Coverage Policy Manual
Policy #: 2022005
Category: DME
Initiated: February 2022
Last Review: April 2024
  Non-Invasive Positive Airway Pressure for Chronic Obstructive Pulmonary Disease

Description:
This policy addresses devices used in the treatment of severe chronic obstructive pulmonary disease (COPD), chronic respiratory failure, thoracic restrictive disorders, and hypoventilation syndromes.
 
Respiratory failure is characterized by low arterial blood oxygen (hypoxemia, PaO2) and/or high arterial carbon dioxide (hypercapnia, PaCO2 greater than 45 mmHg). Chronic respiratory insufficiency or failure can occur with chronic obstructive pulmonary disease (COPD) and may result in poor quality of life, sleepiness, hospital admission, intubation, and death. Non-invasive positive airway pressure ventilation (NPPV) including continuous positive airway pressure (CPAP), bilevel positive airway pressure (BPAP) and home mechanical ventilators (HMV) that are pressure, rate, and volume targeted are proposed for the treatment of COPD.
 
Chronic Obstructive Pulmonary Disease
Chronic obstructive pulmonary disease (COPD) is a common condition, affecting more than 5% of the population, and is associated with high morbidity and mortality. COPD is the fourth leading cause of death in the United States. It is a clinical syndrome with multiple etiologies that is characterized by chronic respiratory symptoms, structural pulmonary abnormalities, and/or lung function impairment. Chronic obstructive pulmonary disease is most frequently associated with cigarette smoking or other air pollutants, and a majority of patients with COPD in the United States have a history of cigarette smoking. Chronic obstructive pulmonary disease is progressive, with expiratory airflow limitation, air trapping/hyperinflation, and destruction of alveoli (emphysema). The Global Initiative for Chronic Obstructive Lung Disease (GOLD) defines COPD as "a heterogeneous lung condition characterized by chronic respiratory symptoms (dyspnea, cough, sputum production and/or exacerbations) due to abnormalities of the airways (bronchitis, bronchiolitis) and/or alveoli (emphysema) that cause persistent, often progressive, airflow obstruction" (GOLD, 2023; Macrea, 2020).
 
Respiratory failure in patients with COPD is characterized by the inability to sustain normal gas exchange, leading to low arterial blood oxygen (hypoxemia, PaO2) and/or high arterial carbon dioxide (hypercapnia, PaCO2). Hypercapnia develops in about one-third of patients with COPD and is associated with poor quality of life, sleepiness, frequent hospital admissions due to exacerbations, and an increase in mortality compared to patients with COPD who are normocapnic. The hypercapnia is due in large part to poor lung biomechanics including low inspiratory muscle reserve, high CO2 production, and a reduced ventilatory capability (Mathews, 2020). The imbalance between the respiratory load and respiratory capability may in turn affect the ventilatory control center in the brain stem. Physiological changes in responsiveness to hypoxemia and hypercapnia during sleep can be particularly pronounced in patients with COPD, with overnight increases in PaCO2 affecting daytime PaCO2, possibly through bicarbonate retention or changes in cerebrospinal fluid (Orr, 2020). Patients with COPD may also have comorbid obstructive sleep apnea and/or obesity hypoventilation syndrome due to decreased ventilatory motor output and upper airway muscle activity during sleep.
 
Thoracic Restrictive Disorders Due to Neuromuscular Disease
Thoracic restrictive disorders result from a variety of underlying diseases all characterized by restrictive patterns on pulmonary function testing (Martinez-Pitre, 2022). Neuromuscular disorders such as muscular dystrophy, amyotrophic lateral sclerosis (ALS), polio, and phrenic neuropathies can result in weakness of the respiratory muscles affecting inspiration and expiration, ultimately resulting in hypoventilation. Impaired cough and swallowing associated with neuromuscular disease increases the risk of respiratory complications in these patients (Carmona, 2023). Nocturnal hypoventilation due to muscular atonia during sleep leads to nocturnal hypercapnia. Frequent nocturnal episodes can result in renal compensation and ultimately result in daytime hypercapnia. Non-invasive positive airway pressure ventilation (NPPV) is often necessary for patients with thoracic restrictive disorders due to neuromuscular disease.
 
Hypoventilation Syndromes
Hypoventilation syndromes are nonspecific disorders characterized by hypercapnia (PaCO2 greater than 45 mm Hg) that is not otherwise categorized (Gay, 2021). Obesity hypoventilation syndrome (OHS), central respiratory depression due to substance or medication use, and decompensated hypercapnic respiratory failure that is not COPD are all included in this category. In patients with OHS, weight loss is useful in normalizing PaCO2; however, NPPV should be initiated early while weight loss is attempted (McConville, 2022).
 
Treatment With Non-invasive Positive Airway Pressure
A major goal of management of patients with COPD is to reduce hospitalizations and mortality. Long-term oxygen therapy is recommended for patients with poor clinical status and noninvasive positive airway pressure ventilation (NPPV) devices for patients with severe chronic hypercapnia and a history of hospitalization for acute respiratory failure. Noninvasive positive airway pressure ventilation devices include nocturnal continuous positive airway pressure (CPAP) for individuals with hypercapnia due to obstructive sleep apnea or hypoventilation and bilevel positive airway pressure (BPAP) devices or non-invasive home mechanical ventilators that are pressure, rate, and volume targeted. The objective of this evidence review is to describe which features of NPPV are required to improve the net health outcome in patients with COPD.
 
Benefits of nocturnal NPPV persist into the daytime with improved breathing patterns (lower frequencies and larger tidal volumes) and improved gas exchange. Explanations for the improvement in daytime respiration with nocturnal NPPV include increased respiratory drive, improved diaphragm function by unloading the respiratory muscles during sleep, increased CO2 sensitivity, and reduction in air trapping and hyperinflation. It is not known which factors (e.g., muscle unloading, gas exchange normalization, decrease in hyperinflation) underlie the benefits of NPPV on health outcomes. It is also unclear if the reduction in PaCO2 has an effect on health outcomes or if it is only a marker of effective ventilation (Orr, 2020).
 
Respiratory Assist Devices
The Centers for Medicare and Medicaid Services (CMS) defines respiratory assist devices (RADs) as bilevel devices with or without back-up respiratory rate capability. While CPAP devices provide continuous air at a pressure that prevents the collapse of the airway during inspiration, BPAP devices work by increasing pressure during inspiration and lowering it during expiration (pressure cycled). In some devices a backup respiratory rate is triggered when the patient's nocturnal respiratory rate decreases below a set threshold. The backup rate is typically set 2 breaths below the patient's spontaneous respiratory rate during wakefulness.
 
Home Mechanical Ventilators
In some patients, nocturnal respiratory assist devices are insufficient to address the respiratory failure. Non-invasive home mechanical ventilators (HMV) are proposed for the treatment of chronic respiratory failure that is refractory to a respiratory assist device. Mechanical ventilators are devices that deliver more controlled breathing with bilevel ventilation at a higher pressure. The ventilators may also have additional features compared to BPAP machines such as alarms and battery backup power. Home mechanical ventilators can be used for patients with tracheostomy in the home but may also be used with a non-invasive interface such as a mask or mouthpiece in patients who do not depend on 24 hour ventilation for survival. Current technology has decreased the size of home ventilators to around 10 pounds. In addition, some models may be wireless with battery backup, allowing greater mobility during the day.
 
Titration
Early studies with low intensity NPPV did not demonstrate health benefits in patients with hypercapnia. More recent studies have reinforced the importance of high-intensity NPPV (> 18 cm H2O) that is titrated to decrease hypercapnia. A high respiratory backup rate that is increased to the level of spontaneous breathing has also been shown to be important to achieve positive health outcomes. Manually set, laboratory or hospital titration of NPPV with pressure control and backup rate have been recommended for stable hypercapnic COPD (Wiles, 2020). The goal of titration of inspiratory positive airway pressure is to achieve normocapnia, a reduction in transcutaneous CO2, or maximum tolerable inspiratory pressure. A fast rise in inspiratory pressure (rise time) allows enough time for expiration within the normal rate of breathing. In patients with air trapping and hyperinflation, use of positive end-expiratory pressure can also be beneficial.
 
A suggested protocol for in-laboratory titration of NPPV in patients with COPD in the U.S. is described by Orr et al (Orr, 2020). Titration of NPPV is usually performed in a monitored environment after the patient has stabilized, as studies have not found an improvement in health outcomes when NPPV is started soon after an acute exacerbation. Polysomnography or respiratory monitoring may be used during titration to evaluate the presence of obstructive sleep apnea or hypoventilation. The inspiratory pressure is typically started at 6 to 8 cm H2O of pressure support above the expiratory pressure and titrated to reduce hypercapnia. A Bilevel-ST (with backup rate) or a VAPS (volume assured) may be used if a Bilevel-S (without backup rate) fails to adequately reduce hypercapnia. Although titration in European studies has been performed with a hospital stay, this is not feasible in the U.S., and titration might be performed over several weeks in the patient's home by an external durable medical equipment (DME) provider.
 
Pulmonary Rehabilitation
Pulmonary rehabilitation is a personalized intervention that includes physical activity (e.g., activities of daily living, endurance exercises and muscle strengthening), health education, and psychological support. It may be performed in the hospital, outpatient clinic, or home, and has been shown to reduce mortality, exacerbation rate, intensive care admissions, and emergency department visits. Pulmonary rehabilitation is common in Europe but is less frequently provided in the U.S.
 
