Increased nocturnal heart rate and wave reflection are early markers of cardiovascular disease in Williams-Beuren syndrome children
Williams-Beuren syndrome (WBS) is a genetic disorder that involves elastin gene causing cardiovascular abnormalities and increased risk. However, data on arterial function in these patients are only few and conflicting. Aim of this study was to evaluate dynamic behaviour of central and peripheral bl...
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| Veröffentlicht in: | Journal of hypertension 2015-04, Vol.33 (4), p.804-809 |
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| creator | Maloberti, Alessandro Cesana, Francesca Hametner, Bernhard Dozio, Dario Villa, Paolo Hulpke-Wette, Martin Schwarz, Achim Selicorni, Angelo Wassertheurer, Siegfried Mancia, Giuseppe Giannattasio, Cristina |
| description | Williams-Beuren syndrome (WBS) is a genetic disorder that involves elastin gene causing cardiovascular abnormalities and increased risk. However, data on arterial function in these patients are only few and conflicting. Aim of this study was to evaluate dynamic behaviour of central and peripheral blood pressure (BP) and arterial stiffness parameters early in the course of WBS.
We enrolled 19 WBS paediatric patients (age 13 ± 4 years) and 23 age, height and BP-matched controls (10 ± 4 years). We evaluated 24-h ambulatory BP values via an ambulatory blood pressure monitoring (ABPM) system (Mobil-O-Graph) also capable to calculate 24-h central BP and 24-h arterial stiffness parameters. Carotid-femoral PWV (cf-PWV) was assessed in all WBS individuals (Complior).
BP values were similar in WBS and control, during the daytime and the night-time. The same behaviour applies to 24-h central BP. However, during the night, WBS showed heart rate values (HR; 78 ± 10 vs. 71 ± 9 bpm; P |
| doi_str_mv | 10.1097/HJH.0000000000000454 |
| format | Article |
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We enrolled 19 WBS paediatric patients (age 13 ± 4 years) and 23 age, height and BP-matched controls (10 ± 4 years). We evaluated 24-h ambulatory BP values via an ambulatory blood pressure monitoring (ABPM) system (Mobil-O-Graph) also capable to calculate 24-h central BP and 24-h arterial stiffness parameters. Carotid-femoral PWV (cf-PWV) was assessed in all WBS individuals (Complior).
BP values were similar in WBS and control, during the daytime and the night-time. The same behaviour applies to 24-h central BP. However, during the night, WBS showed heart rate values (HR; 78 ± 10 vs. 71 ± 9 bpm; P < 0.03), augmentation index (Aix; 24.6 ± 13.5% vs. 16.5 ± 8.9%; P = 0.03) and reflection magnitude (68 5.8 vs. 63.5 8.1; P = 0.02) higher than controls. The HR, Aix and reflection magnitude reduction in the day-night shift was lower in WBS than in controls. Cf-PWV in WBS children did not differ when compared with their normalized expected value.
In WBS children, the higher night-time HR, Aix and reflection magnitude and their impaired physiological reduction in the day-night shift suggests an abnormal sympathetic cardiovascular control, an augmented wave reflection and an increase in small arteries resistance. These alterations possibly due to a sympathetic overactivity can be regarded as earlier hallmarks of cardiovascular dysfunction in these patients.</description><identifier>ISSN: 0263-6352</identifier><identifier>EISSN: 1473-5598</identifier><identifier>DOI: 10.1097/HJH.0000000000000454</identifier><identifier>PMID: 25915885</identifier><language>eng</language><publisher>England</publisher><subject>Adolescent ; Blood Pressure ; Blood Pressure Determination ; Blood Pressure Monitoring, Ambulatory ; Cardiovascular Diseases - etiology ; Case-Control Studies ; Child ; Female ; Heart Rate ; Humans ; Male ; Vascular Stiffness ; Williams Syndrome - complications ; Williams Syndrome - physiopathology</subject><ispartof>Journal of hypertension, 2015-04, Vol.33 (4), p.804-809</ispartof><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c237t-6e11a8c479b964aab39733eaa4401cb7239d3a9ad513221f8698f9de9f34af113</citedby><cites>FETCH-LOGICAL-c237t-6e11a8c479b964aab39733eaa4401cb7239d3a9ad513221f8698f9de9f34af113</cites></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/25915885$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Maloberti, Alessandro</creatorcontrib><creatorcontrib>Cesana, Francesca</creatorcontrib><creatorcontrib>Hametner, Bernhard</creatorcontrib><creatorcontrib>Dozio, Dario</creatorcontrib><creatorcontrib>Villa, Paolo</creatorcontrib><creatorcontrib>Hulpke-Wette, Martin</creatorcontrib><creatorcontrib>Schwarz, Achim</creatorcontrib><creatorcontrib>Selicorni, Angelo</creatorcontrib><creatorcontrib>Wassertheurer, Siegfried</creatorcontrib><creatorcontrib>Mancia, Giuseppe</creatorcontrib><creatorcontrib>Giannattasio, Cristina</creatorcontrib><title>Increased nocturnal heart rate and wave reflection are early markers of cardiovascular disease in Williams-Beuren syndrome children</title><title>Journal of hypertension</title><addtitle>J Hypertens</addtitle><description>Williams-Beuren syndrome (WBS) is a genetic disorder that involves elastin gene causing cardiovascular abnormalities and increased risk. However, data on arterial function in these patients are only few and conflicting. Aim of this study was to evaluate dynamic behaviour of central and peripheral blood pressure (BP) and arterial stiffness parameters early in the course of WBS.
