Mitral annulus disjunction is highly prevalent in patients with Marfan syndrome and Loyes-Dietz syndrome
Abstract Background Mitral valve prolapse is a common finding in patients with Marfan and Loyes-Dietz syndrome. Mitral annulus disjunction (MAD), an atrial displacement of the hinge point of the mitral valve, has previously been associated with non-syndromic MVP and life-threatening ventricular arrh...
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| Veröffentlicht in: | European heart journal 2020-11, Vol.41 (Supplement_2) |
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| creator | Chivulescu, M Krohg-Sorensen, K Scheirlynk, E Lindberg, B Dejgaard, L Lie, O Helle-Valle, T Skjolsvik, E Aabel, E Hunt, T Estensen, M Edvardsen, T Lingaas, P Haugaa, K |
| description | Abstract
Background
Mitral valve prolapse is a common finding in patients with Marfan and Loyes-Dietz syndrome. Mitral annulus disjunction (MAD), an atrial displacement of the hinge point of the mitral valve, has previously been associated with non-syndromic MVP and life-threatening ventricular arrhythmias.
Purpose
We aimed to assess the prevalence of MAD in patients with Marfan and Loyes-Dietz syndrome and relate the presence of MAD to mitral valve prolapse.
Methods
We included genotype positive patients with Marfan and Loyes-Dietz syndrome, and patients with Marfan syndrome fulfilling Revised Ghent Criteria. Mitral valve prolapse was defined as superior mitral leaflets' displacement ≥2mm beyond the mitral annulus. MAD was diagnosed by echocardiography and quantified as the distance from the ventricular myocardium to the hinge point of the posterior mitral leaflet (Figure, left panel).
Results
We included 111 patients with Marfan syndrome and 66 patients with Loyes-Dietz syndrome (age 26 [IQR, 13–39] years, 53% male). Mitral valve prolapse was observed in 54 (31%) and MAD in 74 (42%) patients. Longitudinal MAD distance was 8 (IQR, 6–10) mm. Forty-six (85%) patients with mitral valve prolapse had concomitant MAD, p |
| doi_str_mv | 10.1093/ehjci/ehaa946.2005 |
| format | Article |
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Background
Mitral valve prolapse is a common finding in patients with Marfan and Loyes-Dietz syndrome. Mitral annulus disjunction (MAD), an atrial displacement of the hinge point of the mitral valve, has previously been associated with non-syndromic MVP and life-threatening ventricular arrhythmias.
Purpose
We aimed to assess the prevalence of MAD in patients with Marfan and Loyes-Dietz syndrome and relate the presence of MAD to mitral valve prolapse.
Methods
We included genotype positive patients with Marfan and Loyes-Dietz syndrome, and patients with Marfan syndrome fulfilling Revised Ghent Criteria. Mitral valve prolapse was defined as superior mitral leaflets' displacement ≥2mm beyond the mitral annulus. MAD was diagnosed by echocardiography and quantified as the distance from the ventricular myocardium to the hinge point of the posterior mitral leaflet (Figure, left panel).
Results
We included 111 patients with Marfan syndrome and 66 patients with Loyes-Dietz syndrome (age 26 [IQR, 13–39] years, 53% male). Mitral valve prolapse was observed in 54 (31%) and MAD in 74 (42%) patients. Longitudinal MAD distance was 8 (IQR, 6–10) mm. Forty-six (85%) patients with mitral valve prolapse had concomitant MAD, p<0.001 (Figure, right panel).
Conclusion
Mitral valve prolapse was observed in one third of patients with Marfan and Loyes-Dietz syndrome. MAD was highly prevalent and found in 42% of all patients and in 85% of those with mitral valve prolapse. Presence of mitral valve prolapse in Marfan and Loyes-Dietz syndrome should raise awareness of MAD which may be of importance in follow-up and monitoring of these patients.