Regulatory Status
Numerous CPAP and BPAP devices are available in the U.S. Examples of HMV devices that have both invasive and non-invasive interfaces and are available in the U.S. are described below:
 
    • Trilogy Evo Ventilator, manufactured by Respironics, (K181166) received FDA clearance in 2019. FDA Product Code: NOU, CBK
    • Vivo 60, manufactured by Breas, (K160481) received FDA clearance in 2016. FDA Product Code: NOU, CBK, DQA, CCK
    • Astral 100/150, manufactured by ResMed, (K152068) received FDA clearance in 2016. FDA Product Code: NOU, CBK
    • Newport, manufactured by Medtronic, (K121891) received FDA clearance in 2012. FDA Product Code: NOU, CBK
    • iVent, manufactured by GE Healthcare, (K092135) received FDA clearance in 2009. FDA Product Code: NOU, CBK
    • LTV, manufactured by Cardinal Health, (K083688) received FDA clearance in 2009. FDA Product Code: CBK 
    • Puritan Bennett 540, manufactured by Covidien, (K082966) received FDA clearance in 2008. FDA Product Code: CBK 

Policy/
Coverage:
Effective November 2024
 
For treatment of obstructive sleep apnea and central sleep apnea, please see coverage policy 1997195, Sleep Apnea Ventilation Support and Respiratory Assist Devices.
 
Meets Primary Coverage Criteria Or Is Covered For Contracts Without Primary Coverage Criteria
 
Nocturnal bilevel positive airway pressure (BPAP) (with backup rate meets member benefit certificate primary coverage criteria that there be scientific evidence of effectiveness in improving health outcomes for individuals with COPD and chronic respiratory failure who meet either of the following:
 
    • Chronic stable daytime (awake) hypercapnia (PaCO2 greater than or equal to 52 mmHg); OR
    • Daytime (awake) hypercapnia (PaCO2 greater than or equal to 52 mmHg) at least 2 weeks after discharge from the hospital for an acute exacerbation with decompensated acidosis.
 
Non-invasive home mechanical ventilation meets member benefit certificate primary coverage criteria that there be scientific evidence of effectiveness in improving health outcomes for individuals with COPD who meet the following criteria:
 
    • Qualify for a bilevel positive airway pressure (BPAP) device AND meet at least one of the following:
a. Higher pressure is needed to reduce hypercapnia than can be achieved with a bilevel device during titration (typically greater than 25cm H2O); OR
b. Severe hypoxemia requiring FIO2 greater than 40% or greater than 5 L/min; OR
c. Daytime use (battery operated unit) is required to reduce hypercapnia.
 
Individuals with COPD who are started on bilevel positive airway pressure (BPAP) at discharge from hospitalization may continue for up to 3 months to provide time to stabilize and complete reevaluation.
 
Bilevel positive airway pressure (BPAP) meets member benefit certificate primary coverage criteria that there be scientific evidence of effectiveness in improving health outcomes for individuals with thoracic restrictive disorders due to neuromuscular disease who meet any of the following:
 
    • Pulmonary function tests:
a. Spirometry (upright or supine) with vital capacity <50% predicted or <80% predicted with associated symptoms (orthopnea, dyspnea, morning headaches, excessive daytime sleepiness, or unrefreshing sleep); OR
b. Maximal inspiratory pressure less than 60 cm H2O or maximum expiratory pressure (MEP) less than 40 cm H2O; OR
c. Peak cough flow (PCF) less than 270 L/min for age 12 years and older or PCF less than 5th percentile for age less than 12 years; OR
d. Sniff nasal inspiratory pressure (SNIP) less than 70 cm H2O in males, SNIP less than 60 cmH2O in females for age 12 years.
    • Hypercapnia
a. Chronic stable daytime (awake) hypercapnia with PaCO2 greater than 45 mmHg (capillary blood gas can be used in children);
b. Venous blood gas PCO2, end-tidal PCO2, or transcutaneous PCO2, greater than 50 mmHg; OR
    • Hypoxia
a. Overnight oximetry in-laboratory or home sleep test with saturation less than 88% for 5 minutes; OR
b. Overnight oximetry: SpO2 less than or equal to 90% for greater than or equal to 2% of sleep time.
 
Non-invasive home mechanical ventilation meets member benefit certificate primary coverage criteria that there be scientific evidence of effectiveness in improving health outcomes for individuals with thoracic restrictive disorders due to neuromuscular disease who meet the following:
 
    • Qualify for a bilevel positive airway pressure (BPAP) device; AND
a. BPAP fails; OR
b. Have extreme loss in function with vital capacity less than 30%; OR
c. Non-invasive ventilation is needed for more than 10 hours per day; OR
d. Severe breathlessness (e.g., with speaking at rest); OR
e. Worsening daytime hypercapnia with need for mouthpiece ventilation; OR
f. Daytime use (battery operated unit) is required to reduce hypercapnia or dyspnea.
 
Bilevel positive airway pressure (BPAP) meets member benefit certificate primary coverage criteria that there be scientific evidence of effectiveness in improving health outcomes for individuals with hypoventilation syndromes who meet the following criteria:
 
    • Awake or sleep hypoventilation with hypercapnia (one of the following is met):
a. Awake hypoventilation with chronic stable daytime (awake) hypercapnia (PaCO2 greater than or equal to 45 mmHg); OR
b. Venous blood gas PCO2, end-tidal PCO2, or transcutaneous PCO2 greater than or equal to 50 mmHg; OR
c. Sleep hypoventilation with hypercapnia:
i.  Greater than or equal to 10 mmHg increase from baseline awake PCO2 and to a value greater than 50 mmHg for 10 min or longer; OR
ii. PCO2 greater than or equal to 55 mmHg for 10 min or longer; AND
    • Low clinical suspicion for COPD or neuromuscular disease; AND
    • One of the following conditions are met:
a. Obesity with body mass index (BMI) greater than or equal to 30 kg/m2; OR
b. Decreased respiratory drive due to opioid or substance use; OR
c. Advanced lung disease other than COPD (e.g., end-stage or advanced interstitial lung disease); AND
    • Individual was discharged from inpatient stay with persistent awake hypoventilation (hypercapnia) on BPAP.
a. A reassessment with a provider within 3 months (30-90 days) is required and an attended polysomnogram (PSG) should be performed to assess appropriateness of positive airway pressure modality (home sleep apnea test is acceptable if attended PSG).
    • Individual is ambulatory and sleep study indicates that bilevel positive airway pressure (BPAP) is necessary for sleep-disordered breathing, or individual with severe obstructive sleep apnea is continuous positive airway pressure intolerant or continuous positive airway pressure was proven ineffective.
 
Non-invasive home mechanical ventilation meets member benefit certificate primary coverage criteria that there be scientific evidence of effectiveness in improving health outcomes for individuals with hypoventilation syndromes who meet the following:
 
    • Qualify for a bilevel positive airway pressure (BPAP) device and at least one of the following:
a. Higher pressure is needed to reduce hypercapnia than can be achieved with a bilevel continuous positive airway pressure device during titration (typically greater than 25 cm H2O); OR
b. Severe hypoxemia requiring fraction of inspired oxygen (FIO2) greater than 40% or greater than 5 L/min; OR
c. Daytime use (battery operated unit) is required to reduce hypercapnia; OR
    • Tried and failed bilevel positive airway pressure (BPAP) device with persistent hypercapnia despite 3 months of adequate adherence to prescribed positive airway pressure therapy with:
a. Awake PaCO2 greater than or equal to 45 mmHg; OR
b. Awake venous blood gas PCO2, end-tidal PCO2, or transcutaneous PCO2 greater than or equal to 50 mmHg.
 
Does Not Meet Primary Coverage Criteria Or Is Investigational For Contracts Without Primary Coverage Criteria
 
Nocturnal bilevel positive airway pressure (BPAP) with backup rate for patients with COPD and chronic respiratory failure not meeting the criteria listed above does not meet member benefit certificate primary coverage criteria that there be scientific evidence of effectiveness in improving health outcomes.
 
For members with contracts without primary coverage criteria, nocturnal bilevel positive airway pressure (BPAP) with backup rate for patients with COPD and chronic respiratory failure not meeting the criteria listed above is considered investigational. Investigational services are specific contract exclusions in most member benefit certificates of coverage.
 
Non-invasive home mechanical ventilation for patients with COPD not meeting the criteria listed above does not meet member benefit certificate primary coverage criteria that there be scientific evidence of effectiveness in improving health outcomes.
 
For members with contracts without primary coverage criteria, non-invasive home mechanical ventilation for patients with COPD not meeting the criteria listed above is considered investigational. Investigational services are specific contract exclusions in most member benefit certificates of coverage.
 
Bilevel positive airway pressure (BPAP) for individuals with thoracic restrictive disorders due to neuromuscular disease not meeting the criteria listed above or for any other indication does not meet member benefit certificate primary coverage criteria that there be scientific evidence of effectiveness in improving health outcomes.
 
For members with contracts without primary coverage criteria, bilevel positive airway pressure (BPAP) for individuals with thoracic restrictive disorders due to neuromuscular disease not meeting the criteria listed above or for any other indication is considered investigational. Investigational services are specific contract exclusions in most member benefit certificates of coverage.
 
Non-invasive home mechanical ventilation for individuals with thoracic restrictive disorders due to neuromuscular disease not meeting the criteria listed above or for any other indication does not meet member benefit certificate primary coverage criteria of effectiveness in improving health outcomes.
  
For members with contracts without primary coverage criteria, non-invasive home mechanical ventilation for individuals with thoracic restrictive disorders due to neuromuscular disease not meeting the criteria listed above or for any other indication is considered investigational. Investigational services are specific contract exclusions in most member benefit certificates of coverage.
 
Bilevel positive airway pressure (BPAP) for individuals with hypoventilation syndromes not meeting the criteria listed above or for any other indication does not meet member benefit certificate primary coverage criteria that there be scientific evidence of effectiveness in improving health outcomes.
 
For members with contracts without primary coverage criteria, bilevel positive airway pressure (BPAP) for individuals with hypoventilation syndromes not meeting the criteria listed above or for any other indication is considered investigational. Investigational services are specific contract exclusions in most member benefit certificates of coverage.
 
