Frequency of Sleep Apnea in Adults With the Marfan Syndrome

Rybczynski, Meike; Koschyk, Dietmar; Karmeier, Andreas; Geßler, Nele; Sheikhzadeh, Sara; Bernhardt, A.; Habermann, Christian R.; Treede, Hendrik; Berger, Jürgen; Robinson, Peter N.; Meinertz, Thomas; Kodolitsch, Yskert Von

doi:10.1016/j.amjcard.2010.01.369

Cited by 50 publications

(42 citation statements)

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“…They attributed, at least in part, the reduced life expectancy due to aortic complications to the high OSA prevalence among patients with Marfan's syndrome [40]. Later, a considerably high OSA prevalence in patients with Marfan's syndrome was confirmed and a moderate correlation between the apnoea-hypopnoea index (AHI) and maximal aortic root diameter was found (r=0.5, p<0.001, 95% CI 0.2-0.69) [41,42]. A follow-up study in 44 Marfan's syndrome was then performed to further explore the longitudinal association between OSA and aortic disease [43].…”

Section: Resultsmentioning

confidence: 91%

The impact of obstructive sleep apnoea on the aorta

2015

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Obstructive sleep apnoea (OSA) has been shown to be a causal factor in the pathogenesis of vascular dysfunction and hypertension, conditions which can promote dilation and subsequent aortic dissection and rupture. The objective of this review is to summarise the current literature on the possible association between OSA and aortic disease and delineate the underlying mechanisms.Relevant studies were found by searching for terms including "obstructive sleep apnoea" in combination with "aortic aneurysm, dissection, and dilation" in the MEDLINE and EMBASE databases.Observational studies consistently reported that OSA is highly prevalent among patients with aortic aneurysms and aortic dissections. Patients with co-occurring OSA and Marfan's syndrome as well as patients at the more severe end of the spectrum of OSA seem to be especially vulnerable to aortic disease.Several mechanisms are discussed concerning the link between OSA and aortic disease: nocturnal negative intrathoracic pressure surges leading to mechanical stretching of the aorta and ultimately aortic distension; arousal-induced reflex sympathetic activation with subsequent hypertension; and intermittent hypoxia associated with autonomic nervous system activation and consequently increased oxidative stress. Further well controlled studies are needed in order to define the exact role of OSA as a risk factor for aortic disease. @ERSpublications Several studies have shown an association between obstructive sleep apnoea and aortic aneurysms/dissections http://ow.ly/Nh9jc

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Section: Resultsmentioning

confidence: 91%

The impact of obstructive sleep apnoea on the aorta

2015

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“…There is some preliminary evidence that OSA may be a risk factor for the development and progression of aortic aneurysms in patients with and without Marfan's syndrome [10,11,12,21,22,23]. Kohler et al [10] recently reported that the severity of OSA in patients with Marfan's syndrome is associated with an increased aortic root diameter and patients with OSA have a larger aortic root than patients without OSA.…”

Section: Discussionmentioning

confidence: 99%

The Impact of Obstructive Sleep Apnea on Aortic Disease in Marfan's Syndrome

Kohler¹,

Pitcher

Blair³

et al. 2012

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Background: Aortic dissection is a life-threatening manifestation of Marfan's syndrome. Preliminary evidence suggests that obstructive sleep apnea (OSA) is associated with aortic disease in Marfan's syndrome. Objectives: To study the effect of OSA on aortic events in Marfan's syndrome. Methods: In patients with Marfan's syndrome, a sleep study was performed at baseline and OSA was defined as >5 events of apnea/hypopnea (A+H) per hour in bed. Operation because of progressive aortic dilatation and death because of aortic rupture were defined as ‘aortic events'. Kaplan-Meier survival analyses were used to compare event-free survival in patients with and without OSA. Cox regression models were used to explore the effects of covariates on event-free survival. Results: Data from 44 patients (mean age 37.4 years, 30 females) were available for analysis; 15 patients (34.1%) had OSA. The median follow-up time was 29 (interquartile range 24-36) months. Five patients had an aortic event within the follow-up time. Median event-free survival was 51.6 months. Event-free survival was significantly shorter in patients with OSA compared to patients without OSA (p = 0.012). In univariate analysis, A+H was associated with aortic events [hazard ratio (HR) 1.09, 95% confidence interval (CI) 1.01-1.18, p = 0.023]. Taking the interaction between BMI and A+H into account increased the HR for A+H (HR 1.75, 95% CI 1.003-3.048, p = 0.049). This association was no longer significant when other covariates were forced into the multivariate analysis. Conclusions: These data suggest that aortic event-free survival may be shorter in patients with Marfan's syndrome and OSA compared to patients without OSA, but more data from well-designed studies are needed to prove this association.

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“…Obstructive and central sleep apnea is observed more frequently in MFS patients and is related to increased risk for mitral valve surgery, atrial fibrillation, decreased left ventricular ejection fraction, and aortic events (25,26).…”

Section: Pulmonary Featuresmentioning

confidence: 99%

Differences in manifestations of Marfan syndrome, Ehlers-Danlos syndrome, and Loeys-Dietz syndrome

Meester

Verstraeten

Schepers

et al. 2017

Ann. Cardiothorac. Surg.

220

184

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Many different heritable connective tissue disorders (HCTD) have been described over the past decades.These syndromes often affect the connective tissue of various organ systems, including heart, blood vessels, skin, joints, bone, eyes, and lungs. The discovery of these HCTD was followed by the identification of mutations in a wide range of genes encoding structural proteins, modifying enzymes, or components of the In contrast, no association is reported between LDS and the presence of ectopia lentis, a key distinguishing feature of MFS. Overlapping features between MFS and LDS include scoliosis, pes planus, anterior chest deformity, spontaneous pneumothorax, and dural ectasia. EDS refers to a group of clinically and genetically heterogeneous connective tissue disorders and all subtypes are characterized by variable abnormalities of skin, ligaments and joints, blood vessels, and internal organs. Typical presenting features include joint hypermobility, skin hyperextensibility, and tissue fragility. Up to one quarter of the EDS patients show aortic aneurysmal disease. The latest EDS nosology distinguishes 13 subtypes. Many phenotypic features show overlap between the different subtypes, which makes the clinical diagnosis rather difficult and highlights the importance of molecular diagnostic confirmation.

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Frequency of Sleep Apnea in Adults With the Marfan Syndrome

Cited by 50 publications

References 28 publications

The impact of obstructive sleep apnoea on the aorta

The impact of obstructive sleep apnoea on the aorta

The Impact of Obstructive Sleep Apnea on Aortic Disease in Marfan's Syndrome

Differences in manifestations of Marfan syndrome, Ehlers-Danlos syndrome, and Loeys-Dietz syndrome

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