Funding Acknowledgement
Type of funding source: Public Institution(s). Main funding source(s): Health Authority of South Eastern Norway</description><identifier>ISSN: 0195-668X</identifier><identifier>EISSN: 1522-9645</identifier><identifier>DOI: 10.1093/ehjci/ehaa946.2005</identifier><language>eng</language><publisher>Oxford University Press</publisher><ispartof>European heart journal, 2020-11, Vol.41 (Supplement_2)</ispartof><rights>Published on behalf of the European Society of Cardiology. All rights reserved. © The Author(s) 2020. For permissions, please email: journals.permissions@oup.com. 2020</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><link.rule.ids>314,780,784,27924,27925</link.rule.ids></links><search><creatorcontrib>Chivulescu, M</creatorcontrib><creatorcontrib>Krohg-Sorensen, K</creatorcontrib><creatorcontrib>Scheirlynk, E</creatorcontrib><creatorcontrib>Lindberg, B</creatorcontrib><creatorcontrib>Dejgaard, L</creatorcontrib><creatorcontrib>Lie, O</creatorcontrib><creatorcontrib>Helle-Valle, T</creatorcontrib><creatorcontrib>Skjolsvik, E</creatorcontrib><creatorcontrib>Aabel, E</creatorcontrib><creatorcontrib>Hunt, T</creatorcontrib><creatorcontrib>Estensen, M</creatorcontrib><creatorcontrib>Edvardsen, T</creatorcontrib><creatorcontrib>Lingaas, P</creatorcontrib><creatorcontrib>Haugaa, K</creatorcontrib><title>Mitral annulus disjunction is highly prevalent in patients with Marfan syndrome and Loyes-Dietz syndrome</title><title>European heart journal</title><description>Abstract
Background
Mitral valve prolapse is a common finding in patients with Marfan and Loyes-Dietz syndrome. Mitral annulus disjunction (MAD), an atrial displacement of the hinge point of the mitral valve, has previously been associated with non-syndromic MVP and life-threatening ventricular arrhythmias.
Purpose
We aimed to assess the prevalence of MAD in patients with Marfan and Loyes-Dietz syndrome and relate the presence of MAD to mitral valve prolapse.
Methods
We included genotype positive patients with Marfan and Loyes-Dietz syndrome, and patients with Marfan syndrome fulfilling Revised Ghent Criteria. Mitral valve prolapse was defined as superior mitral leaflets' displacement ≥2mm beyond the mitral annulus. MAD was diagnosed by echocardiography and quantified as the distance from the ventricular myocardium to the hinge point of the posterior mitral leaflet (Figure, left panel).
Results
We included 111 patients with Marfan syndrome and 66 patients with Loyes-Dietz syndrome (age 26 [IQR, 13–39] years, 53% male). Mitral valve prolapse was observed in 54 (31%) and MAD in 74 (42%) patients. Longitudinal MAD distance was 8 (IQR, 6–10) mm. Forty-six (85%) patients with mitral valve prolapse had concomitant MAD, p<0.001 (Figure, right panel).
Conclusion
Mitral valve prolapse was observed in one third of patients with Marfan and Loyes-Dietz syndrome. MAD was highly prevalent and found in 42% of all patients and in 85% of those with mitral valve prolapse. Presence of mitral valve prolapse in Marfan and Loyes-Dietz syndrome should raise awareness of MAD which may be of importance in follow-up and monitoring of these patients.
Funding Acknowledgement
Type of funding source: Public Institution(s). Main funding source(s): Health Authority of South Eastern Norway</description><issn>0195-668X</issn><issn>1522-9645</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2020</creationdate><recordtype>article</recordtype><recordid>eNqNkLtOwzAUhi0EEqXwAkx-ARfbiU08onIpUiqWDmyR4xwTR6kT2QkoPD0prZhZzn-k_zJ8CN0yumJUJXdQN8bNV2uVyhWnVJyhBROcEyVTcY4WlClBpMzeL9FVjA2lNJNMLlC9dUPQLdbej-0YceViM3ozuM5jF3HtPup2wn2AT92CH7DzuNeDm9-Iv9xQ460OVnscJ1-Fbg_zUIXzboJIHh0M33_GNbqwuo1wc9Il2j0_7dYbkr-9vK4fcmIyJggIYayo0tIIqhQHYyyUttSl4ZlhVSo0T6XkIEuQkFgG9l4llEJlVJqViiZLxI-zJnQxBrBFH9xeh6lgtDigKn5RFSdUxQHVXCLHUjf2_8n_AOCWccU</recordid><startdate>20201101</startdate><enddate>20201101</enddate><creator>Chivulescu, M</creator><creator>Krohg-Sorensen, K</creator><creator>Scheirlynk, E</creator><creator>Lindberg, B</creator><creator>Dejgaard, L</creator><creator>Lie, O</creator><creator>Helle-Valle, T</creator><creator>Skjolsvik, E</creator><creator>Aabel, E</creator><creator>Hunt, T</creator><creator>Estensen, M</creator><creator>Edvardsen, T</creator><creator>Lingaas, P</creator><creator>Haugaa, K</creator><general>Oxford