., Barbe F., Corral J., Fernández G., Ordax-Carbajo E., González-Mangado N., Gómez-García T., Martínez-Martínez M. Á, Ojeda-Castillejo E., López Padilla D., Carrizo S. J., Gallego B., Pallero M., Romero O., Ramón M. A., Arias E., Muñoz-Méndez J., Senent C., Sancho-Chust J. N., Navarro Soriano N. B., Barrot E., Benítez J. M., Sánchez-Gómez J., Golpe R., Santiago-Recuerda A., Gómez S., and Bengoa M., Long-term clinical effectiveness of continuous positive airway pressure therapy versus non-invasive ventilation therapy in patients with obesity hypoventilation syndrome: A multicentre, open-label, randomised controlled trial, The Lancet. (2019) 393, no. 10182, 1721–1732, https://doi.org/10.1016/s0140-6736(18)32978-7, 2-s2.0-85065508787.
10.1016/S0140-6736(18)32978-7
PubMed Web of Science® Google Scholar
44 Masa J. F., Corral J., Caballero C., Barrot E., Teran-Santos J., Alonso-Alvarez M. L., Gomez-Garcia T., Gonzalez M., Lopez-Martin S., De Lucas P., Marin J. M., Marti S., Diaz-Cambriles T., Chiner E., Egea C., Miranda E., Mokhlesi B., García-Ledesma E., Sánchez-Quiroga M. Á, Ordax E., González-Mangado N., Troncoso M. F., Martinez-Martinez M. Á, Cantalejo O., Ojeda E., Carrizo S. J., Gallego B., Pallero M., Ramón M. A., Díaz-de-Atauri J., Muñoz-Méndez J., Senent C., Sancho-Chust J. N., Ribas-Solís F. J., Romero A., Benítez J. M., Sanchez-Gómez J., Golpe R., Santiago-Recuerda A., Gomez S., Bengoa M., and Bengoa M., Non-invasive ventilation in obesity hypoventilation syndrome without severe obstructive sleep apnoea, Thorax. (2016) 71, no. 10, 899–906, https://doi.org/10.1136/thoraxjnl-2016-208501, 2-s2.0-84978786203.
10.1136/thoraxjnl-2016-208501
PubMed Web of Science® Google Scholar
45 Murphy P. B., Suh E. S., and Hart N., Non-invasive ventilation for obese patients with chronic respiratory failure: are two pressures always better than one?, Respirology. (2019) 24, no. 10, 952–961, https://doi.org/10.1111/resp.13588, 2-s2.0-85066817328.
10.1111/resp.13588
PubMed Web of Science® Google Scholar
46 Masa J. F., Mokhlesi B., Benitez I., Gomez de Terreros Caro F. J., Sanchez-Quiroga M. A., Romero A., Caballero C., Alonso-Alvarez M. L., Ordax-Carbajo E., Gomez-Garcia T., Gonzalez M., Lopez-Martin S., Marin J. M., Marti S., Diaz-Cambriles T., Chiner E., Egea C., Barca J., Vazquez-Polo F. J., Negrin M. A., Martel-Escobar M., Barbe F., Corral-Penafiel J., and Spanish Sleep N., Cost-effectiveness of positive airway pressure modalities in obesity hypoventilation syndrome with severe obstructive sleep apnoea, Thorax. (2020) 75, no. 6, 459–467, https://doi.org/10.1136/thoraxjnl-2019-213622.
10.1136/thoraxjnl-2019-213622
PubMed Web of Science® Google Scholar
47 Delorme M., Leotard A., Lebret M., Lefeuvre C., Hazenberg A., Pallero M., Nickol A. H., Hannan L. M., Boentert M., Yuksel A., Windisch W., Howard M. E., Hart N., Wijkstra P. J., Prigent H., Pepin J. L., Lofaso F., Khouri C., and Borel J. C., 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, Archivos de Bronconeumología. (2023) 59, no. 8, 488–496, https://doi.org/10.1016/j.arbres.2023.05.002.
10.1016/j.arbres.2023.05.002
PubMed Web of Science® Google Scholar
48 Feldman E. L., Goutman S. A., Petri S., Mazzini L., Savelieff M. G., Shaw P. J., and Sobue G., Amyotrophic lateral sclerosis, The Lancet. (2022) 400, no. 10360, 1363–1380, https://doi.org/10.1016/s0140-6736(22)01272-7.
10.1016/S0140-6736(22)01272-7
CAS PubMed Web of Science® Google Scholar
49 Bourke S. C., Tomlinson M., Williams T. L., Bullock R. E., Shaw P. J., and Gibson G. J., Effects of non-invasive ventilation on survival and quality of life in patients with amyotrophic lateral sclerosis: A randomised controlled trial, The Lancet Neurology. (2006) 5, no. 2, 140–147, https://doi.org/10.1016/s1474-4422(05)70326-4, 2-s2.0-30844439854.