We enrolled 19 WBS paediatric patients (age 13 ± 4 years) and 23 age, height and BP-matched controls (10 ± 4 years). We evaluated 24-h ambulatory BP values via an ambulatory blood pressure monitoring (ABPM) system (Mobil-O-Graph) also capable to calculate 24-h central BP and 24-h arterial stiffness parameters. Carotid-femoral PWV (cf-PWV) was assessed in all WBS individuals (Complior).
BP values were similar in WBS and control, during the daytime and the night-time. The same behaviour applies to 24-h central BP. However, during the night, WBS showed heart rate values (HR; 78 ± 10 vs. 71 ± 9 bpm; P < 0.03), augmentation index (Aix; 24.6 ± 13.5% vs. 16.5 ± 8.9%; P = 0.03) and reflection magnitude (68 5.8 vs. 63.5 8.1; P = 0.02) higher than controls. The HR, Aix and reflection magnitude reduction in the day-night shift was lower in WBS than in controls. Cf-PWV in WBS children did not differ when compared with their normalized expected value.
In WBS children, the higher night-time HR, Aix and reflection magnitude and their impaired physiological reduction in the day-night shift suggests an abnormal sympathetic cardiovascular control, an augmented wave reflection and an increase in small arteries resistance. These alterations possibly due to a sympathetic overactivity can be regarded as earlier hallmarks of cardiovascular dysfunction in these patients.</description><subject>Adolescent</subject><subject>Blood Pressure</subject><subject>Blood Pressure Determination</subject><subject>Blood Pressure Monitoring, Ambulatory</subject><subject>Cardiovascular Diseases - etiology</subject><subject>Case-Control Studies</subject><subject>Child</subject><subject>Female</subject><subject>Heart Rate</subject><subject>Humans</subject><subject>Male</subject><subject>Vascular Stiffness</subject><subject>Williams Syndrome - complications</subject><subject>Williams Syndrome - physiopathology</subject><issn>0263-6352</issn><issn>1473-5598</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2015</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNpdkEFLxDAQhYMouq7-A5EcvVSbJk2bo4q6yoIXxWOZTaYYTdM1aVf27B83siriHGZgeG-G9xFyxPJTlqvqbHY3O83_lijFFpkwUfGsLFW9TSZ5IXkmeVnskf0YX5KmVhXfJXtFqVhZ1-WEfNx6HRAiGup7PYzBg6PPCGGgAQak4A19hxXSgK1DPdjeUwhIk8KtaQfhFUOkfUs1BGP7FUQ9OgjU2Ph1lVpPn6xzFrqYXeAY0NO49ib0HVL9bJ1JmwOy04KLePg9p-Tx-urhcpbN729uL8_nmS54NWQSGYNai0otlBQAC57CcAQQImd6URVcGQ4KTMl4UbC2lqpulUHVcgEtY3xKTjZ3l6F_GzEOTWejRufAYz_GhslKytRymaRiI9WhjzFlb5bBprTrhuXNF_4m4W_-40-24-8P46JD82v64c0_ASfbgno</recordid><startdate>201504</startdate><enddate>201504</enddate><creator>Maloberti, Alessandro</creator><creator>Cesana, Francesca</creator><creator>Hametner, Bernhard</creator><creator>Dozio, Dario</creator><creator>Villa, Paolo</creator><creator>Hulpke-Wette, Martin</creator><creator>Schwarz, Achim</creator><creator>Selicorni, Angelo</creator><creator>Wassertheurer, Siegfried</creator><creator>Mancia, Giuseppe</creator><creator>Giannattasio, Cristina</creator><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></search><sort><creationdate>201504</creationdate><title>Increased nocturnal heart rate and wave reflection are early markers of cardiovascular disease in Williams-Beuren syndrome children</title><author>Maloberti, Alessandro ; Cesana, Francesca ; Hametner, Bernhard ; Dozio, Dario ; Villa, Paolo ; Hulpke-Wette, Martin ; Schwarz, Achim ; Selicorni, Angelo ; Wassertheurer, Siegfried ; Mancia, Giuseppe ; Giannattasio, Cristina</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c237t-6e11a8c479b964aab39733eaa4401cb7239d3a9ad513221f8698f9de9f34af113</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2015</creationdate><topic>Adolescent</topic><topic>Blood Pressure</topic><topic>Blood Pressure Determination</topic><topic>Blood Pressure Monitoring, Ambulatory</topic><topic>Cardiovascular Diseases - etiology</topic><topic>Case-Control Studies</topic><topic>Child</topic><topic>Female</topic><topic>Heart Rate</topic><topic>Humans</topic><topic>Male</topic><topic>Vascular