Sleep apnea predicts cardiovascular death in patients with Marfan syndrome A cohort study

Abstract Background Identification of possible risk factors is crucial to optimize predictive, preventive, and personalized medicine (PPPM) in Marfan syndrome (MFS). Despite a well known relationship between sleep apnea and MFS, the impact of sleep apnea on cardiovascular death and on aortic events remains unclear. Methods This is an investigator-initiated study with long-term follow-up data of 105 MFS patients. All participants were screened for sleep apnea regardless of symptoms. The primary endpoint was cardiovascular death and the secondary outcome was the occurrence of aortic events. Results Sleep apnea with an apnea-hypopnea-index (AHI) > 5/h was observed in 21.0% (22/105) with mild sleep apnea in 13% (14/105) and moderate to severe sleep apnea in 7.6% (8/105). After a median follow-up of 7.76 years [interquartile range: 6.84, 8.41], 10% (10/105) had died, with cardiovascular cause of death in 80% (8/10). After adjusting for age and body-mass-index (BMI), the AHI-score emerged as an independent risk factor for cardiovascular death (hazard ratio 1.712, 95%-confidence interval [1.061–2.761], p = 0.0276). The secondary outcome of aortic events occurred in 33% (35/105). There was no effect of the AHI-score on aortic events after adjusting for age and BMI (hazard ratio 0.965, 95%-confidence interval [0.617–1.509]), possibly due to a high number of patients with prior aortic surgery. Interpretation: Sleep apnea shows to be independently predictive of cardiovascular death in patients with Marfan syndrome. These findings highlight the necessity of early sleep apnea screening in MFS patients as an important adjunct to the PPPM management of the disease.