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The dynamic process of how non-invasive positive pressure ventilation (NPPV) improves spontaneous ventilation is unclear. Therefore, daytime trends of blood gases and breathing pattern were assessed by measurements 0, 0.5, 1, 3, 7, 11 and 15 h after cessation of nocturnal controlled NPPV in patients with chronic hypercapnic respiratory failure. Twelve patients (six COPD/six restrictive) who were established on NPPV and 12 controls (six COPD/six restrictive) completed. PaCO2 decreased during controlled NPPV (P < 0.02). PaCO2 additionally decreased step by step during the first 3 h of spontaneous breathing after switching from NPPV to spontaneous breathing (P < 0.05), but remained unchanged in controls. The PaCO2 decrease was due to a stepwise increase in tidal volume (P < 0.05) at an unchanged breathing frequency. Accordingly, minute ventilation also stepwise increased (P < 0.03). There were no significant changes in controls. Improvements of PaCO2 and tidal volume occurred even after 5.7 +/- 3.1 days following first NPPV trials, but became more evident after 2 months. Maximal inspiratory mouth pressures increased in chronic obstructive pulmonary disease (COPD) patients (P < 0.05), and respiratory drive increased in restrictive patients (P < 0.05) following 2 months of NPPV. Lung function parameters and inspiratory impedance remained unchanged. Improvements in health-related quality of life were evident and were correlated to the decline of elevated bicarbonate levels (r = 0.72, P < 0.01). In conclusion, there is a stepwise adaptation process lasting 3h when switching from nocturnal controlled NPPV to daytime spontaneous breathing in which tidal volume increases and PaCO2 drops after an initial PaCO2 decrease while on NPPV.

Both pressure- and volume-limited non-invasive positive pressure ventilation (NPPV) have been used in patients with chronic respiratory failure. The aim of the present study was to compare the efficacy of ventilation during nocturnal volume- and pressure-limited NPPV. Fifteen patients (nine COPD, six non-COPD) were randomly assigned to receive either volume-limited or pressure-limited NPPV and were switched to the complementary mode after 6 weeks. Ten patients (five COPD, five non-COPD) completed the study. PaCO2 during sleep comparably decreased from 54.6+/-8.0 to 46.2+/-6.1 mmHg during volume-limited NPPV (P<0.05), and to 46.5+/-6.4 mmHg during pressure-limited NPPV (P<0.05). Improvements in sleep quality assessed by polysomnography were comparable, but less gastrointestinal side effects were reported for pressure-limited NPPV (P<0.05). Using a pneumotachograph the variance of inspiratory volumes was lower, but the variance of peak inspiratory pressures was higher during volume-limited NPPV compared to pressure-limited NPPV. Substantial leak volumes which accounted for 57% (volume-limited NPPV) and for 58% (pressure-limited NPPV) of the applied inspiratory volume were independent from the mode of ventilation. In conclusion, nocturnal volume- and pressure-limited NPPV have similar effects on gas exchange and sleep quality in patients with hypercapnic chronic respiratory failure, but volume-limited NPPV is associated with more gastrointestinal side effects.

Untreated idiopathic congenital central hypoventilation syndrome (CCHS) is thought to cause infant death within 1-2 months. Here we present an adult patient with CCHS who survived without continuous ventilatory support, despite hypoventilation from early childhood onward. The diagnosis was confirmed at the age of 22 years, when the patient presented with hypoventilation during the night (PaCO2 60 mm Hg, PaO2 56 mm Hg, pH 7.32 HCO3- 30 mmol/l) but hyperventilation when awake (PaCO2 26 mm Hg, PaO2 81 mm Hg, pH 7.56, HCO3- 23 mmol/l). The maximal hematocrit was 77%. Despite mental retardation, noninvasive positive pressure ventilation (NPPV) could be successfully established. NPPV-supported ventilation during the night (PaCO2 36, PaO2 84 mm Hg, pH 7.47, HCO3- 25 mmol/l) reduced hematocrit values (40.6 to 36.8%) over a period of 4 years. In conclusion, long-term survival with CCHS is possible without continuous ventilatory support. Spontaneous improvement of hypoventilation during sleep throughout childhood is possible and hyperventilation during wakefulness may occur in patients with CCHS. CCHS can be managed with NPPV despite mental retardation, even over a long-term period., (Copyright 2004 S. Karger AG, Basel)

Only few data concerning weaning by nasal positive pressure ventilation (NPPV) are available, and successful weaning by using NPPV in patients with acute respiratory distress syndrome (ARDS) and severe complications has not yet been described. Two cases with ARDS and both preexisting thoracopulmonary disease (infundibulum abnormality and suspected COPD) and associated complications (recurrent sepsis, acute renal failure, need for lobectomy, severe malnutrition) could not be weaned by invasive ventilatory techniques. Both patients presented with rapid shallow breathing and PaCO(2) values >60 mm Hg during intermittent trials of spontaneous breathing, although the primary pathology and associated complications had been resolved. Patients were successfully adapted on NPPV in a stepwise approach after 93 days and 67 days of invasive ventilation. In one patient withdrawal from NPPV was possible after 2 months. In the other patient the duration of daily ventilation could be significantly reduced from 18 to 6 h/day after 9 months on NPPV. Therefore, patients with ARDS who cannot be weaned by invasive ventilatory strategies might be removed successfully from invasive mechanical ventilation by using NPPV even when there are preexisting thoracopulmonary disease and major complications during invasive ventilation., (Copyright 2002 S. Karger AG, Basel)