10.1016/S1474-4422(05)70326-4
PubMed Web of Science® Google Scholar
50 Bach J. R., Ishikawa Y., and Kim H., Prevention of pulmonary morbidity for patients with Duchenne muscular dystrophy, Chest. (1997) 112, no. 4, 1024–1028, https://doi.org/10.1378/chest.112.4.1024, 2-s2.0-0030793790.
10.1378/chest.112.4.1024
CAS PubMed Web of Science® Google Scholar
51 Morrow B., Argent A., Zampoli M., Human A., Corten L., and Toussaint M., Cough augmentation techniques for people with chronic neuromuscular disorders, Cochrane Database of Systematic Reviews. (2021) 4, no. 4, CD013170, https://doi.org/10.1002/14651858.cd013170.pub2.
10.1002/14651858.cd013170.pub2
PubMed Google Scholar
52 Shneerson J. M. and Simonds A. K., Noninvasive ventilation for chest wall and neuromuscular disorders, European Respiratory Journal. (2002) 20, no. 2, 480–487, https://doi.org/10.1183/09031936.02.00404002, 2-s2.0-0035989843.
10.1183/09031936.02.00404002
CAS PubMed Web of Science® Google Scholar
53 Sancho J., Servera E., Diaz J., and Marin J., Predictors of ineffective cough during a chest infection in patients with stable amyotrophic lateral sclerosis, American Journal of Respiratory and Critical Care Medicine. (2007) 175, no. 12, 1266–1271, https://doi.org/10.1164/rccm.200612-1841oc, 2-s2.0-34250203194.
10.1164/rccm.200612-1841OC
PubMed Web of Science® Google Scholar
54 Kaminska M., Noel F., and Petrof B. J., Optimal method for assessment of respiratory muscle strength in neuromuscular disorders using sniff nasal inspiratory pressure (SNIP), PLoS One. (2017) 12, no. 5, e0177723, https://doi.org/10.1371/journal.pone.0177723, 2-s2.0-85019648948.
10.1371/journal.pone.0177723
PubMed Web of Science® Google Scholar
55 Dorst J. and Ludolph A. C., Non-invasive ventilation in amyotrophic lateral sclerosis, Therapeutic Advances in Neurological Disorders. (2019) 12, 175628641985704, https://doi.org/10.1177/1756286419857040, 2-s2.0-85067789493.
10.1177/1756286419857040
Web of Science® Google Scholar
56 Allen S. M., Hunt B., and Green M., Fall in vital capacity with posture, British Journal of Diseases of the Chest. (1985) 79, 267–271, https://doi.org/10.1016/0007-0971(85)90047-6, 2-s2.0-0021861158.
10.1016/0007-0971(85)90047-6
CAS PubMed Web of Science® Google Scholar
57 National Institute for Health and Care Excellence, Motor Neuron Disease: Assessment and Management, 2016, NICE Guideline, London, UK.
Google Scholar
58 Ward S., Chatwin M., Heather S., and Simonds A. K., Randomised controlled trial of non-invasive ventilation (NIV) for nocturnal hypoventilation in neuromuscular and chest wall disease patients with daytime normocapnia, Thorax. (2005) 60, no. 12, 1019–1024, https://doi.org/10.1136/thx.2004.037424, 2-s2.0-26944453349.
10.1136/thx.2004.037424
CAS PubMed Web of Science® Google Scholar
59 Simonds A. K., Muntoni F., Heather S., and Fielding S., Impact of nasal ventilation on survival in hypercapnic Duchenne muscular dystrophy, Thorax. (1998) 53, no. 11, 949–952, https://doi.org/10.1136/thx.53.11.949, 2-s2.0-0031759227.
10.1136/thx.53.11.949
CAS PubMed Web of Science® Google Scholar
60 Budweiser S., Murbeth R. E., Jorres R. A., Heinemann F., and Pfeifer M., Predictors of long-term survival in patients with restrictive thoracic disorders and chronic respiratory failure undergoing non-invasive home ventilation, Respirology. (2007) 12, no. 4, 551–559, https://doi.org/10.1111/j.1440-1843.2007.01086.x, 2-s2.0-34250785075.
10.1111/j.1440-1843.2007.01086.x
CAS PubMed Web of Science® Google Scholar
