Gessler Nele, Wohlmuth Peter, Anwar Omar, Debus Eike Sebastian, Eickholt Christian, Gunawardene Melanie A, Hakmi Samer, Heitmann Kathrin, Rybczynski Meike, Schueler Helke, Sheikhzadeh Sara, Tigges Eike, Wiest Gunther H, Willems Stephan, Adam Ekaterina, von Kodolitsch Yskert
Department of Cardiology and Internal Intensive Care Medicine, Asklepios Clinic St. Georg, Semmelweis University, Campus Hamburg, Lohmuehlenstrasse 5, 20099 Hamburg, Germany.
DZHK (German Center for Cardiovascular Research), Partner Site Hamburg/Kiel/Luebeck, Berlin, Germany.
EPMA J. 2022 Jul 29;13(3):451-460. doi: 10.1007/s13167-022-00291-4. eCollection 2022 Sep.
Surgical replacement of the aortic root is the only intervention that can prevent aortic dissection and cardiovascular death in Marfan syndrome (MFS). However, in some individuals, MFS also causes sleep apnea. If sleep apnea predicts cardiovascular death, a new target for predictive, preventive, and personalized medicine (PPPM) may emerge for those individuals with MFS who have sleep apnea.
This is an investigator-initiated study with long-term follow-up data of 105 individuals with MFS. All individuals were screened for sleep apnea regardless of symptoms. Cardiovascular death served as a primary endpoint, and aortic events as a secondary outcome.
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], = 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.
Sleep apnea is emerging as an independent predictor of cardiovascular death in MFS. It seems mandatory to screen all individuals with MFS for sleep apnea and to include these individuals, with both MFS and sleep apnea, in further studies to evaluate the impact of preventive measures with regard to cardiovascular death.
The online version contains supplementary material available at 10.1007/s13167-022-00291-4.
主动脉根部手术置换是预防马凡综合征（MFS）患者主动脉夹层和心血管死亡的唯一干预措施。然而，在一些患者中，MFS也会导致睡眠呼吸暂停。如果睡眠呼吸暂停可预测心血管死亡，那么对于患有睡眠呼吸暂停的MFS患者，可能会出现预测、预防和个性化医学（PPPM）的新靶点。
这是一项由研究者发起的研究，纳入了105例MFS患者的长期随访数据。无论有无症状，所有患者均接受睡眠呼吸暂停筛查。心血管死亡作为主要终点，主动脉事件作为次要结局。
呼吸暂停低通气指数（AHI）>5次/小时的睡眠呼吸暂停在21.0%（22/105）的患者中被观察到，其中轻度睡眠呼吸暂停患者占13%（14/105），中度至重度睡眠呼吸暂停患者占7.6%（8/105）。中位随访7.76年（四分位间距：6.84，8.41）后，10%（10/105）的患者死亡，其中80%（8/10）死于心血管疾病。在调整年龄和体重指数（BMI）后，AHI评分成为心血管死亡的独立危险因素（风险比1.712，95%置信区间[1.061 - 2.761]，P = 0.0276）。33%（35/105）的患者发生了主动脉事件这一次要结局。在调整年龄和BMI后，AHI评分对主动脉事件无影响（风险比0.965，95%置信区间[0.617 - 1.509]），这可能是由于既往接受主动脉手术的患者数量较多。
睡眠呼吸暂停正在成为MFS患者心血管死亡的独立预测因素。似乎有必要对所有MFS患者进行睡眠呼吸暂停筛查，并将这些患有MFS和睡眠呼吸暂停的患者纳入进一步研究，以评估预防措施对心血管死亡的影响。
在线版本包含可在10.1007/s13167 - 022 - 00291 - 4获取的补充材料。
Zotero 插件