Non-invasive home mechanical ventilation for individuals with hypoventilation syndromes not meeting the criteria listed above or for any other indication does not meet member benefit certificate primary coverage criteria that there be scientific evidence of effectiveness in improving health outcomes.
 
For members with contracts without primary coverage criteria, non-invasive home mechanical ventilation for individuals with hypoventilation syndromes not meeting the criteria listed above or for any other indication is considered investigational. Investigational services are specific contract exclusions in most member benefit certificates of coverage.
 
Effective October 2023 through October 2024
 
Meets Primary Coverage Criteria Or Is Covered For Contracts Without Primary Coverage Criteria
 
Nocturnal bilevel positive airway pressure with backup rate meets member benefit certificate primary coverage criteria that there be scientific evidence of effectiveness in improving health outcomes for individuals with COPD and chronic respiratory failure who meet either of the following:
 
      1. Chronic stable daytime (awake) hypercapnia (PaCO2 greater than or equal to 52 mmHg); OR
      2. Daytime (awake) hypercapnia (PaCO2 greater than or equal to 52 mmHg) at least 2 weeks after discharge from the hospital for an acute exacerbation with decompensated acidosis.
 
Non-invasive home mechanical ventilation meets member benefit certificate primary coverage criteria that there be scientific evidence of effectiveness in improving health outcomes for individuals with COPD who meet the following criteria:
 
      1. Qualify for a bilevel positive airway pressure device AND meet at least one of the following:
a. Higher pressure (is needed to reduce hypercapnia than can be achieved with a bilevel device during titration (typically greater than 25cm H2O); OR
b. Severe hypoxemia requiring FIO2 > 40% or > 5 L/min; OR
c. Daytime use (battery operated unit) is required to reduce hypercapnia.
 
Individuals with COPD who are started on bilevel positive airway pressure at discharge from hospitalization may continue for up to 3 months to provide time to stabilize and complete reevaluation.
 
Bilevel positive airway pressure meets member benefit certificate primary coverage criteria of effectiveness in improving health outcomes for individuals with thoracic restrictive disorders due to neuromuscular disease who meet any of the following:
 
      1. Pulmonary function tests:
a. Spirometry (upright or supine) with vital capacity <50% predicted or <80% predicted with associated symptoms (orthopnea, dyspnea, morning headaches, excessive daytime sleepiness, or unrefreshing sleep); OR
b. Maximal inspiratory pressure <60 cm H2O or maximum expiratory pressure (MEP) <40 cm H2O; OR
c. Peak cough flow (PCF) <270 L/min for age 12 years or PCF <5th percentile for age <12 years; OR
d. Sniff nasal inspiratory pressure (SNIP) <70 cm H2O in males, SNIP <60 cmH2O in females for age 12 years.
2. Hypercapnia
a. Chronic stable daytime (awake) hypercapnia with PaCO2 >45 mmHg (capillary blood gas can be used in children);
b. Venous blood gas PCO2, end-tidal PCO2, or transcutaneous PCO2, >50 mmHg; OR
3. Hypoxia
a. Overnight oximetry in-laboratory or home sleep test with saturation <88% for 5 minutes; OR
b. Overnight oximetry: SpO2 90% for 2% of sleep time.
 
Non-invasive home mechanical ventilation meets member benefit certificate primary coverage criteria of effectiveness in improving health outcomes for individuals with thoracic restrictive disorders due to neuromuscular disease who meet the following:
 
      1. Qualify for a bilevel positive airway pressure device; AND
a. BPAP fails; OR
b. Have extreme loss in function with vital capacity <30%; OR
c. Non-invasive ventilation is needed for >10 hours per day; OR
d. Severe breathlessness (e.g., with speaking at rest); OR
e. Worsening daytime hypercapnia with need for mouthpiece ventilation; OR
f. Daytime use (battery operated unit) is required to reduce hypercapnia or dyspnea.
 
Bilevel positive airway pressure meets member benefit certificate primary coverage criteria of effectiveness in improving health outcomes for individuals with hypoventilation syndromes who meet the following criteria:
 
      1. Awake or sleep hypoventilation with hypercapnia (one of the following is met):
a. Awake hypoventilation with chronic stable daytime (awake) hypercapnia (PaCO2 45 mmHg); OR
b. Venous blood gas PCO2, end-tidal PCO2, or transcutaneous PCO2 50 mmHg; OR
c. Sleep hypoventilation with hypercapnia:
i.  10 mmHg increase from baseline awake PCO2 and to a value > 50 mmHg for 10 min; OR
ii. PCO2 55 mmHg for 10 min; AND
2. Low clinical suspicion for COPD or neuromuscular diaseas; AND
3. One of the following conditions are met:
a. Obesity with body mass index (BMI) 30 kg/m2; OR
b. Decreased respiratory drive due to opioid or substance use; OR
c. Advanced lung disease other than COPD (e.g., end-stage or advanced interstitial lung disease); AND
4. Individual was discharged from inpatient stay with persistent awake hypoventilation (hypercapnia) on BPAP.
a. A reassessment with a provider within 3 months (30-90 days) is required and an attended polysomnogram (PSG) should be performed to assess appropriateness of positive airway pressure modality (home sleep apnea test is acceptable if attended PSG is not obtainable); OR
5. Individual is ambulatory and sleep study indicates that bilevel positive airway pressure is necessary for sleep-disordered breathing, or individual with severe obstructive sleep apnea is continuous positive airway pressure intolerant or continuous positive airway pressure was proven ineffective.
 
Non-invasive home mechanical ventilation meets member benefit certificate primary coverage criteria of effectiveness in improving health outcomes for individuals with hypoventilation syndromes who meet the following:
 
1. Qualify for a bilevel positive airway pressure device and at least one of the following:
a. Higher pressure is needed to reduce hypercapnia than can be achieved with a bilevel continuous positive airway pressure device during titration (typically >25 cm H2O); OR
b. Severe hypoxemia requiring fraction of inspired oxygen (FIO2) >40% or >5 L/min; OR
c. Daytime use (battery operated unit) is required to reduce hypercapnia; OR
2. Tried and failed bilevel positive airway pressure device with persistent hypercapnia despite 3 months of adequate adherence to prescribed positive airway pressure therapy with:
a. Awake PaCO2 45 mmHg; OR
b. Awake venous blood gas PCO2, end-tidal PCO2, or transcutaneous PCO2 50 mmHg.
 
 
Does Not Meet Primary Coverage Criteria Or Is Investigational For Contracts Without Primary Coverage Criteria
 
Nocturnal bilevel positive airway pressure with backup rate for patients with COPD and chronic respiratory failure not meeting the criteria listed above does not meet member benefit certificate primary coverage criteria that there be scientific evidence of effectiveness in improving health outcomes.
 
For members with contracts without primary coverage criteria, nocturnal bilevel positive airway pressure with backup rate for patients with COPD and chronic respiratory failure not meeting the criteria listed above is considered investigational. Investigational services are specific contract exclusions in most member benefit certificates of coverage.
 
Non-invasive home mechanical ventilation for patients with COPD not meeting the criteria listed above does not meet member benefit certificate primary coverage criteria that there be scientific evidence of effectiveness in improving health outcomes.
 
For members with contracts without primary coverage criteria, non-invasive home mechanical ventilation for patients with COPD not meeting the criteria listed above is considered investigational. Investigational services are specific contract exclusions in most member benefit certificates of coverage.
 
Bilevel positive airway pressure for individuals with thoracic restrictive disorders due to neuromuscular disease not meeting the criteria listed above or for any other indication does not meet member benefit certificate primary coverage criteria of effectiveness in improving health outcomes.
 
For members with contracts without primary coverage criteria, bilevel positive airway pressure for individuals with thoracic restrictive disorders due to neuromuscular disease not meeting the criteria listed above or for any other indication is considered investigational. Investigational services are specific contract exclusions in most member benefit certificates of coverage.
 
Non-invasive home mechanical ventilation for individuals with thoracic restrictive disorders due to neuromuscular disease not meeting the criteria listed above or for any other indication does not meet member benefit certificate primary coverage criteria of effectiveness in improving health outcomes.
  
For members with contracts without primary coverage criteria, non-invasive home mechanical ventilation for individuals with thoracic restrictive disorders due to neuromuscular disease not meeting the criteria listed above or for any other indication is considered investigational. Investigational services are specific contract exclusions in most member benefit certificates of coverage.
 
Bilevel positive airway pressure for individuals with hypoventilation syndromes not meeting the criteria listed above or for any other indication does not meet member benefit certificate primary coverage criteria of effectiveness in improving health outcomes.
 
For members with contracts without primary coverage criteria, bilevel positive airway pressure for individuals with hypoventilation syndromes not meeting the criteria listed above or for any other indication is considered investigational. Investigational services are specific contract exclusions in most member benefit certificates of coverage.
 
Non-invasive home mechanical ventilation for individuals with hypoventilation syndromes not meeting the criteria listed above or for any other indication does not meet member benefit certificate primary coverage criteria of effectiveness in improving health outcomes.
 
For members with contracts without primary coverage criteria, non-invasive home mechanical ventilation for individuals with hypoventilation syndromes not meeting the criteria listed above or for any other indication is considered investigational. Investigational services are specific contract exclusions in most member benefit certificates of coverage.
 
Effective May 15, 2022 through September 2023
 
Meets Primary Coverage Criteria Or Is Covered For Contracts Without Primary Coverage Criteria
 
Nocturnal bilevel positive airway pressure with backup rate meets member benefit certificate primary coverage criteria that there be scientific evidence of effectiveness in improving health outcomes for patients with COPD and chronic respiratory failure who meet either of the following:
 
    • Chronic stable daytime (awake) hypercapnia (PaCO2 greater than or equal to 52 mmHg); OR
    • Daytime (awake) hypercapnia (PaCO2 greater than or equal to 52 mmHg) at least 2 weeks after discharge from the hospital for an acute exacerbation with decompensated acidosis.
 
