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

Johns Hopkins University

Status: Completed

Conditions: Snoring; Sleep-disordered Breathing

Treatments: Device: CPAP

Study type: Interventional

Funder types: Other; NIH

Identifiers

NCT03985657

IRB00157403

Details and patient eligibility

About

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.

Full 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)

Enrollment: 31 patients

Sex: All

Ages: 18+ years old

Volunteers: Accepts Healthy Volunteers

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

Trial design

Primary purpose: Treatment

Allocation: Randomized

Interventional model: Crossover Assignment

Masking: None (Open label)

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31 participants in 2 patient groups

Baseline Sleep Study

Active Comparator group

Description:

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.

Treatment:

Device: CPAP

CPAP Sleep Study

Experimental group

Description:

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.

Treatment:

Device: CPAP