title: The impact of obstructive sleep apnea on aortic disease in Marfan's syndrome.
abstract: 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.
  
  
  OBJECTIVE
  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.
authors.affiliation: Sleep Disorders Center and Pulmonary Division, University Hospital Zurich, Zurich, Switzerland. Malcolm.K@bluewin.ch
authors.forename: Malcolm
authors.initials: M
authors.lastname: Kohler
authors.forename: Alex
authors.initials: A
authors.lastname: Pitcher
authors.forename: Edward
authors.initials: E
authors.lastname: Blair
authors.forename: Peter
authors.initials: P
authors.lastname: Risby
authors.forename: Oliver
authors.initials: O
authors.lastname: Senn
authors.forename: Colin
authors.initials: C
authors.lastname: Forfar
authors.forename: Paul
authors.initials: P
authors.lastname: Wordsworth
authors.forename: John R
authors.initials: JR
authors.lastname: Stradling
mesh_terms: D000328:Adult; D000784:Aortic Dissection / Q000150:complications; D001014:Aortic Aneurysm / Q000150:complications / Q000601:surgery; D001018:Aortic Diseases / Q000150:complications* / Q000601:surgery; D001019:Aortic Rupture / Q000150:complications; D018450:Disease Progression; D018572:Disease-Free Survival; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D053208:Kaplan-Meier Estimate; D008297:Male; D008382:Marfan Syndrome / Q000150:complications*; D008875:Middle Aged; D017286:Polysomnography; D016016:Proportional Hazards Models; D020181:Sleep Apnea, Obstructive / Q000453:epidemiology*; D055815:Young Adult