Hemodynamics Response to Upper Airway Obstruction in Marfan Syndrome

NCT03985657 | Completed Results FDA Device

• 1 other identifier: IRB00157403
• Study Type: interventional
• Target: 31
• Locations: 1 country
• Sites: 1

Brief Summary

Upper airway obstruction (UAO) is an unrecognized source of hemodynamic stress that may contribute to aortic adverse events in persons with Marfan Syndrome (MFS). UAO occurs during snoring and sleep apnea and is characterized by repetitive partial or complete obstruction of the upper airway during sleep. These obstructive breathing events lead to intermittent surges in blood pressure (BP) REF and large decreases in pleural pressure (Pes), thereby increasing the trans-mural aortic pressure (TMP) and imposing mechanical stress on the aorta during sleep. Although UAO is known to increase mechanical stress on the aorta, the magnitude of the increase is not known for persons with MFS. In this project, therefore, the investigators will also examine the changes in Pes and BP responses in periods of obstructed breathing and compare the diurnal markers or vascular stress between Baseline and CPAP studies in MFS persons.

Conditions

Sleep-disordered Breathing; Snoring

Outcome Measures

Primary Outcomes (2)

• Pleural Pressure (Pes)

  Pleural pressure (Pes) in mmHg monitored using an esophageal catheter.

  Overnight on both CPAP and No CPAP nights

• Mean Arterial Blood Pressure (MAP)

  Continuous blood pressure monitored using a non-invasive finger cuff. Data captured in mmHg.

  Overnight on both Baseline and CPAP studies

Secondary Outcomes (2)

• Augmentation Index (AI)

  15 minutes in the morning post Baseline and CPAP studies

• Reactive Hyperemia Index (RHI)

  15 minutes

Study Arms (2)

Baseline Sleep Study

ACTIVE COMPARATOR

Baseline sleep polysomnography will involve the collection of electroencephalogram, electromyogram, electrocardiogram, airflow, heart rate, blood pressure, and pleural pressure during sleep with no CPAP. Participants in this arm would switch to CPAP within one week of the study.

Device: CPAP

CPAP Sleep Study

EXPERIMENTAL

Participants will be treated with continuous positive airway pressure to relieve sleep-disordered breathing. Participants in this arm would switch to Baseline study within one week of the study.

Device: CPAP

Interventions

CPAP DEVICE

Continuous positive airway pressure (CPAP). Room air at pressures between 6-8 centimeters of water (cmH2O) delivered via heated humidified tubing and a nasal mask.

Baseline Sleep Study; CPAP Sleep Study

Eligibility Criteria

• Age: 18 Years+
• Sex: all
• Healthy Volunteers: Yes
• Age Groups: Adult (18-64), Older Adult (65+)

You may qualify if:

• People with Marfan syndrome.
• Age ≥ 18yrs
• Able and willing to provide informed consent
• Willing to sleep connected to research apparatus

You may not qualify if:

• Unstable cardiovascular disease (CHF, myocardial infarction or revascularization procedures, and unstable arrhythmias)
• Uncontrolled hypertension (BP \> 190/110)
• Underlying obstructive or other intrinsic lung disease
• Renal failure on dialysis
• Cirrhosis
• Pregnancy

Sponsors & Collaborators

• Johns Hopkins University: lead
• The Marfan Foundation: collaborator
• National Institutes of Health (NIH): collaborator

Study Sites (1)

Johns Hopkins University

Baltimore, Maryland, 21224, United States

Location

MeSH Terms

Conditions

Sleep Apnea Syndromes; Snoring

Condition Hierarchy (Ancestors)

Apnea; Respiration Disorders; Respiratory Tract Diseases; Sleep Disorders, Intrinsic; Dyssomnias; Sleep Wake Disorders; Nervous System Diseases; Respiratory Sounds; Signs and Symptoms, Respiratory; Signs and Symptoms; Pathological Conditions, Signs and Symptoms

Results Point of Contact

• Title: Mudiaga Sowho
• Organization: Johns Hopkins University

msowho1@jhmi.edu

4105506264

Study Officials

• Mudiaga Sowho

  Johns Hopkins University

  PRINCIPAL INVESTIGATOR

Publication Agreements

• PI is Sponsor Employee: No
• Restrictive Agreement: No

Study Design

• Study Type: interventional
• Phase: not applicable
• Allocation: RANDOMIZED
• Masking: NONE
• Purpose: TREATMENT
• Intervention Model: CROSSOVER
• Sponsor Type: OTHER
• Responsible Party: SPONSOR

Model Details: Participants will be assigned to a Baseline polysomnography and CPAP polysomnography in a randomized fashion. Participants will then be switched to the second polysomnography within 14 days of the first. The baseline polysomnography represents the exposure to sleep disordered breathing and the CPAP polysomnography represents the relief of the exposure. Markers of hemodynamic stress will be assessed in the morning after both studies.

Study Record Dates

• First Submitted: June 11, 2019
• First Posted: June 14, 2019
• Study Start: June 6, 2018
• Primary Completion: December 6, 2019
• Study Completion: December 6, 2019
• Last Updated: December 28, 2020
• Results First Posted: December 28, 2020

Record last verified: 2020-12

Data Sharing

• IPD Sharing: Will not share

Locations

US (1)