61 Budweiser S., Heinemann F., Fischer W., Dobroschke J., Wild P. J., and Pfeifer M., Impact of ventilation parameters and duration of ventilator use on non-invasive home ventilation in restrictive thoracic disorders, Respiration. (2006) 73, no. 4, 488–494, https://doi.org/10.1159/000088712, 2-s2.0-33745222587.
10.1159/000088712
PubMed Web of Science® Google Scholar
62 Domenech-Clar R., Nauffal-Manzur D., Perpina-Tordera M., Compte-Torrero L., and Macian-Gisbert V., Home mechanical ventilation for restrictive thoracic diseases: Effects on patient quality-of-life and hospitalizations, Respiratory Medicine. (2003) 97, no. 12, 1320–1327, https://doi.org/10.1016/j.rmed.2003.08.002, 2-s2.0-0347531908.
10.1016/j.rmed.2003.08.002
PubMed Web of Science® Google Scholar
63 Duiverman M. L., Vonk J. M., Bladder G., van Melle J. P., Nieuwenhuis J., Hazenberg A., Kerstjens H. A. M., van Boven J. F. M., and Wijkstra P. J., Home initiation of chronic non-invasive ventilation in COPD patients with chronic hypercapnic respiratory failure: a randomised controlled trial, Thorax. (2019) 75, no. 3, 244–252, https://doi.org/10.1136/thoraxjnl-2019-213303, 2-s2.0-85072032100.
10.1136/thoraxjnl-2019-213303
PubMed Web of Science® Google Scholar
64 Murphy P. B., Patout M., Arbane G., Mandal S., Kaltsakas G., Polkey M. I., Elliott M., Muir J. F., Douiri A., Parkin D., Janssens J. P., Pepin J. L., Cuvelier A., Flach C., and Hart N., Cost-effectiveness of outpatient versus inpatient non-invasive ventilation setup in obesity hypoventilation syndrome: The OPIP trial, Thorax. (2022) 78, no. 1, 24–31, https://doi.org/10.1136/thorax-2021-218497.
10.1136/thorax-2021-218497
PubMed Web of Science® Google Scholar
65 Peseschkian T., Cordts I., Gunther R., Stolte B., Zeller D., Schroter C., Weyen U., Regensburger M., Wolf J., Schneider I., Hermann A., Metelmann M., Kohl Z., Linker R. A., Koch J. C., Buchner B., Weiland U., Schonfelder E., Heinrich F., Osmanovic A., Klopstock T., Dorst J., Ludolph A. C., Boentert M., Hagenacker T., Deschauer M., Lingor P., Petri S., and Schreiber-Katz O., A nation-wide, multi-center study on the quality of life of ALS patients in Germany, Brain Sciences. (2021) 11, no. 3, https://doi.org/10.3390/brainsci11030372.
10.3390/brainsci11030372
PubMed Web of Science® Google Scholar
66 Mansell S. K., Kilbride C., Wood M. J., Gowing F., and Mandal S., Experiences and views of patients, carers and healthcare professionals on using modems in domiciliary non-invasive ventilation (NIV): A qualitative study, BMJ Open Respiratory Research. (2020) 7, no. 1, e000510, https://doi.org/10.1136/bmjresp-2019-000510.
10.1136/bmjresp-2019-000510
PubMed Web of Science® Google Scholar
67 Vitacca M., Bianchi L., Guerra A., Fracchia C., Spanevello A., Balbi B., and Scalvini S., Tele-assistance in chronic respiratory failure patients: A randomised clinical trial, European Respiratory Journal. (2008) 33, no. 2, 411–418, https://doi.org/10.1183/09031936.00005608, 2-s2.0-59649120956.
10.1183/09031936.00005608
PubMed Web of Science® Google Scholar
68 Hoet F., Libert W., Sanida C., Van den Broecke S., Bruyneel A. V., and Bruyneel M., Telemonitoring in continuous positive airway pressure-treated patients improves delay to first intervention and early compliance: A randomized trial, Sleep Medicine. (2017) 39, 77–83, https://doi.org/10.1016/j.sleep.2017.08.016, 2-s2.0-85032725165.
10.1016/j.sleep.2017.08.016
CAS PubMed Web of Science® Google Scholar
69 Lopes de Almeida J. P., Pinto A., Pinto S., Ohana B., and de Carvalho M., Economic cost of home-telemonitoring care for BiPAP-assisted ALS individuals, Amyotrophic Lateral Sclerosis. (2012) 13, no. 6, 533–537, https://doi.org/10.3109/17482968.2012.703675, 2-s2.0-84866079882.
10.3109/17482968.2012.703675
PubMed Google Scholar
