Eight-year follow-up study of a patient with central alveolar hypoventilation treated with diaphragm pacing.

We had treated a 17-year-old girl with central alveolar hypoventilation with diaphragm pacing at home for the past 8 years. Nocturnal diaphragm pacing with an open tracheostomy was effective in maintaining an adequate ventilation after 4 months of undergoing training of the diaphragm on the pacemaker implantation. However, the diaphragm pacemaker failed to maintain it mostly in the presence of respiratory tract infection, when she was treated with intermittent positive-pressure ventilation. Pulse oximetry was used at home for monitoring the adequacy of respiratory support. We conclude that the respiratory assistance by the diaphragm pacemaker or the use of a mechanical ventilator as a backup was highly useful for the home care of a patient with central alveolar hypoventilation.