Noninvasive Ventilatory Support in Restrictive Disorders

The following sections will review the indications for NIV in the most frequent diagnostic categories (Table 1). 

Patients with thoracic restriction (TR) have an increased risk of developing respiratory failure 

Therefore, in symptomatic patients with daytime normocapnia but significant nocturnal desaturation (Sao2 90% for 10% total sleep time, or mean REM Sa02 90% and minimum Sa02 90% or Sao2 88% for 5 consecutive minutes), NIV is warranted (3). 

NMD can be divided into two groups stable and progressive. In this chapter, we will discuss two of the more frequently encountered examples in each group. Symptoms are similar to those described previously (Table 2). 

ALS is an acquired disorder with a rapidly progressive evolution. Respiratory failure is the principal cause of death (92). Median survival is 32 months from the onset of symptoms and 19 months from diagnosis (93). Despite fears that NFV would delay death without improving quality of life, for some patients with respiratory involvement, NIV has been shown to improve symptoms, quality of life (18,19,94), and survival (81). As ALS patients eventually become quadriplegic, they require a high level of assistance from caregivers (92) 

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