Sleep-phase-related home therapy in congenital central hypoventilation syndrome (CCHS)

Patients and Method: Eight children with congenital central hypoventilation syndrome (CCHS) (aged 3 to 16 years) underwent repeated polysomnographic ( sleep-EEG, induction plethysmography, PtcO2, PtcCO2, PACO2, FO2, SaO(2), EC G) during spontaneous breathing and during therapy. The result led to indiv idual therapeutic plans. Result: During NREM sleep a close relationship between increasing EEG-delta -activity and increasing PCO2 could be observed (PCO2 max. 107 mm Hg in NRE M IV). A similar effect was seen during mechanical ventilation with decreas ing spontaneous respiratory activity during increasing sleep depth (PCO(2)m ax. 89 mm Hg in NREM IV). Associated with NREM I/II and REM sleep strong va riations in spontaneous breathing with consecutive variations of blood gase s were observed. Hyperventilation during REM sleep (PCO2 min. 20 mm Hg) cou ld occur with continuous mechanical ventilation. A continuous blood gas mon itoring improved home therapy since blood gas adapted control of mechanical ventilation was possible now. This caused a stabilization of blood gases i n sleep. Conclusion: Patients with CCHS show a vigilance-dependent, enlarged variabi lity of blood gases which should be considered in the management of home th erapy. Continuous monitoring and blood gas adapted mechanical ventilation o btain a stabilization of acid-base balance during sleep. Preliminary data s uggest a positive effect on sleep-wake quality and mental performance.