Mitral annular disjunction and Pickelhaube sign in children with mitral valve prolapse: A prospective cohort study
[Display omitted] •Prevalence and consequences of MAD in children are unreported.•MAD is common in children with MVP.•Pickelhaube sign is a marker of MAD in children with MVP.•Pickelhaube sign is significantly related to VAs in children with MVP.•Myxomatous mitral valve is significantly related to V...
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| Veröffentlicht in: | Archives of cardiovascular diseases 2023-11, Vol.116 (11), p.514-522 |
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| creator | Vaksmann, Guy Bouzguenda, Ivan Guillaume, Marie-Paule Gras, Pauline Silvestri, Valentina Richard, Adélaïde |
| description | [Display omitted]
•Prevalence and consequences of MAD in children are unreported.•MAD is common in children with MVP.•Pickelhaube sign is a marker of MAD in children with MVP.•Pickelhaube sign is significantly related to VAs in children with MVP.•Myxomatous mitral valve is significantly related to VAs in children with MVP.•Myocardial fibrosis seen on CMR imaging in children with Pickelhaube sign and VAs.
Mitral annular disjunction (MAD) and the Pickelhaube sign are identified as risk factors for malignant ventricular arrhythmias (VAs) and sudden cardiac death in adults with mitral valve prolapse (MVP); their prevalence and consequences in children have never been studied.
To determine the proportion of MAD in children with MVP, and its potential link with VAs.
A cohort of 49 consecutive children (mean age 12.8±3.0 years; 33 females) with MVP and comprehensive clinical arrhythmia (24-hour monitoring) and Doppler echocardiographic characterization, including pulsed-wave tissue Doppler (PWTD) of the lateral mitral annulus, was identified. The relationship between clinical and echocardiographic data and presence of VAs was studied.
MAD was common (n=25; 51%). Only five patients had significant VAs (Lown grade>2) characterized by polymorphic premature ventricular contractions or couplets. MAD was not associated with VAs on 24-hour Holter monitoring, but an association was found between VAs and spiked high-velocity midsystolic signal>16cm/s on PWTD (Pickelhaube sign) (P=0.004), myxomatous mitral valve (P=0.004) and left ventricular dilatation (P=0.01). T-wave inversion in inferolateral leads on electrocardiogram was more frequent in patients with versus without the Pickelhaube sign (P=0.03). No difference was found between patients with or without MAD regarding sex, history of palpitation, severity of mitral regurgitation, aortic root diameter and incidence of connective tissue disorders. Myocardial fibrosis was detected in two of three patients who underwent a complementary cardiac magnetic resonance examination.
MAD is common in children with MVP; its presence was not associated with significant VAs on 24-hour Holter monitoring, but the Pickelhaube sign and presence of myxomatous mitral valve may help to detect patients prone to significant VAs. Myocardial fibrosis can be detected by cardiac magnetic resonance in children with significant VAs. |
| doi_str_mv | 10.1016/j.acvd.2023.08.004 |
| format | Article |
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•Prevalence and consequences of MAD in children are unreported.•MAD is common in children with MVP.•Pickelhaube sign is a marker of MAD in children with MVP.•Pickelhaube sign is significantly related to VAs in children with MVP.•Myxomatous mitral valve is significantly related to VAs in children with MVP.•Myocardial fibrosis seen on CMR imaging in children with Pickelhaube sign and VAs.
Mitral annular disjunction (MAD) and the Pickelhaube sign are identified as risk factors for malignant ventricular arrhythmias (VAs) and sudden cardiac death in adults with mitral valve prolapse (MVP); their prevalence and consequences in children have never been studied.
To determine the proportion of MAD in children with MVP, and its potential link with VAs.
A cohort of 49 consecutive children (mean age 12.8±3.0 years; 33 females) with MVP and comprehensive clinical arrhythmia (24-hour monitoring) and Doppler echocardiographic characterization, including pulsed-wave tissue Doppler (PWTD) of the lateral mitral annulus, was identified. The relationship between clinical and echocardiographic data and presence of VAs was studied.