70 Blouet S., Sutter J., Fresnel E., Kerfourn A., Cuvelier A., and Patout M., Prediction of severe acute exacerbation using changes in breathing pattern of COPD patients on home noninvasive ventilation, International Journal of Chronic Obstructive Pulmonary Disease. (2018) 13, 2577–2586, https://doi.org/10.2147/copd.s170242, 2-s2.0-85056735364.
10.2147/COPD.S170242
CAS PubMed Web of Science® Google Scholar
71 Elliott M. W., The interface: Crucial for successful noninvasive ventilation, European Respiratory Journal. (2004) 23, no. 1, 7–8, https://doi.org/10.1183/09031936.03.00115903, 2-s2.0-0347136009.
10.1183/09031936.03.00115903
CAS PubMed Web of Science® Google Scholar
72 Jackson C. E., Heiman-Patterson T. D., Sherman M., Daohai Y. U., Kasarskis E. J., and Nutrition/Niv Study G, Factors associated with Noninvasive ventilation compliance in patients with ALS/MND, Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration. (2021) 22, no. sup1, 40–47, https://doi.org/10.1080/21678421.2021.1917617.
10.1080/21678421.2021.1917617
CAS PubMed Web of Science® Google Scholar
73 Ramsay M., Mandal S., Suh E., Steier J., Douiri A., Murphy P. B., Polkey M. I., Simonds A., and Hart N., Parasternal electromyography to determine the relationship between patient-ventilator asynchrony and nocturnal gas exchange during home mechanical ventilation set-up, Thorax. (2015) 70, no. 10, 946–952, https://doi.org/10.1136/thoraxjnl-2015-206944, 2-s2.0-84942092184.
10.1136/thoraxjnl-2015-206944
PubMed Web of Science® Google Scholar
74 Bausewein C., Farquhar M., Booth S., Gysels M., and Higginson I. J., Measurement of breathlessness in advanced disease: a systematic review, Respiratory Medicine. (2007) 101, no. 3, 399–410, https://doi.org/10.1016/j.rmed.2006.07.003, 2-s2.0-33846828639.
10.1016/j.rmed.2006.07.003
CAS PubMed Web of Science® Google Scholar
75 D′Cruz R. F., Kaltsakas G., Suh E. S., and Hart N., Quality of life in patients with chronic respiratory failure on home mechanical ventilation, European Respiratory Review. (2023) 32, no. 168, 220237, https://doi.org/10.1183/16000617.0237-2022.
10.1183/16000617.0237-2022
PubMed Web of Science® Google Scholar
76 Spurr L., The treatment burden of long-term home noninvasive ventilation, Breathe. (2021) 17, no. 1, 200291, https://doi.org/10.1183/20734735.0291-2020.
10.1183/20734735.0291-2020
PubMed Web of Science® Google Scholar
77 Shah A. J., Florman K., Kaushal N., Kwong H. F., Karoshi A., White L., Walker R., Lin Y. P., Ko H. J., Saigal A., Devani N., Mansell S. K., and Mandal S., Factors affecting domiciliary non-invasive ventilation compliance, Lung. (2022) 200, no. 4, 457–462, https://doi.org/10.1007/s00408-022-00557-8.
10.1007/s00408-022-00557-8
PubMed Web of Science® Google Scholar
78 Borg G. A., Psychophysical bases of perceived exertion, Medicine and Science in Sports and Exercise. (1982) 14, no. 5, 377–381, https://doi.org/10.1249/00005768-198205000-00012.
10.1249/00005768-198205000-00012
CAS PubMed Web of Science® Google Scholar
79 Medical Research Council, Medical Research Council Dyspnoea Scale, 1959, Primary Care Respiratory Society, London, UK.
Google Scholar
80 Windisch W., Freidel K., Schucher B., Baumann H., Wiebel M., Matthys H., and Petermann F., The Severe Respiratory Insufficiency (SRI) Questionnaire: A specific measure of health-related quality of life in patients receiving home mechanical ventilation, Journal of Clinical Epidemiology. (2003) 56, no. 8, 752–759, https://doi.org/10.1016/s0895-4356(03)00088-x, 2-s2.0-0042385020.
10.1016/S0895-4356(03)00088-X
PubMed Web of Science® Google Scholar
