Li Mo1, Quanying He2, Yinna Wang3, Birong Dong1, Jinhan He4. 1. Center of Gerontology and Geriatrics, West China Hospital, Sichuan University, Chengdu, Sichuan 610041, China. 2. Department of Respiratory and Critical Care Medicine, Peking University People's Hospital, Beijing 100044, China. 3. Vascular Medicine Institute, University of Pittsburgh School of Medicine, Pittsburgh, PA 15261, USA. 4. Department of Pharmacy, West China Hospital, Sichuan University, Chengdu, Sichuan 610041, China. Email: jinhanhe@gmail.com.
Abstract
OBJECTIVE: To review the current evidence about the prevalence of obstructive sleep apnea in patients with Marfan's syndrome, and discuss some proposed potential mechanisms for this relationship. DATA SOURCES: The data in this review were mainly from Medline and PubMed articles published in English from 1990 to 2013. The search term was "Marfan's syndrome and sleep apnea". STUDY SELECTION: Clinical evidence about the epidemiology of obstructive sleep apnea in patients with Marfan's syndrome; the mechanism that causes obstructive sleep apnea; interventional therapy for patients with Marfan's syndrome, and coexisting obstructive sleep apnea. RESULTS: A high prevalence of obstructive sleep apnea exists in patients with Marfan's syndrome. The potential reasons are craniofacial abnormalities and lax upper airway muscles, which lead to high nasal airway resistance and upper airway collapse. Obstructive sleep apnea mechanically deteriorates aortic dilatation and accelerates progression of aortic aneurysms. The condition is reversible and rapid maxillary expansion and adequate continuous positive airway pressure therapy are possible effective therapies to delay the expansion of aortic diameter in patients with Marfan's syndrome. CONCLUSIONS: Obstructive sleep apnea is strongly associated with Marfan's syndrome. Craniofacial abnormalities and lax upper airway are the main mechanisms. Untreated obstructive sleep apnea accelerates progression of aortic dissection and rupture. Effective therapies for obstructive sleep apnea could postpone the aortic dilatation in patients with Marfan's syndrome.
OBJECTIVE: To review the current evidence about the prevalence of obstructive sleep apnea in patients with Marfan's syndrome, and discuss some proposed potential mechanisms for this relationship. DATA SOURCES: The data in this review were mainly from Medline and PubMed articles published in English from 1990 to 2013. The search term was "Marfan's syndrome and sleep apnea". STUDY SELECTION: Clinical evidence about the epidemiology of obstructive sleep apnea in patients with Marfan's syndrome; the mechanism that causes obstructive sleep apnea; interventional therapy for patients with Marfan's syndrome, and coexisting obstructive sleep apnea. RESULTS: A high prevalence of obstructive sleep apnea exists in patients with Marfan's syndrome. The potential reasons are craniofacial abnormalities and lax upper airway muscles, which lead to high nasal airway resistance and upper airway collapse. Obstructive sleep apnea mechanically deteriorates aortic dilatation and accelerates progression of aortic aneurysms. The condition is reversible and rapid maxillary expansion and adequate continuous positive airway pressure therapy are possible effective therapies to delay the expansion of aortic diameter in patients with Marfan's syndrome. CONCLUSIONS:Obstructive sleep apnea is strongly associated with Marfan's syndrome. Craniofacial abnormalities and lax upper airway are the main mechanisms. Untreated obstructive sleep apnea accelerates progression of aortic dissection and rupture. Effective therapies for obstructive sleep apnea could postpone the aortic dilatation in patients with Marfan's syndrome.
Authors: Olivia J Veatch; Christopher R Bauer; Brendan T Keenan; Navya S Josyula; Diego R Mazzotti; Kanika Bagai; Beth A Malow; Janet D Robishaw; Allan I Pack; Sarah A Pendergrass Journal: BMC Med Genomics Date: 2020-07-25 Impact factor: 3.622
Authors: Giuseppina Laganà; Nicolò Venza; Arianna Malara; Claudio Liguori; Paola Cozza; Calogera Pisano Journal: Int J Environ Res Public Health Date: 2021-03-16 Impact factor: 3.390