Assessment of pleural pressure during sleep in Marfan syndrome.

Autor: Sowho M; Division of Pulmonary and Critical Care Medicine, Johns Hopkins School of Medicine, Baltimore, Maryland., Jun J; Division of Pulmonary and Critical Care Medicine, Johns Hopkins School of Medicine, Baltimore, Maryland., Sgambati F; Center for Interdisciplinary Sleep Research and Education, Johns Hopkins School of Medicine, Baltimore, Maryland., Potocki M; Division of Pulmonary and Critical Care Medicine, Johns Hopkins School of Medicine, Baltimore, Maryland., Schneider H; American Sleep Clinic, Center for Sleep Medicine, Frankfurt, Germany., Smith P; Division of Pulmonary and Critical Care Medicine, Johns Hopkins School of Medicine, Baltimore, Maryland., Schwartz A; American Sleep Clinic, Center for Sleep Medicine, Frankfurt, Germany., Dietz H; McKusick-Nathans Institute of Genetic Medicine, Johns Hopkins School of Medicine, Baltimore, Maryland., MacCarrick G; McKusick-Nathans Institute of Genetic Medicine, Johns Hopkins School of Medicine, Baltimore, Maryland., Neptune E; Division of Pulmonary and Critical Care Medicine, Johns Hopkins School of Medicine, Baltimore, Maryland.
Jazyk: angličtina
Zdroj: Journal of clinical sleep medicine : JCSM : official publication of the American Academy of Sleep Medicine [J Clin Sleep Med] 2022 Jun 01; Vol. 18 (6), pp. 1583-1592.
DOI: 10.5664/jcsm.9920
Abstrakt: Study Objectives: Patients with Marfan syndrome (MFS) have a high risk for aortic aneurysms. They are also susceptible to sleep-disordered breathing that may expose them to highly negative intrathoracic pressures known to increase aortic transmural pressure, which may accelerate aortic dilatation. Our objective was to quantify overnight intrathoracic pressure changes during sleep in snoring patients with MFS and the therapeutic effect of continuous positive airway pressure (CPAP).
Methods: We used a questionnaire to identify self-reported snoring patients with MFS. In these patients, we monitored intrathoracic pressure using esophageal pressure (Pes) during overnight baseline and CPAP sleep studies. We defined a peak-inspiratory Pes (Pes peak-insp ) < - 5 cm H 2 O as greater than normal and examined the distribution of Pes peak-insp during baseline and CPAP studies.
Results: In our sample of 23 snorers with MFS, we found that 70% of sleep breaths exhibited Pes peak-insp < -5 cm H 2 O, with apnea/hypopneass accounting for only 12%, suggesting prevalent stable flow-limited breathing and snoring. In a subset (n = 12) with Pes monitoring during a CPAP night, CPAP lowered the mean proportion of breaths with Pes peak-insp < -5 cm H 2 O from 83.7% ± 14.9% to 3.6% ± 3.0% ( P < .001). In addition, contemporaneous aortic root diameter was associated with the mean Pes peak-insp during inspiratory flow-limited breathing and apneas/hypopneas ( β = -0.05, r = .675, P = .033).
Conclusions: The sleep state in MFS revealed prolonged exposure to exaggerated negative inspiratory Pes, which was reversible with CPAP. Since negative intrathoracic pressure can contribute to thoracic aortic stress and aortic dilatation, snoring may be a reversible risk factor for progression of aortic pathology in MFS.
Citation: Sowho M, Jun J, Sgambati F, et al. Assessment of pleural pressure during sleep in Marfan syndrome. J Clin Sleep Med . 2022;18(6):1583-1592.
(© 2022 American Academy of Sleep Medicine.)
Databáze: MEDLINE
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