Ventilation patterns mimicking COPD in patients with diaphragmatic pacing for Ondine's curse

Ventilation was studied with 133Xe in 18 patients with central hypoventilation (Ondine's Curse) in whom diaphragmatic pacers were implanted. Three distinct patterns emerged: Type I, improvement in ventilation on the paced side (11 of 18 patients); Type II, improvement on both the paced and unpaced side (4 of 18); and Type III, no improvement (3 of 18). With the pacer off, many of these patients have patterns that mimic chronic obstructive pulmonary disease and that revert to normal with pacing. This retention, clearly reversible, cannot reflect permanent airways or airspace disease.