University Press</general><scope>AAYXX</scope><scope>CITATION</scope></search><sort><creationdate>20201101</creationdate><title>Mitral annulus disjunction is highly prevalent in patients with Marfan syndrome and Loyes-Dietz syndrome</title><author>Chivulescu, M ; Krohg-Sorensen, K ; Scheirlynk, E ; Lindberg, B ; Dejgaard, L ; Lie, O ; Helle-Valle, T ; Skjolsvik, E ; Aabel, E ; Hunt, T ; Estensen, M ; Edvardsen, T ; Lingaas, P ; Haugaa, K</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c815-e55cf5d4bc50992eccfebfbabc28c1d45a24662e6be6e3f1ef79300edc948b903</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2020</creationdate><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Chivulescu, M</creatorcontrib><creatorcontrib>Krohg-Sorensen, K</creatorcontrib><creatorcontrib>Scheirlynk, E</creatorcontrib><creatorcontrib>Lindberg, B</creatorcontrib><creatorcontrib>Dejgaard, L</creatorcontrib><creatorcontrib>Lie, O</creatorcontrib><creatorcontrib>Helle-Valle, T</creatorcontrib><creatorcontrib>Skjolsvik, E</creatorcontrib><creatorcontrib>Aabel, E</creatorcontrib><creatorcontrib>Hunt, T</creatorcontrib><creatorcontrib>Estensen, M</creatorcontrib><creatorcontrib>Edvardsen, T</creatorcontrib><creatorcontrib>Lingaas, P</creatorcontrib><creatorcontrib>Haugaa, K</creatorcontrib><collection>CrossRef</collection><jtitle>European heart journal</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Chivulescu, M</au><au>Krohg-Sorensen, K</au><au>Scheirlynk, E</au><au>Lindberg, B</au><au>Dejgaard, L</au><au>Lie, O</au><au>Helle-Valle, T</au><au>Skjolsvik, E</au><au>Aabel, E</au><au>Hunt, T</au><au>Estensen, M</au><au>Edvardsen, T</au><au>Lingaas, P</au><au>Haugaa, K</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Mitral annulus disjunction is highly prevalent in patients with Marfan syndrome and Loyes-Dietz syndrome</atitle><jtitle>European heart journal</jtitle><date>2020-11-01</date><risdate>2020</risdate><volume>41</volume><issue>Supplement_2</issue><issn>0195-668X</issn><eissn>1522-9645</eissn><abstract>Abstract
Background
Mitral valve prolapse is a common finding in patients with Marfan and Loyes-Dietz syndrome. Mitral annulus disjunction (MAD), an atrial displacement of the hinge point of the mitral valve, has previously been associated with non-syndromic MVP and life-threatening ventricular arrhythmias.
Purpose
We aimed to assess the prevalence of MAD in patients with Marfan and Loyes-Dietz syndrome and relate the presence of MAD to mitral valve prolapse.
Methods
We included genotype positive patients with Marfan and Loyes-Dietz syndrome, and patients with Marfan syndrome fulfilling Revised Ghent Criteria. Mitral valve prolapse was defined as superior mitral leaflets' displacement ≥2mm beyond the mitral annulus. MAD was diagnosed by echocardiography and quantified as the distance from the ventricular myocardium to the hinge point of the posterior mitral leaflet (Figure, left panel).
Results
We included 111 patients with Marfan syndrome and 66 patients with Loyes-Dietz syndrome (age 26 [IQR, 13–39] years, 53% male). Mitral valve prolapse was observed in 54 (31%) and MAD in 74 (42%) patients. Longitudinal MAD distance was 8 (IQR, 6–10) mm. Forty-six (85%) patients with mitral valve prolapse had concomitant MAD, p<0.001 (Figure, right panel).
Conclusion
Mitral valve prolapse was observed in one third of patients with Marfan and Loyes-Dietz syndrome. MAD was highly prevalent and found in 42% of all patients and in 85% of those with mitral valve prolapse. Presence of mitral valve prolapse in Marfan and Loyes-Dietz syndrome should raise awareness of MAD which may be of importance in follow-up and monitoring of these patients.
Funding Acknowledgement
Type of funding source: Public Institution(s). Main funding source(s): Health Authority of South Eastern Norway</abstract><pub>Oxford University Press</pub><doi>10.1093/ehjci/ehaa946.2005</doi></addata></record> |
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| source | Elektronische Zeitschriftenbibliothek - Frei zugängliche E-Journals; Oxford University Press Journals All Titles (1996-Current); Alma/SFX Local Collection |
| title | Mitral annulus disjunction is highly prevalent in patients with Marfan syndrome and Loyes-Dietz syndrome |
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