The acute effects of nasal positive pressure ventilation in patients with advanced cystic fibrosis.

OBJECTIVE

To evaluate the acute effects of noninvasive positive pressure ventilation (NPPV) in patients with stable chronic respiratory failure secondary to cystic fibrosis.

PATIENTS: Eight patients (29+/-5 years of age) with severe airflow limitation (mean FEV1, 24+/-3% predicted) and chronic respiratory failure (PaO2=67+/-15 mm Hg and PaCO2=50+/-4 mm Hg) were evaluated.

METHODS

Tidal volume, respiratory rate, minute ventilation, oxygen saturation, and transcutaneous CO2 (TcCO2) measurements were made over a 20-min period before and after the application of NPPV (inspiratory pressure of 10 to 12 cm H2O and expiratory pressure of 4 to 6 cm H2O).

RESULTS

NPPV increased saturation from 88+/-2% to 90+/-1% (p<0.05) and decreased TcCO2 from 51+/-3 mm Hg to 50+/-2 mm Hg (p<0.05). Tidal volume increased from 219+/-20 mL to 256+/-37 mL (p=not significant [NS]) and respiratory rate decreased from 24+/-2 to 18+/-1 (p<0.01). Minute ventilation decreased from 5.3+/-0.8 L/min to 4.6+/-0.6 L/min (p=0.08). There was no change in duty cycle (32+/-5% to 34+/-5%, p=NS). In two patients, esophageal pressure measurements were also recorded. There was a decrease in pressure from -21+/-1 cm H2O to -11+/-2 cm H2O and -14+/-1 cm H2O to -7+/-1 cm H2O.

CONCLUSIONS

In patients with stable, severe cystic fibrosis, NPPV (1) acutely improves gas exchange, (2) decreases minute ventilation, suggesting either a reduction in CO2 production or an increase in alveolar ventilation, and (3) reduces work of breathing.