Canadian respiratory journal : journal of the Canadian Thoracic Society
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To evaluate the long term effects of home mechanical ventilation (HMV) on pulmonary function, nighttime gas exchange, daytime arterial blood gases, sleep architecture and functional exercise capacity (6 min walk). Patients with respiratory failure attributable to thoracic restrictive disease (TRD) (kyphoscoliosis) or neuromuscular disease (NMD) were assessed, ventilated, trained and followed in a dedicated unit for the care of patients requiring long term ventilation. ⋯ HMV was associated with sustained, long term improvements in nighttime and daytime gas exchange in patients with TRD and NMD. Function exercise capacity increased in patients with TRD and in a subgroup of ambulatory patients with NMD. In patients with TRD, these improvements were maintained for up to 10 years after HMV was established.