NCT04447196

Prevalence of Rest Dyspnea and Impact of Non Invasive Ventilation on Breathing Sensations in CCHS Patients

Prevalence of Rest Dyspnea and Impact of Non Invasive Ventilation on Breathing Sensations in Patients With Congenital Central Hypoventilation Syndrome

• Status: Unknown
• Phase: —
• Study type: Observational
• Enrollment: 20 (estimated)
• Sponsor: Association pour le Développement et l'Organisation de la Recherche en Pneumologie et sur le Sommeil · Academic / Other
• Sex: All
• Age: 18 Years
• Healthy volunteers: Not accepted

Summary

Congenital central hypoventilation syndrome (CCHS) is a neuro-respiratory disease characterized by lifethreatening sleep-related hypoventilation involving an alteration of CO2/H+ chemosensitivity. This suggests cortical activity during awakening to maintain breathing. Cortical activity to keep breathing is usually associated with breathing discomfort ; this is the case in healthy subjects under non invasive ventilation (NIV) or with expiratory charge as well as in patients with amyotrophic lateral sclerosis. This can suggest that CCHS may be breathless at rest and this discomfort could be reduced by NIV. The objective is to evaluate dyspnea with a multi dimensional score, MDP, in CCHS patient at rest in every day life and during 1H session of NIV. The investigators will perform a prospective, including 20 CCHS patients. MDP scores will be measure before and after 1H-non invasive ventilation as well as a visual scale of 100mm in order to evaluate variation of breathing discomfort before/after NIV. The investigators expect that CCHS patients don't have rest dyspnea but NIV would improve breathing discomfort that would mean they have latent rest dyspnea.

Conditions

• CCHS
• Congenital Central Hypoventilation Syndrome
• Dyspnea

Interventions

Type	Name	Description
OTHER	MDP	Multi dimensional dyspnea profile scores at rest and during non invasive ventilation

Timeline

• Start date: 2021-12-15
• Primary completion: 2022-10-01
• Completion: 2022-10-01
• First posted: 2020-06-25
• Last updated: 2021-07-12

Locations

1 site across 1 country: France