 
Non-invasive home mechanical ventilation meets member benefit certificate primary coverage criteria that there be scientific evidence of effectiveness in improving health outcomes for patients with COPD who meet the following criteria:
 
    • Qualify for a bilevel positive airway pressure device AND meet at least one of the following:
 
        • Higher pressure (e.g., greater than 25 cm H2O) is needed to reduce hypercapnia than can be achieved with a bilevel device during titration; OR
        • Severe hypoxemia requiring FIO2 > 40% or > 5 L/min; OR
        • Daytime use (battery operated unit) is required to reduce hypercapnia.
 
Individuals with COPD who are started on bilevel positive airway pressure at discharge from hospitalization may continue for up to 3 months to provide time to stabilize and complete reevaluation.
 
Does Not Meet Primary Coverage Criteria Or Is Investigational For Contracts Without Primary Coverage Criteria
 
Nocturnal bilevel positive airway pressure with backup rate for patients with COPD and chronic respiratory failure not meeting the criteria listed above does not meet member benefit certificate primary coverage criteria that there be scientific evidence of effectiveness in improving health outcomes.
 
For members with contracts without primary coverage criteria, nocturnal bilevel positive airway pressure with backup rate for patients with COPD and chronic respiratory failure not meeting the criteria listed above is considered investigational. Investigational services are specific contract exclusions in most member benefit certificates of coverage.
 
Non-invasive home mechanical ventilation for patients with COPD not meeting the criteria listed above does not meet member benefit certificate primary coverage criteria that there be scientific evidence of effectiveness in improving health outcomes.
 
For members with contracts without primary coverage criteria, non-invasive home mechanical ventilation for patients with COPD not meeting the criteria listed above is considered investigational. Investigational services are specific contract exclusions in most member benefit certificates of coverage.

Rationale:
This evidence review was created in December 2021 with a search of the PubMed database. The most recent literature update was performed through November 9, 2021.
 
Continuous Positive Airway Pressure
A 2021 evidence review by the American Thoracic Society found several studies suggesting that identification and treatment with CPAP in patients with overlap syndrome improves outcomes (Macrea, 2020). However, no trials were identified that compared an OSA screening strategy with no screening strategy in patients with stable hypercapnic COPD or with use of CPAP in patients already on other modes of positive airway pressure (PAP).
 
Marin et al evaluated outcomes of patients who have both COPD and OSA from a prospective database of patients referred to a university sleep clinic (Marin, 2010). The authors analyzed 3 groups of patients; those with overlap syndrome who were started on CPAP (n=228), those with overlap syndrome not treated with CPAP (n=213), and patients with COPD who did not have OSA (n=210). Patients were seen at least once a year or until death, and the primary outcome was time to death from any cause. The secondary outcome was the time to first severe COPD exacerbation requiring hospitalization. There were 589 patients with overlap syndrome and 210 with COPD and simple snoring. Positive pressure was recommended for 468 patients, another 121 patients did not qualify for CPAP and were given other options for treatment.
 
Of the 468 patients with overlap syndrome who received a recommendation for PAP, 228 were treated with CPAP, 27 were treated with bilevel positive airway pressure (BPAP), and 213 did not accept treatment. The apnea/hypopnea index (AHI) was similar (35 vs. 34) in the 2 groups; these patients would have received a recommendation for treatment based on presence of clinically significant OSA. Median follow-up was 9.4 years (range, 3.3 to 12.7). When compared to patients with COPD only, patients with overlap syndrome who were not treated with PAP had a higher mortality risk (42.2% vs. 24.2%; relative risk [RR] 1.79; 95% confidence interval [CI]: 1.16 to 2.77) and were more likely to suffer a severe COPD exacerbation leading to hospitalization (RR 1.70; 95% CI: 1.21 to 2.38). Patients with overlap syndrome treated with CPAP did not have a statistically significant increased risk for death from any cause (31.6%) compared to patients with COPD only (24.2%). There were a number of baseline differences between the COPD only and the overlap syndrome groups which may impact mortality, but the 2 overlap groups were well matched for baseline characteristics. The main conclusion of the study was that patients with COPD should be screened for OSA and offered treatment.
 
Similar findings were reported by Machado et al, who reported a prospective comparative study of 95 patients with moderate to severe OSA and hypoxemic COPD who were offered CPAP treatment (Machado, 2010). Of the 95 patients, 61 (64%) accepted CPAP and were adherent while 34 did not accept or were not adherent. After adjusting for confounders, patients treated with CPAP had a significantly lower risk of death (hazard ratio of 0.19; 95% CI: 0.08 to 0.48).
 
Other studies have evaluated the patient characteristics that show most benefit from PAP. Jaoude et al evaluated 271 consecutive patients with overlap syndrome who were seen at a Veterans Administration sleep center (Kapide. 2014). Of the 271 overlap patients identified, 104 were considered hypercapnic (mean PaCO2 = 51.6 + 4.3 mmHg), indicating that the level of hypercapnia was lower than in other studies that had a threshold of 52 mmHg. The normocapnic and hypercapnic patients had similar AHI (29.3 + 23.8 and 35.2 + 29.2 events per hour, respectively; p=.07) and similar adherences rates to CPAP (43% and 42%, respectively). During a median follow-up of 71 months, mortality was higher in patients who were hypercapnic (35%) compared to patients who were normocapnic (17%, p=.001). Mortality in patients who were hypercapnic was lower in those who were adherent to CPAP compared to those who were not (p=.04), but adherence to CPAP had little impact on mortality in normocapnic patients (p=.42).
 
Singh et al evaluated the impact of PAP therapy on emergency room visits and hospitalization rates in Medicare beneficiaries with overlap syndrome (Singh, 2019). Using a 5% Medicare sample of claims data from 2010 to 2012, they identified 319 patients with overlap syndrome who were new users of PAP therapy in 2011. Subjects were categorized by age (66-74, 75-84, greater than or equal to 85), gender, race, socioeconomic status, CMS geographic region, comorbidity score, COPD complexity, tobacco use, and selected comorbidities. Comorbidities included hypertension (84%), diabetes (42%), and congestive heart failure (32%); 63% of individuals had 3 or more comorbidities. COPD complexity was based on pulmonary and non-pulmonary comorbid conditions and the prevalence of exacerbations and utilization of healthcare services; 26.0% of individuals were considered low complexity, 57.7% moderate complexity, and 16.3% high complexity. When compared with the year before PAP initiation, hospitalization for COPD-related conditions was significantly reduced (19.4% vs. 25.4%, p=.03). Rates of emergency room visits and hospitalization for any cause were not significantly different for the pre- and post-initiation periods. Although the benefit was seen primarily in patients who were considered to be of higher complexity, no information could be obtained from the claims data on the severity of COPD and OSA.
 
Bilevel Positive Airway Pressure
An updated Cochrane review by Raveling et al evaluated the evidence for nocturnal NPPV for the treatment of either stable COPD or COPD after an acute exacerbation (Raveling, 2021). The primary outcomes were arterial blood gas and exercise capacity measured with the 6-minute walk distance (6MWD) as well as readmissions for acute exacerbations. Where available, the meta-analysis included individual patient data (chronic: n=778; acute exacerbation: n=364) along with missing data from the primary RCTs. Criteria for inclusion were RCT, NPPV prescribed for at least 4 hours per night, and use for greater than 3 weeks; trials that allowed daytime use were excluded from the review. Most of the trials were conducted in Europe and excluded patients with OSA or high body mass index (BMI).
 
The update included 17 trials on stable COPD and 4 trials on NPPV after an acute exacerbation of COPD. Bilevel positive airway pressure with a backup rate was used in 3 studies, including the trials in 2014 by Kohnlein, 2017 by Murphy et al, and 2014 by Struik et al described below. Eight studies were judged to be at low risk of bias, including the 3 by Kohnlein et al, Murphy et al and Struik et al. Sources of heterogeneity were baseline hypercapnia (PaCO2 < 7.3 kPa vs. greater than or equal to 7.3 kPa) and the mean inspiratory pressure of NPPV (< 18 cm H2O vs. greater than or equal to 18 cm H2O). Sensitivity analysis removing studies with high or unclear risk of bias in the chronic COPD cohort increased the treatment effects for PaO2, PaCO2, 6MWD, and health related quality of life (HRQL). The authors concluded that the addition of chronic NPPV to standard treatment improves diurnal hypercapnia (high certainty), and there was evidence that NPPV improved PaO2 and all cause mortality (moderate certainty) and HRQL (very low certainty). In patients with stable COPD, the effect on gas exchange seemed to be larger in people with more severe hypercapnia (PaCO2 greater than or equal to 7.3 kPa), better treatment compliance (> 5 hours per night), and treated with a high inspiratory pressure (IPAP  greater than or equal to > 18 cm H2O). The uncertain contribution of improvements in gas exchange, along with possible mechanisms, for an improvement in health outcomes were discussed.
 
Wilson et al published a meta-analysis that included 21 RCTs and 12 observational studies (N=51,085) on patients with COPD and hypercapnia treated with NPPV (Wilson, 2020). Of these, 15 RCTs and 6 observational studies evaluated BPAP compared with no device. The BPAP modes were spontaneous/timed, volume-assured pressure support, pressure-controlled ventilation, or the mode was not specified. The primary outcomes were mortality, all-cause hospital admissions, intubation, and quality of life. Analysis indicated that overall, use of BPAP, compared with no device, was significantly associated with a lower risk of mortality (odds ratio [OR] 0.66), fewer patients with hospital admissions (OR 0.22), and lower need for intubation (OR 0.34). There was no significant difference in quality of life, and sensitivity analysis indicated that observational studies were driving the significant results. Further analysis of subgroups evaluated the timing of the initiation of NPPV (stable vs. recent exacerbation) and separated the PaCO2 threshold categories as 45 to 49 mmHg, 50 to 51 mmHg, and at least 52 mmHg. Post-hoc subgroup analysis found no significant differences in mortality or all-cause hospital admissions based on PaCO2 levels, but improved quality of life in patients with higher PaCO2 levels.
 
