Hemodynamics Response to Upper Airway Obstruction in Marfan Syndrome
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.
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)
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