MAD was common (n=25; 51%). Only five patients had significant VAs (Lown grade>2) characterized by polymorphic premature ventricular contractions or couplets. MAD was not associated with VAs on 24-hour Holter monitoring, but an association was found between VAs and spiked high-velocity midsystolic signal>16cm/s on PWTD (Pickelhaube sign) (P=0.004), myxomatous mitral valve (P=0.004) and left ventricular dilatation (P=0.01). T-wave inversion in inferolateral leads on electrocardiogram was more frequent in patients with versus without the Pickelhaube sign (P=0.03). No difference was found between patients with or without MAD regarding sex, history of palpitation, severity of mitral regurgitation, aortic root diameter and incidence of connective tissue disorders. Myocardial fibrosis was detected in two of three patients who underwent a complementary cardiac magnetic resonance examination.
MAD is common in children with MVP; its presence was not associated with significant VAs on 24-hour Holter monitoring, but the Pickelhaube sign and presence of myxomatous mitral valve may help to detect patients prone to significant VAs. Myocardial fibrosis can be detected by cardiac magnetic resonance in children with significant VAs.</description><identifier>ISSN: 1875-2136</identifier><identifier>ISSN: 1875-2128</identifier><identifier>EISSN: 1875-2128</identifier><identifier>DOI: 10.1016/j.acvd.2023.08.004</identifier><identifier>PMID: 37775440</identifier><language>eng</language><publisher>Netherlands: Elsevier Masson SAS</publisher><subject>Adolescent ; Age Factors ; Child ; Children ; Death, Sudden, Cardiac - epidemiology ; Death, Sudden, Cardiac - etiology ; Echocardiography, Doppler, Pulsed ; Electrocardiography, Ambulatory ; Female ; Humans ; Male ; Mitral annular disjunction ; Mitral Valve - diagnostic imaging ; Mitral Valve - physiopathology ; Mitral valve prolapse ; Mitral Valve Prolapse - complications ; Mitral Valve Prolapse - diagnostic imaging ; Mitral Valve Prolapse - epidemiology ; Mitral Valve Prolapse - physiopathology ; Myocardial fibrosis ; Pickelhaube sign ; Predictive Value of Tests ; Prevalence ; Prospective Studies ; Risk Factors ; Ventricular Premature Complexes - diagnosis ; Ventricular Premature Complexes - diagnostic imaging ; Ventricular Premature Complexes - epidemiology ; Ventricular Premature Complexes - etiology ; Ventricular Premature Complexes - physiopathology</subject><ispartof>Archives of cardiovascular diseases, 2023-11, Vol.116 (11), p.514-522</ispartof><rights>2023 Elsevier Masson SAS</rights><rights>Copyright © 2023 Elsevier Masson SAS. All rights reserved.</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><cites>FETCH-LOGICAL-c307t-366ea1abebe13bc06c062a106a4b1c1ad9ec10d23ba5e77701999557a67003e13</cites><orcidid>0000-0002-2061-1437</orcidid></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><link.rule.ids>314,780,784,27924,27925</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/37775440$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Vaksmann, Guy</creatorcontrib><creatorcontrib>Bouzguenda, Ivan</creatorcontrib><creatorcontrib>Guillaume, Marie-Paule</creatorcontrib><creatorcontrib>Gras, Pauline</creatorcontrib><creatorcontrib>Silvestri, Valentina</creatorcontrib><creatorcontrib>Richard, Adélaïde</creatorcontrib><title>Mitral annular disjunction and Pickelhaube sign in children with mitral valve prolapse: A prospective cohort study</title><title>Archives of cardiovascular diseases</title><addtitle>Arch Cardiovasc Dis</addtitle><description>[Display omitted]
•Prevalence and consequences of MAD in children are unreported.•MAD is common in children with MVP.•Pickelhaube sign is a marker of MAD in children with MVP.•Pickelhaube sign is significantly related to VAs in children with MVP.•Myxomatous mitral valve is significantly related to VAs in children with MVP.•Myocardial fibrosis seen on CMR imaging in children with Pickelhaube sign and VAs.