81 Carone M., Bertolotti G., Anchisi F., Zotti A. M., Donner C. F., and Jones P. W., Analysis of factors that characterize health impairment in patients with chronic respiratory failure. Quality of Life in Chronic Respiratory Failure Group, European Respiratory Journal. (1999) 13, no. 6, 1293–1300, https://doi.org/10.1183/09031936.99.13613019.
10.1183/09031936.99.13613019
CAS PubMed Web of Science® Google Scholar
82 Jones P. W., Harding G., Berry P., Wiklund I., Chen W. H., and Kline Leidy N., Development and first validation of the COPD assessment test, European Respiratory Journal. (2009) 34, no. 3, 648–654, https://doi.org/10.1183/09031936.00102509, 2-s2.0-70349097551.
10.1183/09031936.00102509
CAS PubMed Web of Science® Google Scholar
83 Vincent K. A., Carr A. J., Walburn J., Scott D. L., and Rose M. R., Construction and validation of a quality of life questionnaire for neuromuscular disease (INQoL), Neurology. (2007) 68, no. 13, 1051–1057, https://doi.org/10.1212/01.wnl.0000257819.47628.41, 2-s2.0-34147108157.
10.1212/01.wnl.0000257819.47628.41
CAS PubMed Web of Science® Google Scholar
84 Ward Flemons W. and Reimer M. A., Development of a disease-specific health-related quality of life questionnaire for sleep apnea, American Journal of Respiratory and Critical Care Medicine. (1998) 158, no. 2, 494–503, https://doi.org/10.1164/ajrccm.158.2.9712036, 2-s2.0-14444282201.
10.1164/ajrccm.158.2.9712036
PubMed Web of Science® Google Scholar
85 Johns M. W., A new method for measuring daytime sleepiness: The Epworth sleepiness scale, Sleep. (1991) 14, no. 6, 540–545, https://doi.org/10.1093/sleep/14.6.540, 2-s2.0-0026333617.
10.1093/sleep/14.6.540
CAS PubMed Web of Science® Google Scholar
86 Conway A., Tipton E., Liu W. H., Conway Z., Soalheira K., Sutherland J., and Fingleton J., Accuracy and precision of transcutaneous carbon dioxide monitoring: A systematic review and meta-analysis, Thorax. (2019) 74, no. 2, 157–163, https://doi.org/10.1136/thoraxjnl-2017-211466, 2-s2.0-85053223670.
10.1136/thoraxjnl-2017-211466
PubMed Web of Science® Google Scholar
87 Olson L. G., Ambrogetti A., and Gyulay S. G., Prediction of sleep-disordered breathing by unattended overnight oximetry, Journal of Sleep Research. (1999) 8, no. 1, 51–55, https://doi.org/10.1046/j.1365-2869.1999.00134.x, 2-s2.0-0033014482.
10.1046/j.1365-2869.1999.00134.x
CAS PubMed Web of Science® Google Scholar
88 Mellies U. and Goebel C., Optimum insufflation capacity and peak cough flow in neuromuscular disorders, Annals of the American Thoracic Society. (2014) 11, no. 10, 1560–1568, https://doi.org/10.1513/annalsats.201406-264oc, 2-s2.0-84920366595.
10.1513/AnnalsATS.201406-264OC
PubMed Google Scholar
89 Chatwin M., Ross E., Hart N., Nickol A. H., Polkey M. I., and Simonds A. K., Cough augmentation with mechanical insufflation/exsufflation in patients with neuromuscular weakness, European Respiratory Journal. (2003) 21, no. 3, 502–508, https://doi.org/10.1183/09031936.03.00048102, 2-s2.0-0347579840.
10.1183/09031936.03.00048102
CAS PubMed Web of Science® Google Scholar
90 Reilly C. C., Ward K., Jolley C. J., Lunt A. C., Steier J., Elston C., Polkey M. I., Rafferty G. F., and Moxham J., Neural respiratory drive, pulmonary mechanics and breathlessness in patients with cystic fibrosis, Thorax. (2011) 66, no. 3, 240–246, https://doi.org/10.1136/thx.2010.142646, 2-s2.0-79951957123.
10.1136/thx.2010.142646
PubMed Web of Science® Google Scholar
91 Steier J., Jolley C. J., Seymour J., Roughton M., Polkey M. I., and Moxham J., Neural respiratory drive in obesity, Thorax. (2009) 64, no. 8, 719–725, https://doi.org/10.1136/thx.2008.109728, 2-s2.0-68049102196.
10.1136/thx.2008.109728
CAS PubMed Web of Science® Google Scholar
