Nocturnal mechanical ventilation may improve chronic hypoventilation symptoms and survival in neuromuscular diseases

Annane D, Orlikowski D, Chevret S, Chevrolet JC, Raphaël JC

Published Online:

July 8, 2009

The development of weakness of respiratory muscles or alterations in breathing control is a major complication of neuromuscular and chest wall disorders. Mechanical ventilation can compensate for the respiratory deficiency and is therefore widely proposed to people with neuromuscular disease who develop chronic hypoventilation. The review of eight randomised controlled trials involving 144 participants found that nocturnal mechanical ventilation may relieve chronic hypoventilation related symptoms and prolong survival. However, the quality of the studies was poor, and the benefit of long-term mechanical ventilation should be confirmed in further trials.

Abstract (click to read)

Background:

Chronic alveolar hypoventilation is a common complication of many neuromuscular and chest wall disorders. Long-term nocturnal mechanical ventilation is increasingly used to treat it.

Objectives:

To examine the efficacy of nocturnal mechanical ventilation in relieving hypoventilation related symptoms and in prolonging survival in people with neuromuscular or chest wall disorders.

Search strategy:

We searched the Cochrane Neuromuscular Disease Group Trials Register, MEDLINE (from January 1966 to June 2006), and EMBASE (from January 1980 to June 2006) for randomised trials and contacted authors of trials and other experts in the field.

Selection criteria:

We searched for quasi-randomised or randomised controlled trials of participants with neuromuscular or chest wall disorder-related stable chronic hypoventilation of all ages and all degrees of severity, receiving any type and any mode of nocturnal mechanical ventilation. The primary outcome measure was short-term and long-term reversal of hypoventilation related clinical symptoms and secondary outcomes were unplanned hospital admission, one year mortality, short-term and long-term reversal of daytime hypercapnia, improvement of lung function and sleep breathing disorders.

Data collection and analysis:

We identified eight randomised trials.

Main results:

The eight eligible trials included a total of 144 participants. The relative risk of 'no improvement of hypoventilation related clinical symptoms' in the short-term following nocturnal mechanical ventilation was available in only one trial with 10 participants and was not significant, 0.09 (95% confidence interval (CI) 0.01 to 1.31). The relative risk of 'no reversal of daytime hypercapnia' in the short-term following nocturnal ventilation was significant and favoured treatment, 0.37 (95% CI 0.20 to 0.65). The weighted mean difference of nocturnal mean oxygen saturation was 5.45% (95% CI 1.47 to 9.44) more improvement in participants treated with nocturnal mechanical ventilation. For most of the outcome measures there was no significant long-term difference between nocturnal mechanical ventilation and no ventilation. However, the estimated risk of death based on three studies was reduced following nocturnal ventilation, 0.62 (95% CI 0.42 to 0.91). There was considerable and significant heterogeneity between the trials possibly related to differences between the study populations. Most of the secondary outcomes were not assessed in the eligible trials. Data from two crossover trials suggested no evidence for a difference in reversal of daytime hypercapnia and sleep study parameters between volume-cycled and pressure-cycled ventilation. No data could be summarised for the comparisons between invasive and non-invasive mechanical ventilation or between intermittent positive pressure and negative pressure ventilation.

Authors' conclusions:

Current evidence about the therapeutic benefit of mechanical ventilation is weak, but consistent, suggesting alleviation of the symptoms of chronic hypoventilation in the short-term. In three small studies survival was prolonged mainly in participants with motor neuron diseases. With the exception of motor neuron disease, further larger randomised trials are needed to confirm long-term beneficial effects of nocturnal mechanical ventilation on quality of life, morbidity and mortality, to assess its cost-benefit ratio in neuromuscular and chest wall diseases and to compare the different types and modes of ventilation.

This record should be cited as:

Annane D, Orlikowski D, Chevret S, Chevrolet JC, Raphaël JC. Nocturnal mechanical ventilation for chronic hypoventilation in patients with neuromuscular and chest wall disorders. Cochrane Database of Systematic Reviews 2007, Issue 4. Art. No.: CD001941. DOI: 10.1002/14651858.CD001941.pub2

Assessed as up to date:

August 21, 2007

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Primary Review Group:

Neuromuscular Disease Group

Health topics:

Neurology > Neuromuscular disease > Complications

Cochrane Summaries^beta

Independent high-quality evidence for health care decision making

Nocturnal mechanical ventilation may improve chronic hypoventilation symptoms and survival in neuromuscular diseases

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