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Annual Respiratory Evaluations in Congenital Central Hypoventilation Syndrome and Changes in Ventilatory Management
Amit S Shah1, Roberta M Leu1, Thomas G Keens2
1Division of Pediatric Pulmonology and Sleep Medicine, Department of Pediatrics, Emory University School of Medicine, Children's Healthcare of Atlanta, Atlanta, Georgia, USA.
Insights
Annual respiratory evaluations (AREs) in congenital central hypoventilation syndrome (CCHS) patients frequently lead to adjustments in assisted ventilation (AV) management, highlighting the importance of these assessments for optimizing care.
Area of Science:
- Pediatric Pulmonology
- Sleep Medicine
- Critical Care
Background:
- Congenital central hypoventilation syndrome (CCHS) necessitates ongoing monitoring of ventilatory function.
- Expert consensus recommends annual in-hospital respiratory evaluations (AREs) for CCHS patients aged ≥2-3 years.
- The impact of AREs on ventilatory management strategies in CCHS patients requires further investigation.
Purpose of the Study:
- To determine if AREs in CCHS patients result in changes to their ventilatory management.
- To assess the clinical significance of ARE findings in optimizing assisted ventilation (AV) for CCHS patients.
Main Methods:
- Retrospective review of 10 CCHS patients (aged 4-20 years) who underwent AREs between 2017-2019.
- Evaluations included assessments during wakefulness and sleep, with or without polysomnography.
- Analysis of clinical symptoms, ARE results, and subsequent modifications in AV settings and duration.
Main Results:
- 70% of patients required changes in AV management following AREs.
- Six patients needed increased AV settings or duration; one required decreased settings.
- AREs identified needs for increased AV duration (up to 16 hours/day) and improved awake hypercapnia.
Conclusions:
- AREs frequently lead to significant adjustments in assisted ventilation management for CCHS patients.
- Regular AREs are crucial for accurately assessing ventilatory requirements and optimizing AV in CCHS.
- Findings underscore the clinical utility of AREs in the long-term management of CCHS.
Abstract:
Annual in-hospital respiratory evaluations (AREs) during wakefulness and sleep are recommended to assess ventilatory requirements in patients with congenital central hypoventilation syndrome (CCHS) aged ≥2-3 years based on expert consensus. This study aimed to determine if AREs in patients with CCHS led to changes in ventilatory management. Retrospective review of patients with CCHS who underwent AREs with or without polysomnography between 2017 and 2019 was conducted. Clinical symptoms, results of AREs, and subsequent changes in ventilatory management were analyzed. We identified 10 patients with CCHS aged 4-20 years. All patients required assisted ventilation (AV) only during sleep delivered by positive pressure ventilation via tracheostomy (n = 7) or diaphragm pacing (n = 3). In total, 7 (70%) patients had abnormal oxygenation and/or ventilation requiring changes in ventilator settings or duration of AV. Six patients required an increase in settings and/or duration of AV, and only 1 patient required a decrease in ventilator settings. Two patients had awake hypercapnia during a routine outpatient visit that improved following increase in ventilator settings and a period of continuous AV. One patient who was previously ventilator-dependent only during sleep was identified to require 16 h per day of AV. All patients (n = 3) who reported symptoms such as headache or oxygen desaturations during sleep required an increase in ventilator settings. We report a high prevalence of changes in AV management following an ARE. Our results demonstrate the importance of regular AREs in patients with CCHS to assess their ventilatory requirements and optimize AV.
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