Retrospective analysis of the effect of angiotensin II receptor blocker versus [beta]-blocker on aortic root growth in paediatric patients with Marfan syndrome

Objectives Cardiovascular pathology, including aortic root dilation at the level of sinus of Valsalva (SV), is one of the major causes of morbidity in paediatric patients with Marfan syndrome (MFS). β-Blocker (BB) is well established to slow aortic dilation in MFS. Less is known about the effectiven...

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Veröffentlicht in:Heart (British Cardiac Society) 2014-02, Vol.100 (3), p.214
Hauptverfasser: Mueller, Goetz C, Stierle, Lydia, Stark, Veronika, Steiner, Kristoffer, von Kodolitsch, Yskert, Weil, Jochen, Mir, Thomas S
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container_issue 3
container_start_page 214
container_title Heart (British Cardiac Society)
container_volume 100
creator Mueller, Goetz C
Stierle, Lydia
Stark, Veronika
Steiner, Kristoffer
von Kodolitsch, Yskert
Weil, Jochen
Mir, Thomas S
description Objectives Cardiovascular pathology, including aortic root dilation at the level of sinus of Valsalva (SV), is one of the major causes of morbidity in paediatric patients with Marfan syndrome (MFS). β-Blocker (BB) is well established to slow aortic dilation in MFS. Less is known about the effectiveness of angiotensin II receptor blocker (ARB) on aortic dilation in paediatric patients with MFS. Methods 215 patients with MFS (9.01±5.7 years) were subject to a standardised diagnostic programme. Aortic root dilation was evaluated and followed up by echocardiography. In 48 cases, BB and ARB effects on aortic root dilation were evaluated. Effect of treatment was measured by comparison of z scores of SV before and after treatment initiation. Results Treatment by ARB and BB leads to significant reduction of SV dilation (p
doi_str_mv 10.1136/heartjnl-2013-304946
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Less is known about the effectiveness of angiotensin II receptor blocker (ARB) on aortic dilation in paediatric patients with MFS. Methods 215 patients with MFS (9.01±5.7 years) were subject to a standardised diagnostic programme. Aortic root dilation was evaluated and followed up by echocardiography. In 48 cases, BB and ARB effects on aortic root dilation were evaluated. Effect of treatment was measured by comparison of z scores of SV before and after treatment initiation. Results Treatment by ARB and BB leads to significant reduction of SV dilation (p&lt;0.05). The deviation of SV enlargement from normal as expressed by the rate of change in z scores was significantly reduced by a mean difference of -0.56±0.71 z scores (p&lt;0.001) under ARB therapy and by a mean difference of -0.35±0.68 z scores (p&lt;0.05) under BB therapy. The prophylactic effect of ARB and BB on aortic root dilation is similar in both groups (p&gt;0.05). Conclusions Both concepts lead to a significant reduction of SV dilation. The effect of ARB and BB is similar. This is the first study concerning the comparison of ARB and BB in previously untreated paediatric patients with MFS. The results of the study show that both treatment strategies are beneficial in paediatric and adolescent patients.</description><identifier>ISSN: 1355-6037</identifier><identifier>EISSN: 1468-201X</identifier><identifier>DOI: 10.1136/heartjnl-2013-304946</identifier><language>eng</language><publisher>London: BMJ Publishing Group LTD</publisher><subject>Child development ; Dissection ; Marfan syndrome ; Mortality ; Pathogenesis ; Surgery ; Teenagers</subject><ispartof>Heart (British Cardiac Society), 2014-02, Vol.100 (3), p.214</ispartof><rights>Copyright: 2014 Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions</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>Mueller, Goetz C</creatorcontrib><creatorcontrib>Stierle, Lydia</creatorcontrib><creatorcontrib>Stark, Veronika</creatorcontrib><creatorcontrib>Steiner, Kristoffer</creatorcontrib><creatorcontrib>von Kodolitsch, Yskert</creatorcontrib><creatorcontrib>Weil, Jochen</creatorcontrib><creatorcontrib>Mir, Thomas S</creatorcontrib><title>Retrospective analysis of the effect of angiotensin II receptor blocker versus [beta]-blocker on aortic root growth in paediatric patients with Marfan syndrome</title><title>Heart (British Cardiac Society)</title><description>Objectives Cardiovascular pathology, including aortic root dilation at the level of sinus of Valsalva (SV), is one of the major causes of morbidity in paediatric patients with Marfan syndrome (MFS). β-Blocker (BB) is well established to slow aortic dilation in MFS. Less is known about the effectiveness of angiotensin II receptor blocker (ARB) on aortic dilation in paediatric patients with MFS. Methods 215 patients with MFS (9.01±5.7 years) were subject to a standardised diagnostic programme. Aortic root dilation