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Hemodynamics Response to Upper Airway Obstruction in Marfan Syndrome (MSB)

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ClinicalTrials.gov Identifier: NCT03985657
Recruitment Status : Completed
First Posted : June 14, 2019
Results First Posted : December 28, 2020
Last Update Posted : December 28, 2020
Sponsor:
Collaborators:
The Marfan Foundation
National Institutes of Health (NIH)
Information provided by (Responsible Party):
Johns Hopkins University

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.


Condition or disease Intervention/treatment Phase
Sleep-disordered Breathing Snoring Device: CPAP Not Applicable

Detailed Description:

Research Objective/Significance:

Specific Aim 1a: To quantify the Pes and BP during periods with and without UAO during sleep in persons with MFS.

Specific Aim 1b: To examine the effect of CPAP treatment of UAO on Pes and BP in MFS persons.

Specific Aim 2: To examine the effect of CPAP treatment of UAO on daytime markers of hemodynamic stress (augmentation index, reactive hyperemia index) in MFS persons.

Primary Outcomes:

  • Overnight measure of hemodynamic stress (blood pressure, pleural pressure swings)

Secondary Outcomes:

  • Changes in diurnal markers of hemodynamic stress (augmentation index, reactive hyperemia index)

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Study Type : Interventional  (Clinical Trial)
Actual Enrollment : 31 participants
Allocation: Randomized
Intervention Model: Crossover Assignment
Intervention Model Description: 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.
Masking: None (Open Label)
Primary Purpose: Treatment
Official Title: Sleep Disordered Breathing in Marfan Syndrome: Susceptibility and Hemodynamics
Actual Study Start Date : June 6, 2018
Actual Primary Completion Date : December 6, 2019
Actual Study Completion Date : December 6, 2019

Resource links provided by the National Library of Medicine


Arm Intervention/treatment
Active Comparator: Baseline Sleep Study
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
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.

Experimental: CPAP Sleep Study
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
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.




Primary Outcome Measures :
  1. Pleural Pressure (Pes) [ Time Frame: Overnight on both CPAP and No CPAP nights ]
    Pleural pressure (Pes) in mmHg monitored using an esophageal catheter.

  2. Mean Arterial Blood Pressure (MAP) [ Time Frame: Overnight on both Baseline and CPAP studies ]
    Continuous blood pressure monitored using a non-invasive finger cuff. Data captured in mmHg.


Secondary Outcome Measures :
  1. Augmentation Index (AI) [ Time Frame: 15 minutes in the morning post Baseline and CPAP studies ]
    The AI (measured as "percentage of pulse pressure") was assessed in the morning after both Baseline and CPAP studies. It is a measure of arterial stiffness that represents the degree of and can range from -10% to +10% in healthy individuals, with values generally higher in females and increases with age.

  2. Reactive Hyperemia Index (RHI) [ Time Frame: 15 minutes ]
    The RHI is a measure of endothelial function that was measured in the morning after both Baseline and CPAP studies. It is unitless and scored on a range of 1 to 3 in healthy individuals with lower values indicating poor endothelial function.



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Ages Eligible for Study:   18 Years and older   (Adult, Older Adult)
Sexes Eligible for Study:   All
Accepts Healthy Volunteers:   Yes
Criteria

Inclusion Criteria:

People with Marfan syndrome.

  • Age ≥ 18yrs
  • Able and willing to provide informed consent
  • Willing to sleep connected to research apparatus

Exclusion Criteria:

  • 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

Information from the National Library of Medicine

To learn more about this study, you or your doctor may contact the study research staff using the contact information provided by the sponsor.

Please refer to this study by its ClinicalTrials.gov identifier (NCT number): NCT03985657


Locations
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United States, Maryland
Johns Hopkins University
Baltimore, Maryland, United States, 21224
Sponsors and Collaborators
Johns Hopkins University
The Marfan Foundation
National Institutes of Health (NIH)
Investigators
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Principal Investigator: Mudiaga Sowho Johns Hopkins University
  Study Documents (Full-Text)

Documents provided by Johns Hopkins University:
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Responsible Party: Johns Hopkins University
ClinicalTrials.gov Identifier: NCT03985657    
Other Study ID Numbers: IRB00157403
First Posted: June 14, 2019    Key Record Dates
Results First Posted: December 28, 2020
Last Update Posted: December 28, 2020
Last Verified: December 2020
Individual Participant Data (IPD) Sharing Statement:
Plan to Share IPD: No

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Studies a U.S. FDA-regulated Drug Product: No
Studies a U.S. FDA-regulated Device Product: Yes
Additional relevant MeSH terms:
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Marfan Syndrome
Arachnodactyly
Respiratory Aspiration
Sleep Apnea Syndromes
Snoring
Respiration Disorders
Respiratory Tract Diseases
Pathologic Processes
Apnea
Sleep Disorders, Intrinsic
Dyssomnias
Sleep Wake Disorders
Nervous System Diseases
Respiratory Sounds
Signs and Symptoms, Respiratory
Bone Diseases, Developmental
Bone Diseases
Musculoskeletal Diseases
Heart Defects, Congenital
Cardiovascular Abnormalities
Cardiovascular Diseases
Heart Diseases
Abnormalities, Multiple
Congenital Abnormalities
Genetic Diseases, Inborn
Connective Tissue Diseases
Limb Deformities, Congenital
Musculoskeletal Abnormalities