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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.To study the effect of OSA on aortic events in Marfan's syndrome.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.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.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.

Original publication

DOI

10.1159/000340008

Type

Journal article

Journal

Respiration; international review of thoracic diseases

Publication Date

01/2013

Volume

86

Pages

39 - 44

Addresses

Sleep Disorders Center and Pulmonary Division, University Hospital Zurich, Zurich, Switzerland. Malcolm.K@bluewin.ch

Keywords

Humans, Marfan Syndrome, Sleep Apnea, Obstructive, Aneurysm, Dissecting, Aortic Rupture, Aortic Aneurysm, Aortic Diseases, Disease Progression, Polysomnography, Disease-Free Survival, Proportional Hazards Models, Follow-Up Studies, Adult, Middle Aged, Female, Male, Young Adult, Kaplan-Meier Estimate