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. 2018 Feb 15;14(2):261-264.
doi: 10.5664/jcsm.6948.

Obstructive Sleep Apnea in Patients With Congenital Central Hypoventilation Syndrome Ventilated by Diaphragm Pacing Without Tracheostomy

Annie Wang  1 , Sheila Kun  2 , Bonnie Diep  1 , Sally L Davidson Ward  1   2 , Thomas G Keens  1   2 , Iris A Perez  1   2
Affiliations

Obstructive Sleep Apnea in Patients With Congenital Central Hypoventilation Syndrome Ventilated by Diaphragm Pacing Without Tracheostomy

Annie Wang et al. J Clin Sleep Med. .

Abstract

Study objectives: To determine presence of obstructive sleep apnea (OSA) in patients with congenital central hypoventilation syndrome (CCHS) ventilated by diaphragm pacing (DP) without tracheostomy, and to determine if OSA can be improved by DP setting changes.

Methods: We reviewed polysomnography (PSG) results of 15 patients with CCHS from October 2001 to April 2014, age 15.4 ± 7.8 years, body mass index 22.0 ± 6.0 kg/m2, and 60% female.

Results: Of the 22 PSG results obtained for the 15 patients with CCHS, 9 were performed with tracheostomy capped, and 13 were performed after patients underwent decannulation. OSA was present on 6 of 9 tests in patients with tracheostomy capped, including 3 patients with immediate, severe OSA necessitating that the studies be completed with tracheostomy uncapped. OSA was present on 2 of 13 tests in patients in whom decannulation had been performed. Hypoventilation was seen on only one test without OSA. On 2 of 5 tests showing OSA, OSA improved by decreasing DP amplitude settings; apnea-hypopnea index decreased from 11.1 ± 2.5 to 1.8 ± 2.5 events/h; PETCO2 decreased from 57.5 ± 3.5 to 38.5 ± 0.7 torr; SpO2 increased from 76.5 ± 0.7% to 93.0 ± 7.1%. OSA improved in one patient with slight increase in respiratory rate. Settings were manipulated in 4 tests showing OSA; no changes were attempted in the remaining study. One patient was placed on bilevel positive airway pressure with temporary suspension of DP. Age (P < .119), previous adenotonsillectomy (P < .211), and body mass index (P < .112) did not significantly contribute to OSA.

Conclusions: OSA occurs in patients with CCHS ventilated by DP. However, decreasing DP amplitude settings can lessen upper airway obstruction without compromising gas exchange.

Keywords: congenital central hypoventilation syndrome (CCHS); diaphragm pacing; obstructive hypopnea; obstructive sleep apnea; tracheostomy; upper airway obstruction.

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Figures

Figure 1
Figure 1. Improvements in AHI, SpO2, and PETCO2 following a decrease in amplitude (voltage) in diaphragm pacer settings for two patients with OSA.
AHI = apnea-hypopnea index, PETCO2 = end-tidal carbon dioxide, OSA = obstructive sleep apnea, SpO2 = oxygen saturation.
See this image and copyright information in PMC

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