Stiffness</topic><topic>Williams Syndrome - complications</topic><topic>Williams Syndrome - physiopathology</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Maloberti, Alessandro</creatorcontrib><creatorcontrib>Cesana, Francesca</creatorcontrib><creatorcontrib>Hametner, Bernhard</creatorcontrib><creatorcontrib>Dozio, Dario</creatorcontrib><creatorcontrib>Villa, Paolo</creatorcontrib><creatorcontrib>Hulpke-Wette, Martin</creatorcontrib><creatorcontrib>Schwarz, Achim</creatorcontrib><creatorcontrib>Selicorni, Angelo</creatorcontrib><creatorcontrib>Wassertheurer, Siegfried</creatorcontrib><creatorcontrib>Mancia, Giuseppe</creatorcontrib><creatorcontrib>Giannattasio, Cristina</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>Journal of hypertension</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Maloberti, Alessandro</au><au>Cesana, Francesca</au><au>Hametner, Bernhard</au><au>Dozio, Dario</au><au>Villa, Paolo</au><au>Hulpke-Wette, Martin</au><au>Schwarz, Achim</au><au>Selicorni, Angelo</au><au>Wassertheurer, Siegfried</au><au>Mancia, Giuseppe</au><au>Giannattasio, Cristina</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Increased nocturnal heart rate and wave reflection are early markers of cardiovascular disease in Williams-Beuren syndrome children</atitle><jtitle>Journal of hypertension</jtitle><addtitle>J Hypertens</addtitle><date>2015-04</date><risdate>2015</risdate><volume>33</volume><issue>4</issue><spage>804</spage><epage>809</epage><pages>804-809</pages><issn>0263-6352</issn><eissn>1473-5598</eissn><abstract>Williams-Beuren syndrome (WBS) is a genetic disorder that involves elastin gene causing cardiovascular abnormalities and increased risk. However, data on arterial function in these patients are only few and conflicting. Aim of this study was to evaluate dynamic behaviour of central and peripheral blood pressure (BP) and arterial stiffness parameters early in the course of WBS.
We enrolled 19 WBS paediatric patients (age 13 ± 4 years) and 23 age, height and BP-matched controls (10 ± 4 years). We evaluated 24-h ambulatory BP values via an ambulatory blood pressure monitoring (ABPM) system (Mobil-O-Graph) also capable to calculate 24-h central BP and 24-h arterial stiffness parameters. Carotid-femoral PWV (cf-PWV) was assessed in all WBS individuals (Complior).
BP values were similar in WBS and control, during the daytime and the night-time. The same behaviour applies to 24-h central BP. However, during the night, WBS showed heart rate values (HR; 78 ± 10 vs. 71 ± 9 bpm; P < 0.03), augmentation index (Aix; 24.6 ± 13.5% vs. 16.5 ± 8.9%; P = 0.03) and reflection magnitude (68 5.8 vs. 63.5 8.1; P = 0.02) higher than controls. The HR, Aix and reflection magnitude reduction in the day-night shift was lower in WBS than in controls. Cf-PWV in WBS children did not differ when compared with their normalized expected value.
In WBS children, the higher night-time HR, Aix and reflection magnitude and their impaired physiological reduction in the day-night shift suggests an abnormal sympathetic cardiovascular control, an augmented wave reflection and an increase in small arteries resistance. These alterations possibly due to a sympathetic overactivity can be regarded as earlier hallmarks of cardiovascular dysfunction in these patients.</abstract><cop>England</cop><pmid>25915885</pmid><doi>10.1097/HJH.0000000000000454</doi><tpages>6</tpages></addata></record> |
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| subjects | Adolescent Blood Pressure Blood Pressure Determination Blood Pressure Monitoring, Ambulatory Cardiovascular Diseases - etiology Case-Control Studies Child Female Heart Rate Humans Male Vascular Stiffness Williams Syndrome - complications Williams Syndrome - physiopathology |
| title | Increased nocturnal heart rate and wave reflection are early markers of cardiovascular disease in Williams-Beuren syndrome children |
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