Marfans Syndrome: 2014 Sep;127(17):3150-5. High prevalence of obstructive sleep apnea in Marfans syndrome. Mo L1, He Q2, Wang Y3, Dong B1, He J4. Author information Abstract OBJECTIVE: To review the current evidence about the prevalence of obstructive sleep apnea in patients with Marfans 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 Marfans syndrome and sleep apnea. STUDY SELECTION: Clinical evidence about the epidemiology of obstructive sleep apnea in patients with Marfans syndrome; the mechanism that causes obstructive sleep apnea; interventional therapy for patients with Marfans syndrome, and coexisting obstructive sleep apnea. RESULTS: A high prevalence of obstructive sleep apnea exists in patients with Marfans 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 Marfans syndrome. CONCLUSIONS: Obstructive sleep apnea is strongly associated with Marfans 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 Marfans syndrome.
Posted on: Tue, 09 Sep 2014 16:35:10 +0000
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