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NCT Number: NCT03985657

Hemodynamics Response to Upper Airway Obstruction in Marfan Syndrome

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.

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Key information

Conditions

Sleep-Disordered Breathing Apnea Dyssomnias Nervous System Diseases Pathological Conditions, Signs and Symptoms Respiration Disorders Respiratory Sounds Respiratory Tract Diseases Signs and Symptoms Signs and Symptoms, Respiratory Sleep Apnea Syndromes Sleep Disorders, Intrinsic Sleep Wake Disorders Snoring

Age range

18 year and older

Sex eligibility

All sexes

Study type

Interventional

Phase

Not applicable

Primary location

Johns Hopkins University

Baltimore, Maryland, 21224, United States

About this study

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)

Who can participate

Healthy volunteers accepted: Yes

Only the study team can determine whether someone qualifies for participation.

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

Treatment and study plan

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.

Primary outcomes

  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 outcomes

  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.

Sponsors and collaborators

Lead sponsor

Johns Hopkins University

Other

Collaborators

  • National Institutes of Health (NIH)
  • The Marfan Foundation

Registry information

Official study title

Sleep Disordered Breathing in Marfan Syndrome: Susceptibility and Hemodynamics

Acronym: MSB

Important dates

Study start
2018
Primary completion
2019
Study completion
2019
First posted
Jun 14, 2019
Registry last updated
Dec 28, 2020

OpenTrials presents study information sourced from ClinicalTrials.gov. The official registry record should be consulted for the latest information.

View the official ClinicalTrials.gov record (opens in a new tab)

This listing is for discovery and informational purposes only. It is not medical advice, does not guarantee that a study is recruiting, and does not determine eligibility. Contact the study team and a qualified healthcare professional when considering participation.

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