A 2020 evidence review by the American Thoracic Society evaluated NPPV on health outcomes in patients with stable hypercapnic COPD (Macrea, 2020). Studies varied in the severity of baseline hypercapnia and lung disease, mode of ventilation, pressure settings, and comparator, all of which may have contributed to the imprecision of the studies.
 
Chronic Hypercapnic Respiratory Failure
Kohnlein et al conducted a multicenter RCT to determine whether BPAP would improve survival in patients with COPD and stable hypercapnic respiratory failure when ventilator settings were targeted to reduce hypercapnia (Kohnlein, 2014). Patients (N=195) with GOLD stage IV COPD, PaCO2 51.9 mmHg or higher, and pH higher than 7.35 were randomized into treatment as usual (including O2), or BPAP plus treatment as usual. Excluded were patients with BMI of 35 kg/m2 or greater, abnormalities of the lung or thorax other than COPD, or other conditions resulting in hypercapnia. Titration was performed in the hospital over a mean of 5.6 days with pressure targeted to reduce the baseline PaCO2 by at least 20% or to achieve PaCO2 < 48.1 mmHg. The mean inspiratory pressure in the NPPV group was 21.6 cm H2O with a backup rate of 16.1. Patients in both groups were admitted to the hospital at 3, 6, 9, and 12 months after randomization to ensure optimized treatment and additionally contacted by telephone every 4 weeks to ensure adherence to therapy. At 12 months, the PaCO2 was 48.8 mmHg in the NPPV group (mean use of 5.9 hours per day) compared to 55.5 in the control group. The primary outcome of 1-year all cause mortality was 12% in the NPPV group compared to 33% in the control group, with a hazard ratio of 0.24 (95% CI: 0.11 to 0.49; p=.004). Emergency hospital admissions were limited to 3 patients (3%) in the control group. The study utilized intention-to-treat analysis with blinded outcomes assessors. Previous studies that had not shown a significant improvement in survival with NPPV included patients with mild hypercapnia who did not have reduced hypercapnia or improved blood gases with treatment (McEvoy, 2009).
 
Post-Acute Hypercapnic Respiratory Failure
Murphy et al reported an RCT on the use of BPAP in addition to home oxygen therapy in patients with persistent hypercapnia following acute respiratory failure in the Home Oxygen Therapy Home Mechanical Ventilation (HOT-HMV) trial (Murphy, 2017). Patients (N=116) who had persistent hypercapnia following hospitalization for an acute exacerbation of COPD were randomized to a BPAP device with home oxygen therapy (NPPV+O2) or to oxygen therapy alone (O2). Randomization occurred between 2 to 4 weeks after resolution of decompensated acidosis (arterial pH > 7.30) in patients who had persistent hypercapnia (PaCO2 > 53 mmHg) and hypoxemia (PaO2 < 55 mmHg or < 60 mmHg; greater than or equal to 1 of polycythemia, pulmonary hypertension, or cor pulmonale; > 30% of sleep time with oxygen saturation < 90% as measured by pulse oximetry) and arterial pH greater than 7.30 while breathing room air. Patients were admitted for inpatient titration with either the Harmony 2 ST (Philips-Respironics) or VPAP IIIST (ResMed) with pressure support and backup rate. Settings were adjusted during overnight sleep studies to control hypoventilation and hypoxemia with a median inspiratory positive airway pressure of 24 cm H2O. Respiratory failure was attributed to COPD if FEV1 was less than 50% predicted, the FEV1/FVC ratio was less than 60%, and the patient had a smoking history in the absence of 1) obesity (BMI > 35), 2) clinically significant obstructive sleep apnea, or 3) neuromuscular or chest wall disease. PaCO2 levels improved significantly more in the NPPV group at 6 weeks (between group difference of -5.0; 95% CI: -9.0 to -1.3) and 3 months (-4.0; 95% CI:-7.1 to -0.8) but not at 6 or 12 months. Improvements in PaO2 levels were not significantly different in the 2 groups. There was a significant reduction in the composite endpoint of readmission or death within 12 months (63.4% in the NPPV+O2 group vs. 80.4% in the O2 alone group), with an adjusted hazard ratio of 0.49 (95% CI: 0.31 to 0.77; p=.002) and a number needed to treat of 5.8 to prevent 1 readmission or death. The median time to readmission or death was 4.3 months in the NPPV+O2 group and 1.4 months in patients receiving oxygen alone, and the exacerbation rate in 12 months was reduced to 3.8 for the NPPV+O2 group compared to 5.1 with O2 alone (adjusted rate ratio 0.66; 95% CI: 0.46 to 0.95, p=.02). Twelve month mortality was not significantly different between the groups. The study utilized intention-to-treat analysis with blinded outcomes assessors.
 
Several differences were noted between the results of the HOT-HMV trial and the RESCUE trial, which failed to demonstrate an improvement in hospitalizations or mortality Struik, 2014). In the RESCUE trial there was less stringent PaCO2 criteria (>45 mmHg) and treatment began immediately following cessation of acute ventilation rather than after a 2 week stabilization period, so that patients with spontaneously reversible hypercapnia would have been included in the study. For example, in the HOT-HMV trial 21% of potential patients were excluded due to not meeting the PaCO2 criteria 2 weeks after the acute exacerbation. The Global Initiative for Chronic Obstructive Lung Disease (GOLD) notes that several factors may account for discrepancies in study results, including differences in patient selection, underpowered studies, NPPV settings, and poor adherence (GOLD, 2021).
 
Home Mechanical Ventilation
 
A technology assessment by the Agency for Healthcare Research and Quality (AHRQ, 2019) for the Centers for Medicare and Medicaid Services identified 5 studies on the initiation of HMV (Wilson, 2019). No studies were identified that compared initiation criteria for HMV versus BPAP. The AHRQ systematic review found low quality evidence that HMV compared to BPAP, CPAP, or no device was associated with significantly fewer hospital admissions. This was based on 2 observational studies that compared HMV to no device and a large study of administrative claims data by Vasquez et al in 2017 that compared HMV, BPAP, and CPAP.
 
In 2020 the AHRQ authors published a meta-analysis of RCTs and comparative observational studies of patients with COPD and hypercapnia treated with BPAP or HMV (Wison, 2020). The primary outcomes were mortality, all-cause hospital admissions, intubation, and quality of life. Based on 2 observational studies, HMV was significantly associated with fewer all-cause hospital admissions (rate ratio, 0.50; 95% CI, 0.35 to 0.71) compared with no device, but there was not a statistically significant difference in mortality (21.84% vs. 34.09%; OR, 0.56 (0.29 to 1.08). However, the evidence was low to moderate in quality and based on small numbers of studies.
 
Vasquez et al  performed a retrospective analysis of claims data of hospitalization in patients with COPD who did or did not receive therapy with CPAP, BPAP, or HMV (Vasquez, 2017). Differences in COPD-related hospitalization were compared from 6 months before the prescription of a device to 6 months after prescription of a device across the device types. Models were stratified by sleep disordered breathing, congestive heart failure, age less than or greater than 65 years, and chronic respiratory failure. There were 1,881,652 enrollees with at least 2 COPD-related claims; 28,774 were on BPAP, 112,119 on CPAP, and 1011 enrolled on HMV. After exclusion criteria (health plan coverage for 12 month before and 6 months after the index date) were applied, there were 39,385 patients on CPAP, 9,156 patients on BPAP, and 315 patients on HMV who were included in the analysis. Propensity matching was used to compare the NPPV groups to medication groups. Most patients (92.5%) were not receiving any form of NPPV. Continuous positive airway pressure was prescribed for 5.6% of patients, BPAP for 1.5% of patients, and HMV in less than 1% of patients. Most patients prescribed HMV were older and there was high geographic variability; a majority (59.1%) of HMV users resided in the south. A majority of patients prescribed CPAP or BPAP had co-morbid sleep-disordered breathing (57.4% and 59.1%, respectively), while HMV was prescribed most frequently in patients with acute respiratory failure (56.5%), hypoxemia (30.2%), and chronic respiratory failure (28.3%). Sleep disordered breathing was present in 20.3% of patients using HMV. Hospitalization rates were highest in the HMV group. While all forms of NPPV reduced hospitalization rates, HMV was associated most strongly with a reduction in hospitalizations (p<.001).
 
Supplemental Information
 
The purpose of the following information is to provide reference material. Inclusion does not imply endorsement or alignment with the evidence review conclusions.
 
Practice Guidelines and Position Statements
 
American Thoracic Society
 
Chronic Obstructive Pulmonary Disease
In 2020, the American Thoracic Society published an evidence-based clinical practice guideline on long-term non-invasive ventilation in chronic stable hypercapnic chronic obstructive pulmonary disease (COPD) (Macrea, 2020). The society included the recommendations, all of which were conditional due to moderate to very low certainty in the evidence base and are listed below.
 
