Treatment algorithm

Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups: see disclaimer

ACUTE

obesity hypoventilation syndrome

1st line – nocturnal ventilation

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ACUTE
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obesity hypoventilation syndrome
1st line – 

nocturnal ventilation

Continuous positive airway pressure (CPAP) may be used as an initial treatment of obesity hypoventilation syndrome (OHS), because most patients with OHS have associated obstructive sleep apnoea.[40] There are reports of successful treatment of OHS with CPAP, usually requiring pressures of 12 to 14 cm H₂O.[13][53][54][55][56][57][58][59]​ However, there are reports of failure with CPAP therapy when used alone.[53][55][60][61][62]

Bi-level positive airway pressure, with individually adjusted inspiratory positive airway pressure and expiratory positive airway pressure, is probably the most effective non-invasive treatment for reversing the hypercapnia associated with OHS.[13][51][63][64]​​​ In addition, use of bi-level positive airway pressure results in better respiratory function improvement compared with CPAP and is associated with greater positive airway pressure adherence when compared to CPAP therapy.[69][70]

Most studies have demonstrated that the differential between inspiratory positive airway pressure and expiratory positive airway pressure must be at least 8 to 10 cm H₂O to correct the hypercapnia and hypoxaemia on a long-term basis with bi-level positive airway pressure therapy.[13][66][67][68][69]

Nocturnal invasive mechanical ventilation by tracheostomy can be used effectively in patients with severe obesity hypoventilation syndrome who have not been able to tolerate or have had unsuccessful treatment with non-invasive forms of positive airway pressure therapy.

Plus – weight-reduction measures

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ACUTE
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obesity hypoventilation syndrome
Plus – 

weight-reduction measures

Treatment recommended for ALL patients in selected patient group

Weight reduction, including diet or the use of gastric bypass surgery, has been shown to be effective.[39]

Many of these patients with obesity hypoventilation syndrome require positive airway pressure therapy following surgery until they have lost a significant amount of weight.[77]

Consider – nocturnal oxygen therapy

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ACUTE
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obesity hypoventilation syndrome
Consider – 

nocturnal oxygen therapy

Additional treatment recommended for SOME patients in selected patient group

Oxygen therapy should not be used alone in patients with obesity hypoventilation syndrome (OHS).[71][72]​ However, approximately half of patients with OHS require the addition of oxygen to some form of positive airway pressure therapy.[13][53][70]​​[73][74]​​

Oxygen therapy is added when bi-level has been titrated but there is residual oxygen desaturation in the absence of obstructive apnoeas and hypopnoeas.[51]

restrictive thoracic disorders

1st line – nocturnal bi-level positive airway pressure or volume-cycled ventilation

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ACUTE
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restrictive thoracic disorders
1st line – 

nocturnal bi-level positive airway pressure or volume-cycled ventilation

In patients with neuromuscular and chest wall diseases, the use of nocturnal ventilation has been associated with improved survival, sleep quality, daytime gas exchange, and daytime function and with decreased daytime sleepiness.[78][79][80][81]

Amyotrophic lateral sclerosis has become the most common restrictive thoracic disorder to be prescribed non-invasive ventilation (NIV), which reportedly improves survival and quality of life, and reduces decline in forced vital capacity.[82][83][84]​​​

NIV using either bi-level positive airway pressure or a volume-cycled ventilator is preferred, with the latter able to generate larger tidal volumes than the standard bi-level positive airway pressures that have a maximum inspiratory positive airway pressure of 30 cm H₂O. Settings should be titrated in a sleep centre or in a controlled setting such as hospital, or, at times, in the patient's home. With positive airway pressure therapy, both inspiratory positive airway pressure and expiratory positive airway pressure should be increased together until all apnoeas and hypopnoeas are resolved, followed by continued increases in inspiratory positive airway pressure to correct the hypoxaemia related to alveolar hypoventilation.[51]

Plus – lung volume recruitment + airway clearance techniques

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ACUTE
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restrictive thoracic disorders
Plus – 

lung volume recruitment + airway clearance techniques

Treatment recommended for ALL patients in selected patient group

Patients with neuromuscular diseases and hypoventilation may benefit from lung volume recruitment (LVR) (e.g., glossopharyngeal breathing or breath stacking using a handheld resuscitation bag or mouthpiece) and airway clearance (e.g., manually assisted cough techniques).[45]

It may be necessary to add regular mechanical insufflation-exsufflation (cough assist device) for continued reduced cough effectiveness or high-frequency chest wall oscillation, with or without cough assistance or LVR, for patients with continued difficulties clearing secretions.[45]

2nd line – nocturnal invasive mechanical ventilation via tracheostomy

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ACUTE
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restrictive thoracic disorders
2nd line – 

nocturnal invasive mechanical ventilation via tracheostomy

Nocturnal invasive mechanical (NIV) ventilation by tracheostomy often becomes necessary in patients intolerant of NIV, including those with extended daytime use, worsening bulbar function, frequent aspiration, insufficient cough, episodes of chest infection despite adequate secretion management, and declining lung function.[45]

Plus – lung volume recruitment + airway clearance techniques

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ACUTE
|
restrictive thoracic disorders
Plus – 

lung volume recruitment + airway clearance techniques

Treatment recommended for ALL patients in selected patient group

Patients with neuromuscular diseases and hypoventilation may benefit from lung volume recruitment (LVR) (e.g., glossopharyngeal breathing or breath stacking using a handheld resuscitation bag or mouthpiece) and airway clearance (e.g., manually assisted cough techniques).[45] 