Mitral annular disjunction (MAD) and the Pickelhaube sign are identified as risk factors for malignant ventricular arrhythmias (VAs) and sudden cardiac death in adults with mitral valve prolapse (MVP); their prevalence and consequences in children have never been studied.
To determine the proportion of MAD in children with MVP, and its potential link with VAs.
A cohort of 49 consecutive children (mean age 12.8±3.0 years; 33 females) with MVP and comprehensive clinical arrhythmia (24-hour monitoring) and Doppler echocardiographic characterization, including pulsed-wave tissue Doppler (PWTD) of the lateral mitral annulus, was identified. The relationship between clinical and echocardiographic data and presence of VAs was studied.
MAD was common (n=25; 51%). Only five patients had significant VAs (Lown grade>2) characterized by polymorphic premature ventricular contractions or couplets. MAD was not associated with VAs on 24-hour Holter monitoring, but an association was found between VAs and spiked high-velocity midsystolic signal>16cm/s on PWTD (Pickelhaube sign) (P=0.004), myxomatous mitral valve (P=0.004) and left ventricular dilatation (P=0.01). T-wave inversion in inferolateral leads on electrocardiogram was more frequent in patients with versus without the Pickelhaube sign (P=0.03). No difference was found between patients with or without MAD regarding sex, history of palpitation, severity of mitral regurgitation, aortic root diameter and incidence of connective tissue disorders. Myocardial fibrosis was detected in two of three patients who underwent a complementary cardiac magnetic resonance examination.
MAD is common in children with MVP; its presence was not associated with significant VAs on 24-hour Holter monitoring, but the Pickelhaube sign and presence of myxomatous mitral valve may help to detect patients prone to significant VAs. Myocardial fibrosis can be detected by cardiac magnetic resonance in children with significant VAs.</description><subject>Adolescent</subject><subject>Age Factors</subject><subject>Child</subject><subject>Children</subject><subject>Death, Sudden, Cardiac - epidemiology</subject><subject>Death, Sudden, Cardiac - etiology</subject><subject>Echocardiography, Doppler, Pulsed</subject><subject>Electrocardiography, Ambulatory</subject><subject>Female</subject><subject>Humans</subject><subject>Male</subject><subject>Mitral annular disjunction</subject><subject>Mitral Valve - diagnostic imaging</subject><subject>Mitral Valve - physiopathology</subject><subject>Mitral valve prolapse</subject><subject>Mitral Valve Prolapse - complications</subject><subject>Mitral Valve Prolapse - diagnostic imaging</subject><subject>Mitral Valve Prolapse - epidemiology</subject><subject>Mitral Valve Prolapse - physiopathology</subject><subject>Myocardial fibrosis</subject><subject>Pickelhaube sign</subject><subject>Predictive Value of Tests</subject><subject>Prevalence</subject><subject>Prospective Studies</subject><subject>Risk Factors</subject><subject>Ventricular Premature Complexes - diagnosis</subject><subject>Ventricular Premature Complexes - diagnostic imaging</subject><subject>Ventricular Premature Complexes - epidemiology</subject><subject>Ventricular Premature Complexes - etiology</subject><subject>Ventricular Premature Complexes - physiopathology</subject><issn>1875-2136</issn><issn>1875-2128</issn><issn>1875-2128</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2023</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNp9kE1rGzEQhkVpaT7_QA9Bx168HWm90m7JJYQmKSQ0h_QsZqVxLUfWOtKui_99ZZzmGBBoGN55mHkY-yKgEiDUt1WFdusqCbKuoK0A5h_YsWh1M5NCth_f6lodsZOcVwBKaq0-s6Naa93M53DM0oMfEwaOMU4BE3c-r6ZoRz_E0nP80dtnCkuceuLZ_4ncR26XPrhEkf_145KvD4Athi3xTRoCbjJ951f7Om-ooErfDsshjTyPk9udsU8LDJnOX_9T9vvmx9P13ez-1-3P66v7ma1Bj7NaKUKBPfUk6t6CKk-iAIXzXliBriMrwMm6x4bKPSC6rmsajUoD1GXmlH09cMsiLxPl0ax9thQCRhqmbGSroevaQixReYjasnNOtDCb5NeYdkaA2bs2K7N3bfauDbSmuC5DF6_8qV-Texv5L7cELg8BKlduPSWTradoyflUvBg3-Pf4_wCgiJGi</recordid><startdate>202311</startdate><enddate>202311</enddate><creator>Vaksmann, Guy</creator><creator>Bouzguenda, Ivan</creator><creator>Guillaume, Marie-Paule</creator><creator>Gras, Pauline</creator><creator>Silvestri, Valentina</creator><creator>Richard, Adélaïde</creator><general>Elsevier