American Thoracic Society Recommendations
Recommendation: "We suggest the use of nocturnal noninvasive ventilation (NIV) in addition to usual care for patients with chronic stable hypercapnic COPD."
Strength of Recommendation: Conditional
Level of Certainty: Moderate
 
Recommendation: "We suggest that patients with chronic stable hypercapnic COPD undergo screening for obstructive sleep apnea before initiation of long-term NIV."
Strength of Recommendation: Conditional
Level of Certainty: Very low
 
Recommendation: "We suggest not initiating long-term NIV during an admission for acute on-chronic hypercapnic respiratory failure, favoring instead reassessment for NIV at 2–4 weeks after resolution."
Strength of Recommendation: Conditional
Level of Certainty: Low
 
Recommendation: "We suggest not using an in-laboratory overnight polysomnogram (PSG) to titrate NIV in patients with chronic stable hypercapnic COPD who are initiating NIV."
Strength of Recommendation: Conditional
Level of Certainty: Very low
 
Recommendation: "We suggest NIV with targeted normalization of PaCO2 in patients with hypercapnic COPD on long-term NIV."
Strength of Recommendation: Conditional
Level of Certainty: Low
 
American College of Chest Physicians et al
In 2021, the American College of Chest Physicians, the American Association for Respiratory Care, the American Academy of Sleep Medicine, and the American Thoracic Society published a technical expert panel report on optimal noninvasive ventilation for COPD (Hill, 2021). The panel recommends that overnight oxygen saturation should not be part of the criteria for bilevel positive airway pressure (BPAP) and that home mechanical ventilators be considered when patients need any of the following:
 
    • "Higher inspiratory pressures than those deliverable by E0471
    • FIO2 higher than 40% or 5 L/min nasally
    • Ventilator support for 10 h per day or greater (ie, daytime use)
    • Both sophisticated alarms and accompanying internal battery (high-dependency patient)
    • Mouthpiece ventilation during the day
    • Persistence of hypercapnia with PaCO2 greater than or equal to 52 mm Hg despite adequate adherence to BPAP therapy"
 
The panel strongly recommended the use of respiratory therapists in the home for initiation and ongoing support for positive pressure ventilation with either BPAP or home ventilators.
 
National Institute for Health and Care Excellence Global
In 2019, the United Kingdom's National Institute for Health and Care Excellence (NICE) published a guideline for the diagnosis and management of COPD (Hill, 2021). NICE recommends that patients with COPD who have chronic hypercapnic respiratory failure despite adequate pharmacologic and oxygen therapy should be referred to a specialist center for consideration of long-term, non-invasive ventilation.
 
Global Initiative for Chronic Obstructive Pulmonary Disease
The Global Initiative for Chronic Obstructive Pulmonary Disease (GOLD) published a revised report for 2022 (GOLD, 2021). GOLD recommendations include:
 
    • "Pulmonary rehabilitation improves dyspnea, health status and exercise tolerance in stable patients (Evidence A)."
    • "Pulmonary rehabilitation reduces hospitalization among patients who have had a recent exacerbation (< 4 weeks from prior hospitalization)(Evidence B).
    • "In patients with severe resting hypoxemia long-term oxygen therapy is indicated (Evidence A)."
    • "In patients with stable COPD and moderate resting or exercise-induced arterial desaturation, prescription of long-term oxygen does not lengthen time to death or first hospitalization or provide sustained benefit in health status, lung function and 6-minute walk distance (Evidence A).
    • "In patients with severe chronic hypercapnia and a history of hospitalization for acute respiratory failure, long term non-invasive ventilation may be considered (Evidence: B)." Pronounced daytime persistent hypercapnia was reported as (PaCO2 greater than or equal to 52 mmHg).
 
Ongoing and Unpublished Clinical Trials
Some currently unpublished trials that might influence this review are listed below.
 
Ongoing
NCT01037387 Effect of the Noninvasive Mechanical Ventilation on the Daily Physical Activity and the Inflammatory Biomarkers in Stable Patients With COPD
Planned Enrollment: 50
Completion Date: December 2021
 
NCT02811588 Registry of Stable Hypercapnic Chronic Obstructive Pulmonary Disease Treated With Non-Invasive Ventilation Amendment: Home Tele-Monitoring of Non-Invasive Ventilation in Chronic Obstructive Pulmonary Disease
Planned Enrollment: 550
Completion Date: Jun 2023
 
NCT03647462 The Impact of Early Diagnosis and Treatment of OSA on Hospital Readmission in Hospitalized Chronic Obstructive Pulmonary Disease Patients: the COPD Readmit Clinical Trial
Planned Enrollment: 100
Completion Date: Apr 2025
 
NCT03221101 Home Non Invasive Ventilation Versus Long Term Oxygen Therapy Alone in COPD Survivors After Acute Hypercapnic Respiratory Failure. A French Multicenter Randomized Controlled Trial
Planned Enrollment: 86
Completion Date: Dec 2025
 
Unpublished
 
NCT01513655a Home Non Invasive Ventilation (NIV) Treatment for COPD-patients After a NIV-treated Exacerbation
Planned Enrollment: 150
Completion Date: July 2020 (unknown)
 
NCT03766542 Optimal Positive Airway Pressure in Overlap Syndrome: a Randomized Controlled Trial
Planned Enrollment: 70
Completion Date: Sep 2020 (unknown)
 
2023 Update
Annual policy review completed with a literature search using the MEDLINE database through January 2023. No new literature was identified that would prompt a change in the coverage statement.
 
2024 Update
Annual policy review completed with a literature search using the MEDLINE database through January 2024. No new literature was identified that would prompt a change in the coverage statement. The key identified literature is summarized below.
 
A Cochrane systematic review by Annane et al identified 10 clinical trials (N=173) evaluating nocturnal mechanical ventilation (volumecycled mechanical ventilation or BPAP) in patients with hypoventilation due to neuromuscular or chest wall disorders (Annane, 2014). The included trials enrolled patients with motor neuron disease (n=3), chest wall deformity (n=3), Duchenne muscular dystrophy (n=1), or mixed populations (n=3). Trials comparing mechanical ventilation to standard of care or those evaluating different methods of ventilation were included. Only 2 trials evaluated HMV; thus, the majority of the evidence is for BPAP. The largest study (N=70) was conducted specifically in patients with Duchenne muscular dystrophy. Although the quality of the studies was low, nocturnal mechanical ventilation lowered mortality among patients with neuromuscular or chest wall disorders.
 
Other systematic reviews have included only observational studies. AlBalawi et al evaluated long-term non-invasive ventilation in children with neuromuscular disease (AlBalawi, 2022). A total of 57 studies in 1948 children were identified. Neuromuscular diseases were classified as spinal muscular atrophy, Duchenne muscular dystrophy, or other/multiple neuromuscular diseases. Methods of ventilation were either not reported or specific to BPAP. Overall, NPPV resulted in lower mortality compared with standard care (risk ratio, 0.30; 95% CI, 0.19 to 0.46; p<.00001; I2=68%), but mortality was not significantly improved compared with invasive mechanical ventilation (risk ratio, 0.91; 95% CI, 0.24 to 3.48; p=.89; I2=88%).
 
A technology assessment by the AHRQ (2019) for CMS identified 3 observational studies on the initiation of HMV in patients with neuromuscular disease (Wilson, 2019). One study (N=144) compared HMV versus BPAP in patients with amyotrophic lateral sclerosis (ALS) (Sancho, 2014). The other studies were not relevant to this review of evidence.
 
Sancho et al retrospectively compared BPAP with volume-cycled ventilation in patients with ALS (Sancho, 2014). A total of 144 patients were identified with 82 receiving BPAP and 62 receiving HMV. No differences in survival were found between groups (median 15.00 months in both groups; p=.533). More patients had effective ventilation (gas exchange and hypoventilation symptoms) with HMV than BPAP (72.41% vs. 48.78%; p<.001).
 
A technology assessment by the AHRQ for CMS identified 1 trial comparing CPAP to no device in patients with OHS and OSA (Wilson, 2019). This study by Masa et al is summarized below (Masa, 2015).
 
Masa et al conducted a series of open-label, multicenter RCTs evaluating NPPV in patients with OHS (Masa, 2015; Masa, 2016; Masa, 2019; Masa 2020). The initial study, Masa et al, enrolled patients with concomitant OHS and OSA. Patients (N=221) were randomized to NPPV (as mixed mechanical ventilation/BPAP), CPAP, or lifestyle modification (Masa, 2015). Lifestyle modifications included a low-calorie diet, sleep hygiene, and avoidance of alcohol or tobacco. NPPV was administered throughout the entire sleep period at bilevel pressure. Daytime use was to be adjusted to normal oxygen saturation (if possible). Both CPAP and NPPV resulted in greater improvements in PaCO2 compared with lifestyle interventions, but no significant difference in PaCO2 was found between CPAP and NPPV. Other outcomes including HRQL, 6MWD, and spirometry had greater improvement with NPPV than CPAP. The trial was limited by a short follow-up duration of 2 months and lack of comparative risk calculations. After this 2 month trial, patients were enrolled in the long-term extension trial (Masa, 2019). Patients treated with only lifestyle interventions were re-randomized to NPPV or CPAP. At a median follow up of 5.44 years, patients treated with NPPV had lower mean hospitalization days compared with CPAP treatment, but the difference was nonsignificant. Mortality was also similar between groups (15% with CPAP vs. 11% with NPPV; hazard ratio [HR], 0.92, 95% CI, 0.36 to 1.87; p=.631).
 
In 2016, Masa et al published a trial conducted in patients with OHS but without severe OSA (Masa, 2016). Patients (N=86) were randomized to BPAP or lifestyle modification. Patients treated with BPAP had greater improvement in PaCO2 than those who received lifestyle modification. Daytime sleepiness and some health-related quality of life parameters were also improved. Similar to the 2015 study previously described, the follow-up was limited to 2 months (Masa, 2015; Masa, 2016). Long-term outcomes were also published by Masa et al in patients (N=98) without severe OSA (Masa, 2020). Compared with control, BPAP did not significantly reduce mean hospitalization days per year, cardiovascular events, or mortality but did improve PaCO2, quality of life, and daytime sleepiness at a median follow-up of 4.98 years.
 
Howard et al compared CPAP and BPAP for initial treatment in patients with OHS and severe OSA in a double-blind RCT (Howard, 2017). A total of 57 patients were treated for 3 months. Outcomes were similar between groups, but the trial is limited by the small sample size and short duration of follow-up prohibiting any conclusions regarding differences in mortality.
 
