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Permanent URL to this publication: http://dx.doi.org/10.5167/uzh-64942

Kohler, Malcolm; Pitcher, Alex; Blair, Edward; Risby, Peter; Senn, Oliver; Forfar, Colin; Wordsworth, Paul; Stradling, John R (2013). The Impact of Obstructive Sleep Apnea on Aortic Disease in Marfan's Syndrome. Respiration, 86(1):39-44.

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Background: Aortic dissection is a life-threatening manifestation of Marfan's syndrome. Preliminary evidence suggests that obstructive sleep apnea (OSA) is associated with aortic disease in Marfan's syndrome. Objectives: To study the effect of OSA on aortic events in Marfan's syndrome. Methods: In patients with Marfan's syndrome, a sleep study was performed at baseline and OSA was defined as >5 events of apnea/hypopnea (A+H) per hour in bed. Operation because of progressive aortic dilatation and death because of aortic rupture were defined as 'aortic events'. Kaplan-Meier survival analyses were used to compare event-free survival in patients with and without OSA. Cox regression models were used to explore the effects of covariates on event-free survival. Results: Data from 44 patients (mean age 37.4 years, 30 females) were available for analysis; 15 patients (34.1%) had OSA. The median follow-up time was 29 (interquartile range 24-36) months. Five patients had an aortic event within the follow-up time. Median event-free survival was 51.6 months. Event-free survival was significantly shorter in patients with OSA compared to patients without OSA (p = 0.012). In univariate analysis, A+H was associated with aortic events [hazard ratio (HR) 1.09, 95% confidence interval (CI) 1.01-1.18, p = 0.023]. Taking the interaction between BMI and A+H into account increased the HR for A+H (HR 1.75, 95% CI 1.003-3.048, p = 0.049). This association was no longer significant when other covariates were forced into the multivariate analysis. Conclusions: These data suggest that aortic event-free survival may be shorter in patients with Marfan's syndrome and OSA compared to patients without OSA, but more data from well-designed studies are needed to prove this association.


6 citations in Web of Science®
6 citations in Scopus®
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Additional indexing

Item Type:Journal Article, refereed, original work
Communities & Collections:04 Faculty of Medicine > Center for Integrative Human Physiology
04 Faculty of Medicine > University Hospital Zurich > Clinic for Pneumology
04 Faculty of Medicine > University Hospital Zurich > Institute of General Practice
Dewey Decimal Classification:570 Life sciences; biology
610 Medicine & health
Deposited On:02 Oct 2012 15:46
Last Modified:09 Jun 2016 10:24
Publisher DOI:10.1159/000340008
PubMed ID:23006517

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