Masson SAS</general><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7X8</scope><orcidid>https://orcid.org/0000-0002-2061-1437</orcidid></search><sort><creationdate>202311</creationdate><title>Mitral annular disjunction and Pickelhaube sign in children with mitral valve prolapse: A prospective cohort study</title><author>Vaksmann, Guy ; Bouzguenda, Ivan ; Guillaume, Marie-Paule ; Gras, Pauline ; Silvestri, Valentina ; Richard, Adélaïde</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c307t-366ea1abebe13bc06c062a106a4b1c1ad9ec10d23ba5e77701999557a67003e13</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2023</creationdate><topic>Adolescent</topic><topic>Age Factors</topic><topic>Child</topic><topic>Children</topic><topic>Death, Sudden, Cardiac - epidemiology</topic><topic>Death, Sudden, Cardiac - etiology</topic><topic>Echocardiography, Doppler, Pulsed</topic><topic>Electrocardiography, Ambulatory</topic><topic>Female</topic><topic>Humans</topic><topic>Male</topic><topic>Mitral annular disjunction</topic><topic>Mitral Valve - diagnostic imaging</topic><topic>Mitral Valve - physiopathology</topic><topic>Mitral valve prolapse</topic><topic>Mitral Valve Prolapse - complications</topic><topic>Mitral Valve Prolapse - diagnostic imaging</topic><topic>Mitral Valve Prolapse - epidemiology</topic><topic>Mitral Valve Prolapse - physiopathology</topic><topic>Myocardial fibrosis</topic><topic>Pickelhaube sign</topic><topic>Predictive Value of Tests</topic><topic>Prevalence</topic><topic>Prospective Studies</topic><topic>Risk Factors</topic><topic>Ventricular Premature Complexes - diagnosis</topic><topic>Ventricular Premature Complexes - diagnostic imaging</topic><topic>Ventricular Premature Complexes - epidemiology</topic><topic>Ventricular Premature Complexes - etiology</topic><topic>Ventricular Premature Complexes - physiopathology</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Vaksmann, Guy</creatorcontrib><creatorcontrib>Bouzguenda, Ivan</creatorcontrib><creatorcontrib>Guillaume, Marie-Paule</creatorcontrib><creatorcontrib>Gras, Pauline</creatorcontrib><creatorcontrib>Silvestri, Valentina</creatorcontrib><creatorcontrib>Richard, Adélaïde</creatorcontrib><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>MEDLINE - Academic</collection><jtitle>Archives of cardiovascular diseases</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Vaksmann, Guy</au><au>Bouzguenda, Ivan</au><au>Guillaume, Marie-Paule</au><au>Gras, Pauline</au><au>Silvestri, Valentina</au><au>Richard, Adélaïde</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Mitral annular disjunction and Pickelhaube sign in children with mitral valve prolapse: A prospective cohort study</atitle><jtitle>Archives of cardiovascular diseases</jtitle><addtitle>Arch Cardiovasc Dis</addtitle><date>2023-11</date><risdate>2023</risdate><volume>116</volume><issue>11</issue><spage>514</spage><epage>522</epage><pages>514-522</pages><issn>1875-2136</issn><issn>1875-2128</issn><eissn>1875-2128</eissn><abstract>[Display omitted]
•Prevalence and consequences of MAD in children are unreported.•MAD is common in children with MVP.•Pickelhaube sign is a marker of MAD in children with MVP.•Pickelhaube sign is significantly related to VAs in children with MVP.•Myxomatous mitral valve is significantly related to VAs in children with MVP.•Myocardial fibrosis seen on CMR imaging in children with Pickelhaube sign and VAs.