Arellano-Maric et al conducted a prospective observational trial to determine whether patients with OHS and OSA could be switched from NPPV to CPAP (Arellano-Maric, 2020). Patients were recruited from the sleep units of 3 hospitals in Germany. All patients had received successful NPPV (PaCO2 45 mm Hg and <20% total sleep time with oxygen saturation <20%) for at least 3 months. Those patients who were successfully switched to CPAP (n=37) continued CPAP for 4 to 6 weeks. Five patients who did not meet criteria for CPAP switch requested to be switched to CPAP for a total sample size of 42 patients. During the study period, 30 of 42 patients (71%) maintained daytime PaCO2 45 mm Hg. Quality of life, sleep parameters, and lung function were similar after the switch to CPAP. The authors concluded that CPAP is a feasible alternative to NPPV in patients with concomitant OHS and OSA.
 
Multiple systematic reviews have evaluated various NPPV methods in patients with hypoventilation syndromes. Xu et al conducted a network meta-analysis evaluating various NPPV methods (CPAP and BPAP) in patients with OHS (Xu, 2022). Trials enrolled obese patients (BMI 30 mg/m2) with hypercapnia (PaCO2 45 mmHg) who had no other causes for the hypercapnia. BPAP-average volume-assured pressure support (AVAPS) and BPAP-spontaneous timed (ST) pressure support were the most effective methods for improving sleep and PaCO2.
 
Afshar et al conducted a systematic review of 25 studies (3 RCTs) evaluating NPPV (CPAP and BPAP) in patients with OHS (Afshar, 2020). 3 RCTS were included in the analysis (the 12 nonrandomized comparative studies and 10 studies without a comparator group are not included). Overall, NPPV was associated with improved mortality, daytime sleepiness, and hospital visits; however, the evidence had low to very low certainty and was considered very low quality.
 
The evidence for NPPV in patients with hypoventilation syndromes unrelated to obesity is limited to case reports and case series primarily in patients with congenital central hypoventilation. In some cases, NPPV may avoid the need for invasive mechanical ventilation (Kam, 2014; Yang, 2022; Xu, 2022).
 
In 2023, the American College of Chest Physicians (ACCP) published clinical practice guidelines for respiratory management of patients with neuromuscular weakness (Khan, 2023). Most evidence is based on observational data from patients with amyotrophic lateral sclerosis. The guidelines recommend non-invasive ventilation (NIV) for patients with neuromuscular disease and chronic respiratory failure for patients who meet the following pulmonary function test criteria:
 
    • Forced vital capacity (FVC) <80% predicted with symptoms or FVC <50% predicted without symptoms;
    • Maximum inspiratory pressure (MIP) <60 cm H2O or maximum expiratory pressure (MEP) <40 cm H2O;
    • Peak cough flow (PCF) <270 L/min for age 12 years or PCF <5th percentile for age <12 years;
    • Sniff nasal inspiratory pressure (SNIP) <70 cm H2O in male patients, SNIP <60 cmH2O in female patients for age 12 years.
 
The panel found no strong evidence to support one method of NIV over another.
 
In 2021, the ACCP, the American Association for Respiratory Care, the American Academy of Sleep Medicine, and the American Thoracic Society published a technical expert panel report on optimal NIV for chronic obstructive pulmonary disease (COPD), thoracic restrictive disorders, and hypoventilation syndromes (Hill, 2021: Wolfe, 2021; Mokhlesi, 2021).
 
For COPD the panel recommends that overnight oxygen saturation should not be part of the criteria for bilevel positive airway pressure (BPAP) and that home mechanical ventilators be considered when patients need any of the following (Hill, 2021):
 
    • "Higher inspiratory pressures than those deliverable by E0471,
    • FIO2 [fraction of inspired oxygen] higher than 40% or 5 L/min nasally,
    • Ventilator support for 10 h per day or greater (i.e., daytime use),
    • Both sophisticated alarms and accompanying internal battery (high-dependency patient),
    • Mouthpiece ventilation during the day,
    • Persistence of hypercapnia with PaCO2 [arterial blood carbon dioxide] greater than or equal to 52 mm Hg despite adequate adherence to BPAP therapy"
    • The panel strongly recommended the use of respiratory therapists in the home for initiation and ongoing support for positive pressure ventilation with either BPAP or home ventilators.
 
For thoracic restrictive disorders, the panel recommends BPAP for patients with any of the following (Wolfe, 2021):
 
    • "Spirometry (upright or supine) with vital capacity less than 50% predicted or less than 80% predicted with associated symptoms (i.e., orthopnea, dyspnea, morning headaches, excessive daytime sleepiness, or unrefreshing sleep),
    • Force testing with maximal inspiratory pressure less than 60 cm H2O,
    • Hypercapnia:
        • Chronic stable daytime (awake) hypercapnia with PaCO2 greater than 45 mmHg,
        • Venous blood gas PCO2, end-tidal PCO2, or transcutaneous PCO2, less than 50 mmHg, or
    • Hypoxia:
        • Overnight oximetry in-laboratory or home sleep test with saturation less than 88% for 5 minutes."
 
Home mechanical ventilation is recommended in patients with vital capacity less than 30% or if BPAP fails.
 
For patients with hypoventilation syndromes who are obese the recommendations include (Mokhlesi, 2021):
 
    • BPAP (spontaneous/timed) or volume-assured pressure support (VAPS) for those who are discharged from the hospital, for those with obesity hypoventilation syndrome (OHS) without obstructive sleep apnea, and for those who have failed continuous positive airway pressure (CPAP).
 
For patients with hypoventilation syndromes due to reduced respiratory drive or advanced lung disease that is not COPD, BPAP (spontaneous/timed) or VAPS is recommended. Patients with hypoventilation syndromes who fail BPAP/VAPS should receive home mechanical ventilation.
 
In 2019, the American Thoracic Society published a clinical practice guideline on OHS (Mokhlesi). These guidelines recommend positive airway pressure for patients with OHS. Generally, CPAP is recommended over other NIV because the majority (greater than 70%) of patients have concomitant obstructive sleep apnea (OSA). The guidelines do recommend non-invasive positive airway pressure ventilation (NPPV) initiation at discharge for patients hospitalized with respiratory failure suspected of having OHS until they undergo outpatient workup and titration of positive airway pressure therapy. Both recommendations were conditional with very low level of certainty in the evidence.
 
2024 Update
Annual policy review completed with a literature search using the MEDLINE database through March  2024. No new literature was identified that would prompt a change in the coverage statement. The key identified literature is summarized below.
 
A systematic review was conducted of 5 observational studies evaluating CPAP in patients with overlap syndrome (Srivali, 2023). Definitions of OSA, COPD, and CPAP use were inconsistent among the studies, and the data were not pooled. However, all-cause mortality was reported in 3 studies and was significantly reduced with CPAP in each study (hazard ratios,0.19 to 0.71).

CPT/HCPCS:
E0466Home ventilator, any type, used with non invasive interface, (e.g., mask, chest shell)
E0467Home ventilator, multi function respiratory device, also performs any or all of the additional functions of oxygen concentration, drug nebulization, aspiration, and cough stimulation, includes all accessories, components and supplies for all functions
E0468Home ventilator, dual-function respiratory device, also performs additional function of cough stimulation, includes all accessories, components and supplies for all functions
E0470Respiratory assist device, bi level pressure capability, without backup rate feature, used with noninvasive interface, e.g., nasal or facial mask (intermittent assist device with continuous positive airway pressure device)
E0471Respiratory assist device, bi level pressure capability, with back up rate feature, used with noninvasive interface, e.g., nasal or facial mask (intermittent assist device with continuous positive airway pressure device)
E0601Continuous positive airway pressure (cpap) device

References: Afshar M, Brozek JL, Soghier I, et al.(2020) The Role of Positive Airway Pressure Therapy in Adults with Obesity Hypoventilation Syndrome. A Systematic Review and Meta-Analysis. Ann Am Thorac Soc. Mar 2020; 17(3): 344-360. PMID 31726017

AlBalawi MM, Castro-Codesal M, Featherstone R, et al.(2022) Outcomes of Long-Term Noninvasive Ventilation Use in Children with Neuromuscular Disease: Systematic Review and Meta-Analysis. Ann Am Thorac Soc. Jan 2022; 19(1): 109-119. PMID 34181865

Annane D, Orlikowski D, Chevret S.(2014) Nocturnal mechanical ventilation for chronic hypoventilation in patients with neuromuscular and chest wall disorders. Cochrane Database Syst Rev. Dec 13 2014; 2014(12): CD001941. PMID 25503955

Arellano-Maric MP, Hamm C, Duiverman ML, et al.(2020) Obesity hypoventilation syndrome treated with non-invasive ventilation: Is a switch to CPAP therapy feasible?. Respirology. Apr 2020; 25(4): 435-442. PMID 31597227

Carmona H, Graustein AD, Benditt JO.(2023) Chronic Neuromuscular Respiratory Failure and Home Assisted Ventilation. Annu Rev Med. Jan 27 2023; 74: 443-455. PMID 36706747

Gay PC, Owens RL, Gay PC, et al.(2021) Executive Summary: Optimal NIV Medicare Access Promotion: A Technical Expert Panel Report From the American College of Chest Physicians, the American Association for Respiratory Care, the American Academy of Sleep Medicine, and the American Thoracic Society. Chest. Nov 2021; 160(5): 1808-1821. PMID 34339685

Global Initiative for Chronic Obstructive Lung Disease (GOLD).(2021) Global Strategy for the Diagnosis, Management and Prevention of Chronic Obstructive Pulmonary Disease: 2022 Report. www.goldcopd.org. Accessed November 29, 2021.