was evaluated and followed up by echocardiography. In 48 cases, BB and ARB effects on aortic root dilation were evaluated. Effect of treatment was measured by comparison of z scores of SV before and after treatment initiation. Results Treatment by ARB and BB leads to significant reduction of SV dilation (p&lt;0.05). The deviation of SV enlargement from normal as expressed by the rate of change in z scores was significantly reduced by a mean difference of -0.56±0.71 z scores (p&lt;0.001) under ARB therapy and by a mean difference of -0.35±0.68 z scores (p&lt;0.05) under BB therapy. The prophylactic effect of ARB and BB on aortic root dilation is similar in both groups (p&gt;0.05). Conclusions Both concepts lead to a significant reduction of SV dilation. The effect of ARB and BB is similar. This is the first study concerning the comparison of ARB and BB in previously untreated paediatric patients with MFS. The results of the study show that both treatment strategies are beneficial in paediatric and adolescent patients.</description><subject>Child development</subject><subject>Dissection</subject><subject>Marfan syndrome</subject><subject>Mortality</subject><subject>Pathogenesis</subject><subject>Surgery</subject><subject>Teenagers</subject><issn>1355-6037</issn><issn>1468-201X</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2014</creationdate><recordtype>article</recordtype><sourceid>ABUWG</sourceid><sourceid>AFKRA</sourceid><sourceid>AZQEC</sourceid><sourceid>BENPR</sourceid><sourceid>CCPQU</sourceid><sourceid>DWQXO</sourceid><sourceid>GNUQQ</sourceid><recordid>eNqNjs1KA0EQhAdRMP68gYcGz6szmWQ3OYtiDl7EgyASJpve7MR1eu3uJORpfFUnoHdP1VVf0ZQxV87eOOfL2xYD6zp1xdA6X3g7mo7KIzNwo3JyiF6P8-3H46K0vjo1ZyJra3NpUg7M9zMqk_RYa9wihBS6vUQBakBbBGyaTA4upFUkxSQxwWwGjDX2SgyLjuoPZNgiy0bgbYEa3ou_lBIEYo01MJHCimmnLeQXfcBlDMqZ9EEjJhXYxcyeAjchgezTkukTL8xJEzrBy189N9cP9y93j0XP9LVB0fmaNpxXy9xVE1v54dBO_f9aP4leZhI</recordid><startdate>20140201</startdate><enddate>20140201</enddate><creator>Mueller, Goetz C</creator><creator>Stierle, Lydia</creator><creator>Stark, Veronika</creator><creator>Steiner, Kristoffer</creator><creator>von Kodolitsch, Yskert</creator><creator>Weil, Jochen</creator><creator>Mir, Thomas S</creator><general>BMJ Publishing Group LTD</general><scope>3V.</scope><scope>7X7</scope><scope>7XB</scope><scope>88E</scope><scope>88I</scope><scope>8AF</scope><scope>8FI</scope><scope>8FJ</scope><scope>8FK</scope><scope>ABUWG</scope><scope>AFKRA</scope><scope>AZQEC</scope><scope>BENPR</scope><scope>BTHHO</scope><scope>CCPQU</scope><scope>DWQXO</scope><scope>FYUFA</scope><scope>GHDGH</scope><scope>GNUQQ</scope><scope>HCIFZ</scope><scope>K9.</scope><scope>M0S</scope><scope>M1P</scope><scope>M2P</scope><scope>PQEST</scope><scope>PQQKQ</scope><scope>PQUKI</scope><scope>Q9U</scope></search><sort><creationdate>20140201</creationdate><title>Retrospective analysis of the effect of angiotensin II receptor blocker versus [beta]-blocker on aortic root growth in paediatric patients with Marfan syndrome</title><author>Mueller, Goetz C ; 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Less is known about the effectiveness of angiotensin II receptor blocker (ARB) on aortic dilation in paediatric patients with MFS. Methods 215 patients with MFS (9.01±5.7 years) were subject to a standardised diagnostic programme. Aortic root dilation was evaluated and followed up by echocardiography. In 48 cases, BB and ARB effects on aortic root dilation were evaluated. Effect of treatment was measured by comparison of z scores of SV before and after treatment initiation. Results Treatment by ARB and BB leads to significant reduction of SV dilation (p&lt;0.05). The deviation of SV enlargement from normal as expressed by the rate of change in z scores was significantly reduced by a mean difference of -0.56±0.71 z scores (p&lt;0.001) under ARB therapy and by a mean difference of -0.35±0.68 z scores (p&lt;0.05) under BB therapy. The prophylactic effect of ARB and BB on aortic root dilation is similar in both groups (p&gt;0.05). Conclusions Both concepts lead to a significant reduction of SV dilation. The effect of ARB and BB is similar. This is the first study concerning the comparison of ARB and BB in previously untreated paediatric patients with MFS. The results of the study show that both treatment strategies are beneficial in paediatric and adolescent patients.</abstract><cop>London</cop><pub>BMJ Publishing Group LTD</pub><doi>10.1136/heartjnl-2013-304946</doi></addata></record>
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subjects Child development
Dissection
Marfan syndrome
Mortality
Pathogenesis
Surgery
Teenagers
title Retrospective analysis of the effect of angiotensin II receptor blocker versus [beta]-blocker on aortic root growth in paediatric patients with Marfan syndrome
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