Mitral annular disjunction (MAD) and the Pickelhaube sign are identified as risk factors for malignant ventricular arrhythmias (VAs) and sudden cardiac death in adults with mitral valve prolapse (MVP); their prevalence and consequences in children have never been studied.
To determine the proportion of MAD in children with MVP, and its potential link with VAs.
A cohort of 49 consecutive children (mean age 12.8±3.0 years; 33 females) with MVP and comprehensive clinical arrhythmia (24-hour monitoring) and Doppler echocardiographic characterization, including pulsed-wave tissue Doppler (PWTD) of the lateral mitral annulus, was identified. The relationship between clinical and echocardiographic data and presence of VAs was studied.
MAD was common (n=25; 51%). Only five patients had significant VAs (Lown grade>2) characterized by polymorphic premature ventricular contractions or couplets. MAD was not associated with VAs on 24-hour Holter monitoring, but an association was found between VAs and spiked high-velocity midsystolic signal>16cm/s on PWTD (Pickelhaube sign) (P=0.004), myxomatous mitral valve (P=0.004) and left ventricular dilatation (P=0.01). T-wave inversion in inferolateral leads on electrocardiogram was more frequent in patients with versus without the Pickelhaube sign (P=0.03). No difference was found between patients with or without MAD regarding sex, history of palpitation, severity of mitral regurgitation, aortic root diameter and incidence of connective tissue disorders. Myocardial fibrosis was detected in two of three patients who underwent a complementary cardiac magnetic resonance examination.
MAD is common in children with MVP; its presence was not associated with significant VAs on 24-hour Holter monitoring, but the Pickelhaube sign and presence of myxomatous mitral valve may help to detect patients prone to significant VAs. Myocardial fibrosis can be detected by cardiac magnetic resonance in children with significant VAs.</abstract><cop>Netherlands</cop><pub>Elsevier Masson SAS</pub><pmid>37775440</pmid><doi>10.1016/j.acvd.2023.08.004</doi><tpages>9</tpages><orcidid>https://orcid.org/0000-0002-2061-1437</orcidid></addata></record> |
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| ispartof | Archives of cardiovascular diseases, 2023-11, Vol.116 (11), p.514-522 |
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| source | MEDLINE; Elsevier ScienceDirect Journals Complete; Elektronische Zeitschriftenbibliothek - Frei zugängliche E-Journals; Alma/SFX Local Collection |
| subjects | Adolescent Age Factors Child Children Death, Sudden, Cardiac - epidemiology Death, Sudden, Cardiac - etiology Echocardiography, Doppler, Pulsed Electrocardiography, Ambulatory Female Humans Male Mitral annular disjunction Mitral Valve - diagnostic imaging Mitral Valve - physiopathology Mitral valve prolapse Mitral Valve Prolapse - complications Mitral Valve Prolapse - diagnostic imaging Mitral Valve Prolapse - epidemiology Mitral Valve Prolapse - physiopathology Myocardial fibrosis Pickelhaube sign Predictive Value of Tests Prevalence Prospective Studies Risk Factors Ventricular Premature Complexes - diagnosis Ventricular Premature Complexes - diagnostic imaging Ventricular Premature Complexes - epidemiology Ventricular Premature Complexes - etiology Ventricular Premature Complexes - physiopathology |
| title | Mitral annular disjunction and Pickelhaube sign in children with mitral valve prolapse: A prospective cohort study |
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