It may be necessary to add regular mechanical insufflation-exsufflation (cough assist device) for continued reduced cough effectiveness or high-frequency chest wall oscillation, with or without cough assistance or LVR, for patients with continued difficulties clearing secretions.[45]

Cheyne-Stokes respiration

1st line – nocturnal non-invasive ventilation + treatment of underlying disorder

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ACUTE
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Cheyne-Stokes respiration
1st line – 

nocturnal non-invasive ventilation + treatment of underlying disorder

Continuous positive airway pressure (CPAP) therapy has been shown to decrease the central apnoea-hypopnoea index in patients with Cheyne-Stokes respiration (CSR) due to congestive heart failure (CHF), both after short-term use and after periods of 1 to 3 months.[8][85][86][87][88][89][90][91]​​ By increasing intrathoracic pressure and decreasing the transmural pressure across the left ventricle, CPAP decreases left ventricular afterload, leading to an improvement in cardiac output.[13]

Bi-level positive airway pressure ventilation allows the individual adjustment of the inspiratory positive airway pressure and expiratory positive airway pressure, and when set with a back-up rate, ensures ventilation during central apnoeic episodes. When compared with CPAP, both forms of therapy equally decreased the baseline apnoea-hypopnoea index and improved sleep quality and daytime fatigue.[86]

Another form of non-invasive positive pressure ventilation, referred to as adaptive servo-ventilation (ASV), has been evaluated in the treatment of CSR. ASV provides a baseline degree of ventilatory support on top of an end-expiratory pressure and a default back-up rate.[94][95]​ However, a large, end point-driven study demonstrated a higher all-cause mortality in patients receiving ASV compared with the control group. As a result, ASV is not recommended in patients with CHF and a left ventricular ejection fraction ≤45% at this time until further analysis of the study is performed and the results from other ongoing trials are completed.[97] Using a flow-targeted ASV device, an ongoing trial has reported preliminary data showing increased hours of use each night and increased compliance at one year as compared to those patients in the prior negative study.[98] In addition, there was no noted increase in mortality at one year in the patients treated with ASV. Final results and recommendations await the completion of this multicenter trial.

Choice of methods is according to physician preference. Practice parameters have been published to help guide physicians in regards to treatment options for CSR.[107]

Patients with CSR due to CHF should also receive treatment for CHF.

Plus – nocturnal oxygen therapy

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ACUTE
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Cheyne-Stokes respiration
Plus – 

nocturnal oxygen therapy

Treatment recommended for ALL patients in selected patient group

Nocturnal oxygen therapy has been shown to significantly decrease the apnoea-hypopnoea index, both acutely and after more prolonged therapy, in patients with Cheyne-Stokes respiration (CSR) due to CHF.[8][17][57][60][61][62][99][100][101][102]

While oxygen therapy has been shown to decrease the apnoea-hypopnoea index, no study has demonstrated an improvement in left ventricular function in patients with CSR and CHF.[88]​[99]​​[102]

COPD

1st line – nocturnal bi-level positive airway pressure + treatment of underlying disorder

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ACUTE
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COPD
1st line – 

nocturnal bi-level positive airway pressure + treatment of underlying disorder

The use of non-invasive positive pressure ventilation has been shown to be beneficial both during an acute exacerbation of COPD and in selected groups of patients with stable chronic emphysema.[108][109][110][111][114]​ Nocturnal non-invasive ventilation has been shown to acutely improve sleep quality without an associated improvement in nocturnal gas exchange in a group of stable hypercapnic patients with COPD, suggesting that factors other than improvement in gas exchange, such as unloading inspiratory muscles or effects on central drive, might play a role.[109]

Bi-level positive airway pressure can be initiated with most patients requiring an inspiratory positive airway pressure to expiratory positive airway pressure differential of at least 8 to 10 cm H₂O to have effective ventilation. Higher expiratory positive airway pressures may be needed in those patients with the overlap syndrome where there is co-existent obstructive sleep apnoea. Otherwise, most patients may do well with an expiratory positive airway pressure of 5 cm H₂O, which is required to take up the dead space of the tubing and mask and allow effective sensing of an inspiratory effort. Excessive inspiratory positive airway pressures are associated with increasing air leaks and less effective ventilation. However, there is great variability in pressure requirements among patients.[118]

Patients should also receive treatment for COPD.

Consider – nocturnal oxygen

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ACUTE
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COPD
Consider – 

nocturnal oxygen

Additional treatment recommended for SOME patients in selected patient group

The hypoxaemia that develops in patients with alveolar hypoventilation most commonly is associated with hypercapnia. Thus, supplemental oxygen must be given with caution to these patients. In patients with COPD and hypoxaemia, continuous low-flow oxygen has been shown to significantly affect mortality.[116] Yet, the use of nocturnal oxygen in COPD patients with REM-associated nocturnal oxygen desaturation has been shown to decrease pulmonary hypertension, but has no significant effect on mortality.[117] Nocturnal non-invasive ventilation combined with oxygen was shown to lower PaCO₂ and improve quality of life after two years in hypercapnic COPD patients when compared with oxygen therapy alone, as well as demonstrating an improvement in survival.[111][113]​​[114]

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Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups. See disclaimer

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