92 Murphy P. B., Kumar A., Reilly C., Jolley C., Walterspacher S., Fedele F., Hopkinson N. S., Man W. D., Polkey M. I., Moxham J., and Hart N., Neural respiratory drive as a physiological biomarker to monitor change during acute exacerbations of COPD, Thorax. (2011) 66, no. 7, 602–608, https://doi.org/10.1136/thx.2010.151332, 2-s2.0-79959304897.
10.1136/thx.2010.151332
PubMed Web of Science® Google Scholar
93 Suh E. S., Mandal S., Harding R., Ramsay M., Kamalanathan M., Henderson K., O’Kane K., Douiri A., Hopkinson N. S., Polkey M. I., Rafferty G., Murphy P. B., Moxham J., and Hart N., Neural respiratory drive predicts clinical deterioration and safe discharge in exacerbations of COPD, Thorax. (2015) 70, no. 12, 1123–1130, https://doi.org/10.1136/thoraxjnl-2015-207188, 2-s2.0-84957847308.
10.1136/thoraxjnl-2015-207188
PubMed Web of Science® Google Scholar
94 D′Cruz R. F., Suh E. S., Kaltsakas G., Dewar A., Shah N. M., Priori R., Douiri A., Rose L., Hart N., and Murphy P. B., Home parasternal electromyography tracks patient-reported and physiological measures of recovery from severe COPD exacerbation, ERJ Open Res. (2021) 7, no. 2, 00709–2020, https://doi.org/10.1183/23120541.00709-2020.
10.1183/23120541.00709-2020
PubMed Google Scholar
95 Oostveen E., MacLeod D., Lorino H., Farre R., Hantos Z., Desager K., Marchal F., and Measurements E. R. S. T. FoR. I., The forced oscillation technique in clinical practice: methodology, recommendations and future developments, European Respiratory Journal. (2003) 22, no. 6, 1026–1041, https://doi.org/10.1183/09031936.03.00089403, 2-s2.0-0346121617.
10.1183/09031936.03.00089403
CAS PubMed Web of Science® Google Scholar
96 Walker P. P., Pompilio P. P., Zanaboni P., Bergmo T. S., Prikk K., Malinovschi A., Montserrat J. M., Middlemass J., Sonc S., Munaro G., Marusic D., Sepper R., Rosso R., Siriwardena A. N., Janson C., Farre R., Calverley P. M. A., and Dellaca R. L., Telemonitoring in chronic obstructive pulmonary disease (CHROMED). A randomized clinical trial, American Journal of Respiratory and Critical Care Medicine. (2018) 198, no. 5, 620–628, https://doi.org/10.1164/rccm.201712-2404oc, 2-s2.0-85052708097.
10.1164/rccm.201712-2404OC
PubMed Web of Science® Google Scholar
97 Spina G., Spruit M. A., Alison J., Benzo R. P., Calverley P. M. A., Clarenbach C. F., Costello R. W., Donaire-Gonzalez D., Durr S., Garcia-Aymerich J., van Gestel A. J. R., Gramm M., Hernandes N. A., Hill K., Hopkinson N. S., Jarreta D., Kohler M., Kirsten A. M., Leuppi J. D., Magnussen H., Maltais F., Man W. D., McKeough Z. J., Mesquita R., Miedinger D., Pitta F., Singh S. J., Smeenk F., Tal-Singer R., Vagaggini B., Waschki B., Watz H., Wouters E. F. M., Zogg S., and den Brinker A. C., Analysis of nocturnal actigraphic sleep measures in patients with COPD and their association with daytime physical activity, Thorax. (2017) 72, no. 8, 694–701, https://doi.org/10.1136/thoraxjnl-2016-208900, 2-s2.0-85011030486.
10.1136/thoraxjnl-2016-208900
PubMed Web of Science® Google Scholar
98 Ehsan M., Khan R., Wakefield D., Qureshi A., Murray L., Zuwallack R., and Leidy N. K., A longitudinal study evaluating the effect of exacerbations on physical activity in patients with chronic obstructive pulmonary disease, Annals of the American Thoracic Society. (2013) 10, no. 6, 559–564, https://doi.org/10.1513/annalsats.201304-100oc, 2-s2.0-84892184723.
10.1513/AnnalsATS.201304-100OC
PubMed Google Scholar
99 Vanaparthy R., Mota P., Khan R., Ehsan M., Qureshi A., ZuWallack R., and Leidy N., A longitudinal assessment of sleep variables during exacerbations of chronic obstructive pulmonary disease, Chronic Respiratory Disease. (2015) 12, no. 4, 299–304, https://doi.org/10.1177/1479972315587517, 2-s2.0-84944072217.
10.1177/1479972315587517
CAS PubMed Web of Science® Google Scholar

