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The impact of obstructive sleep apnea on aortic disease in Marfan's syndrome
Malcolm Kohler1, Alex Pitcher, Edward Blair
1Sleep Disorders Center and Pulmonary Division, University Hospital Zurich, Zurich, Switzerland. Malcolm.K@bluewin.ch
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.
Insights
Obstructive sleep apnea (OSA) may shorten aortic event-free survival in Marfan syndrome patients. Further research is needed to confirm this link between OSA and aortic events in this population.
Area of Science:
- Cardiovascular Medicine
- Sleep Medicine
- Genetics
Background:
- Marfan syndrome is a genetic disorder associated with life-threatening aortic dissection.
- Preliminary evidence links obstructive sleep apnea (OSA) to aortic disease in Marfan syndrome patients.
Purpose of the Study:
- To investigate the impact of OSA on the occurrence of aortic events in individuals with Marfan syndrome.
Main Methods:
- A sleep study was conducted to diagnose OSA (defined as >5 apnea/hypopnea events per hour).
- Aortic events were defined as surgery for aortic dilatation or death from aortic rupture.
- Kaplan-Meier survival analysis and Cox regression models were used to assess event-free survival.
Main Results:
- Of 44 Marfan syndrome patients, 15 (34.1%) had OSA. Median follow-up was 29 months.
- Patients with OSA experienced significantly shorter event-free survival compared to those without OSA (p=0.012).
- Univariate analysis showed an association between apnea/hypopnea events (A+H) and aortic events (HR 1.09, p=0.023), which was modified by BMI.
Conclusions:
- The study suggests a potential association between OSA and reduced aortic event-free survival in Marfan syndrome.
- More robust studies are required to definitively establish the relationship between OSA and aortic events in this patient group.
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