Global Initiative for Chronic Obstructive Lung Disease (GOLD).(2023) Global Strategy for Prevention, Diagnosis, and Management of COPD: 2023 Report. www.goldcopd.org. Accessed May 15, 2023.

Hill NS, Criner GJ, Branson RD, et al.(2021) Optimal NIV Medicare Access Promotion: Patients With COPD: A Technical Expert Panel Report From the American College of Chest Physicians, the American Association for Respiratory Care, the American Academy of Sleep Medicine, and the American Thoracic Society. Chest. Nov 2021; 160(5): e389-e397. PMID 34339684

Howard ME, Piper AJ, Stevens B, et al.(2017) A randomised controlled trial of CPAP versus non-invasive ventilation for initial treatment of obesity hypoventilation syndrome. Thorax. May 2017; 72(5): 437-444. PMID 27852952

Jaoude P, Kufel T, El-Solh AA.(2014) Survival benefit of CPAP favors hypercapnic patients with the overlap syndrome. Lung. Apr 2014; 192(2): 251-8. PMID 24452812

Kam K, Bjornson C, Mitchell I.(2014) Congenital central hypoventilation syndrome; safety of early transition to non-invasive ventilation. Pediatr Pulmonol. Apr 2014; 49(4): 410-3. PMID 23843332

Khan A, Frazer-Green L, Amin R, et al.(2023) Respiratory Management of Patients With Neuromuscular Weakness: An American College of Chest Physicians Clinical Practice Guideline and Expert Panel Report. Chest. Mar 13 2023. PMID 36921894

Kohnlein T, Windisch W, Kohler D, et al.(2014) Non-invasive positive pressure ventilation for the treatment of severe stable chronic obstructive pulmonary disease: a prospective, multicentre, randomised, controlled clinical trial. Lancet Respir Med. Sep 2014; 2(9): 698-705. PMID 25066329

Machado MC, Vollmer WM, Togeiro SM, et al.(2010) CPAP and survival in moderate-to-severe obstructive sleep apnoea syndrome and hypoxaemic COPD. Eur Respir J. Jan 2010; 35(1): 132-7. PMID 19574323

Macrea M, Oczkowski S, Rochwerg B, et al.(2020) Long-Term Noninvasive Ventilation in Chronic Stable Hypercapnic Chronic Obstructive Pulmonary Disease. An Official American Thoracic Society Clinical Practice Guideline. Am J Respir Crit Care Med. Aug 15 2020; 202(4): e74-e87. PMID 32795139

Marin JM, Soriano JB, Carrizo SJ, et al.(2010) Outcomes in patients with chronic obstructive pulmonary disease and obstructive sleep apnea: the overlap syndrome. Am J Respir Crit Care Med. Aug 01 2010; 182(3): 325-31. PMID 20378728

Martinez-Pitre PJ, Sabbula BR, Cascella M.(2022) Restrictive Lung Disease. In: StatPearls. Treasure Island (FL): StatPearls Publishing; July 25, 2022.

Masa JF, Benítez I, Sánchez-Quiroga MÁ, et al.(2020) Long-term Noninvasive Ventilation in Obesity Hypoventilation Syndrome Without Severe OSA: The Pickwick Randomized Controlled Trial. Chest. Sep 2020; 158(3): 1176-1186. PMID 32343963

Masa JF, Corral J, Alonso ML, et al.(2015) Efficacy of Different Treatment Alternatives for Obesity Hypoventilation Syndrome. Pickwick Study. Am J Respir Crit Care Med. Jul 01 2015; 192(1): 86-95. PMID 25915102

Masa JF, Corral J, Caballero C, et al.(2016) Non-invasive ventilation in obesity hypoventilation syndrome without severe obstructive sleep apnoea. Thorax. Oct 2016; 71(10): 899-906. PMID 27406165

Masa JF, Mokhlesi B, Benítez I, et al.(2019) 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. Lancet. Apr 27 2019; 393(10182): 1721-1732. PMID 30935737

Mathews AM, Wysham NG, Xie J, et al.(2020) Hypercapnia in Advanced Chronic Obstructive Pulmonary Disease: A Secondary Analysis of the National Emphysema Treatment Trial. Chronic Obstr Pulm Dis. Oct 2020; 7(4): 336-345. PMID 32877962

McConville JF, Solway J, Mokhlesi B.(2022) Disorders of Ventilation. In: Loscalzo J, Fauci A, Kasper D, Hauser S, Longo D, Jameson J. eds. Harrison's Principles of Internal Medicine, 21e. McGraw Hill; 2022. Accessed May 10, 2023. https://accessmedicine.mhmedical.com/content.aspx?bookid=3095§ionid=265457022

McEvoy RD, Pierce RJ, Hillman D, et al.(2009) Nocturnal non-invasive nasal ventilation in stable hypercapnic COPD: a randomised controlled trial. Thorax. Jul 2009; 64(7): 561-6. PMID 19213769

Mokhlesi B, Masa JF, Brozek JL, et al.(2019) Evaluation and Management of Obesity Hypoventilation Syndrome. An Official American Thoracic Society Clinical Practice Guideline. Am J Respir Crit Care Med. Aug 01 2019; 200(3): e6-e24. PMID 31368798

Mokhlesi B, Won CH, Make BJ, et al.(2021) Optimal NIV Medicare Access Promotion: Patients With Hypoventilation Syndromes: A Technical Expert Panel Report From the American College of Chest Physicians, the American Association for Respiratory Care, the American Academy of Sleep Medicine, and the American Thoracic Society. Chest. Nov 2021; 160(5): e377-e387. PMID 34339686

Murphy PB, Rehal S, Arbane G, et al.(2017) 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. Jun 06 2017; 317(21): 2177-2186. PMID 28528348

National Institute for Health and Care Excellence (NICE).(2021) Chronic obstructive pulmonary disease in over 16s: diagnosis and management [NG115]. Uidance/ng115. Accessed November 29, 2021. 2019 https://www.nice.org.uk/g

Noninvasive Positive Pressure Ventilation in the Home. Project ID: PULT0717 (Prepared by the Mayo Clinic Evidence-Based Practice Center under Contract No. HHSA290201500013I_HHSA29032004T). Rockville, MD: Agency for Healthcare Research and Quality. March 2019. https://www.ahrq.gov/sites/default/files/wysiwyg/research/findings/ta/hmv/hmv-ta-fullreport.pdf.

Orr JE, Azofra AS, Tobias LA.(2020) Management of Chronic Respiratory Failure in Chronic Obstructive Pulmonary Disease: High-Intensity and Low-Intensity Ventilation. Sleep Med Clin. Dec 2020; 15(4): 497-509. PMID 33131660

Raveling T, Vonk J, Struik FM, et al.(2021) Chronic non-invasive ventilation for chronic obstructive pulmonary disease. Cochrane Database Syst Rev. Aug 09 2021; 8: CD002878. PMID 34368950

Sancho J, Servera E, Morelot-Panzini C, et al.(2014) Non-invasive ventilation effectiveness and the effect of ventilatory mode on survival in ALS patients. Amyotroph Lateral Scler Frontotemporal Degener. Mar 2014; 15(1-2): 55-61. PMID 24266679

Singh G, Agarwal A, Zhang W, et al.(2019) Impact of PAP therapy on hospitalization rates in Medicare beneficiaries with COPD and coexisting OSA. Sleep Breath. Mar 2019; 23(1): 193-200. PMID 29931497

Struik FM, Sprooten RT, Kerstjens HA, et al.(2014) Nocturnal non-invasive ventilation in COPD patients with prolonged hypercapnia after ventilatory support for acute respiratory failure: a randomised, controlled, parallel-group study. Thorax. Sep 2014; 69(9): 826-34. PMID 24781217

Vasquez MM, McClure LA, Sherrill DL, et al.(2017) Positive Airway Pressure Therapies and Hospitalization in Chronic Obstructive Pulmonary Disease. Am J Med. Jul 2017; 130(7): 809-818. PMID 28089799

Wiles SP, Aboussouan LS, Mireles-Cabodevila E.(2020) Noninvasive positive pressure ventilation in stable patients with COPD. Curr Opin Pulm Med. Mar 2020; 26(2): 175-185. PMID 31895118

Wilson M, Wang Z, Dobler C, et al.(2019) Noninvasive Positive Pressure Ventilation in the Home. Project ID: PULT0717 (Prepared by the Mayo Clinic Evidence-Based Practice Center under Contract No. HHSA290201500013I_HHSA29032004T). Rockville, MD: Agency for Healthcare Research and Quality. March 2019. https://www.ahrq.gov/sites/default/files/wysiwyg/research/findings/ta/hmv/hmv-ta-fullreport.pdf. Accessed June 5, 2023.

Wilson ME, Dobler CC, Morrow AS, et al.(2020) Association of Home Noninvasive Positive Pressure Ventilation With Clinical Outcomes in Chronic Obstructive Pulmonary Disease: A Systematic Review and Meta-analysis. JAMA. Feb 04 2020; 323(5): 455-465. PMID 32016309

Wolfe LF, Benditt JO, Aboussouan L, et al.(2021) Optimal NIV Medicare Access Promotion: Patients With Thoracic Restrictive Disorders: A Technical Expert Panel Report From the American College of Chest Physicians, the American Association for Respiratory Care, the American Academy of Sleep Medicine, and the American Thoracic Society. Chest. Nov 2021; 160(5): e399-e408. PMID 34339688

Xu J, Wei Z, Li W, et al.(2022) Effect of different modes of positive airway pressure treatment on obesity hypoventilation syndrome: a systematic review and network meta-analysis. Sleep Med. Mar 2022; 91: 51-58. PMID 35272117

Yang L, Qiu S, Zhong J, et al.(2022) Noninvasive ventilation via bilevel positive airway pressure improved sleep in a child with congenital central hypoventilation syndrome: A case report. Clin Case Rep. Oct 2022; 10(10): e6320. PMID 36276908


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