All articles

Update in Noninvasive Home Mechanical Ventilation: A Narrative Review of Indications, Outcomes, and Monitoring

Abstract

1. Introduction

2. Principles of Ventilatory Homeostasis

3. Principles of Noninvasive Ventilation

4. Applications of Home Mechanical Ventilation

4.1. Chronic Obstructive Pulmonary Disease

4.1.1. Pathophysiology of Respiratory Failure in COPD

4.1.2. Stable Hypercapnic Respiratory Failure

4.1.3. Persistent Hypercapnia following Acute Exacerbation

4.2. Obesity-Related Respiratory Failure

4.2.1. Pathophysiology of Obesity-Related Respiratory Failure

4.2.2. Ventilatory Management of Obesity-Related Respiratory Failure

4.3. Neuromuscular and Restrictive Thoracic Disease

4.3.1. Pathophysiology of Respiratory Failure in Neuromuscular and Restrictive Thoracic Disease

4.3.2. HMV in Neuromuscular Disease

4.3.3. HMV in Restrictive Thoracic Disease

4.4. Location of HMV Setup

4.5. Monitoring HMV Users

4.5.1. Clinical Evaluation

4.5.2. Software Analysis and Telemonitoring

4.5.3. NIV Troubleshooting

4.5.4. Patient-Reported Outcomes

4.5.5. Physiological Measurements

4.5.6. Physiological Techniques under Evaluation

5. Conclusions

Conflicts of Interest

Authors’ Contributions

References

Figures

References

Information

About Wiley Online Library

Help & Support

Opportunities

Connect with Wiley