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Clinical Trial 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.


Clinical Trial 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) ;


Study Design


Related Conditions & MeSH terms


NCT number NCT03985657
Study type Interventional
Source Johns Hopkins University
Contact
Status Completed
Phase N/A
Start date June 6, 2018
Completion date December 6, 2019

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