Department of Systems Medicine, University of Rome Tor Vergata, 00133 Rome, Italy.
Sleep Medicine Centre, Department of Systems Medicine, University of Rome Tor Vergata, 00133 Rome, Italy.
Int J Environ Res Public Health. 2021 Mar 16;18(6):3045. doi: 10.3390/ijerph18063045.
The main cause of mortality in Marfan syndrome (MS) is aortic disease. Obstructive sleep apnea (OSA) is highly prevalent in MS, and it is also associated with cardiovascular risk and maxillary deformities. The aim of this research was to analyze the possible relations between OSA, palatal morphology, and aortic root diameters in growing subjects. A group of 15 growing subjects with MS and a control group of healthy peers was selected. All of them underwent nocturnal polygraphic monitoring, digital dental casts, and transthoracic echocardiography. The results showed that OSA indexes and aortic diameters were significantly higher in the study group. Transversal palatal dimension was smaller in MS patients. Moreover, OSA severity was not related with maxillary contraction severity in MS. No correlation between OSA severity and increased aortic diameters occurred. A high prevalence of OSA, increased aortic dimensions, and significant contraction of palatal dimensions were observed in MS compared with the control group. None of these single factors may directly be associated with aortic dilation, but together, they might contribute to the development and progression of aortic aneurysm from a young age.
马凡综合征（Marfan syndrome，MS）患者的主要死亡原因是主动脉疾病。阻塞性睡眠呼吸暂停（obstructive sleep apnea，OSA）在 MS 中非常普遍，并且与心血管风险和上颌畸形有关。本研究旨在分析 OSA、腭形态和主动脉根部直径在生长中的 MS 患者之间的可能关系。选择了一组 15 名患有 MS 的生长中的患者和一组健康的同龄患者作为对照组。所有患者均接受了夜间多导睡眠监测、数字牙列模型和经胸超声心动图检查。结果显示，研究组的 OSA 指数和主动脉直径明显更高。MS 患者的腭横向尺寸较小。此外，MS 患者的 OSA 严重程度与上颌后缩的严重程度无关。OSA 严重程度与主动脉直径增加之间无相关性。与对照组相比，MS 患者中 OSA 的高患病率、主动脉尺寸的增加以及腭尺寸的显著收缩更为明显。这些单一因素可能都不会直接导致主动脉扩张，但它们共同作用可能会导致年轻患者的主动脉瘤从早期开始发展和进展。
