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

NCT04447196 | Unknown

• 1 other identifier: 2020-A00215-34
• Study Type: observational
• Target: 20
• Locations: 1 country
• Sites: 1

Brief 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

Outcome Measures

Primary Outcomes (1)

• "Immediate unpleasantness" corresponding to A.1 item of the multidimensional dyspnea profile

  Measure MDP scores before and after NIV

  Change in MDP score between baseline and after 1 hour of NIV

Study Arms (1)

CCHS

20 patients presenting with central hypoventilation syndrome during spontaneous breathing and with Non Invasive Ventilation

Other: MDP

Interventions

MDP OTHER

Multi dimensional dyspnea profile scores at rest and during non invasive ventilation

CCHS

Eligibility Criteria

• Age: 18 Years+
• Sex: all
• Healthy Volunteers: No
• Age Groups: Adult (18-64), Older Adult (65+)
• Sampling Method: Non-Probability Sample

Study Population

Adults with CCHS who already require long terme NIV but not phrenic nerve stimulation

You may qualify if:

• patients with CCHS confirmed by genetic test
• patients with long term non invasive ventilation
• no recent condition that could impact breathing sensations such as infection, pulmonary embolism, heart failure

You may not qualify if:

• pregnancy
• phrenic nerve stimulation

Sponsors & Collaborators

• Association pour le Développement et l'Organisation de la Recherche en Pneumologie et sur le Sommeil: lead

Study Sites (1)

INSERM-UPC UMR_S 1158, Service de Pneumologie et Réanimation, GH Pitié-Salpêtrière

Paris, 75013, France

Location

MeSH Terms

Conditions

Congenital central hypoventilation syndrome; Dyspnea

Condition Hierarchy (Ancestors)

Respiration Disorders; Respiratory Tract Diseases; Signs and Symptoms, Respiratory; Signs and Symptoms; Pathological Conditions, Signs and Symptoms

Central Study Contacts

MARLENE BRET

CONTACT

0698674449; marlene.bret@aphp.fr

Study Design

• Study Type: observational
• Observational Model: COHORT
• Time Perspective: PROSPECTIVE
• Sponsor Type: OTHER
• Responsible Party: SPONSOR

Study Record Dates

• First Submitted: May 30, 2020
• First Posted: June 25, 2020
• Study Start: December 15, 2021
• Primary Completion: October 1, 2022
• Study Completion: October 1, 2022
• Last Updated: July 12, 2021

Record last verified: 2021-07

Locations

FR (1)