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Published in: Heart and Vessels 5/2015

01-09-2015 | Case Report

A high-risk patient with long-QT syndrome with no response to cardioselective beta-blockers

Authors: Naoki Toyota, Aya Miyazaki, Heima Sakaguchi, Wataru Shimizu, Hideo Ohuchi

Published in: Heart and Vessels | Issue 5/2015

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Abstract

We present a case of a high-risk 19-year-old female with long-QT syndrome (LQTS) with compound mutations. She had a history of aborted cardiac arrest and syncope and had received treatment with propranolol for 15 years. However, because she developed adult-onset asthma we tried to switch propranolol, a nonselective beta-blocker, to beta-1-cardioselective agents, bisoprolol and metoprolol. These resulted in both a markedly prolonged corrected QT interval and the development of LQTS-associated arrhythmias. Eventually, propranolol was reinitiated at a higher dose with the addition of verapamil, and she has had no further cardiac or asthmatic events for 5 years.
Literature
1.
go back to reference Moss AJ, Zareba W, Hall WJ, Schwartz PJ, Crampton RS, Benhorin J, Vincent GM, Locati EH, Priori SG, Napolitano C, Medina A, Zhang L, Robinson JL, Timothy K, Towbin JA, Andrews ML (2000) Effectiveness and limitations of beta-blocker therapy in congenital long-QT syndrome. Circulation 101:616–623CrossRefPubMed Moss AJ, Zareba W, Hall WJ, Schwartz PJ, Crampton RS, Benhorin J, Vincent GM, Locati EH, Priori SG, Napolitano C, Medina A, Zhang L, Robinson JL, Timothy K, Towbin JA, Andrews ML (2000) Effectiveness and limitations of beta-blocker therapy in congenital long-QT syndrome. Circulation 101:616–623CrossRefPubMed
2.
go back to reference Goldenberg I, Bradley J, Moss A, Mcnitt S, Polonsky S, Robinson JL, Andrewa M, Zareba W (2010) Beta-blocker efficacy in high-risk patients with the congenital long-QT syndrome types 1 and 2: implications for patient management. J Cardiovasc Electrophysiol 21:893–901PubMedCentralPubMed Goldenberg I, Bradley J, Moss A, Mcnitt S, Polonsky S, Robinson JL, Andrewa M, Zareba W (2010) Beta-blocker efficacy in high-risk patients with the congenital long-QT syndrome types 1 and 2: implications for patient management. J Cardiovasc Electrophysiol 21:893–901PubMedCentralPubMed
3.
go back to reference Goldenberg I, Moss AJ, Peterson DR, McNitt S, Zareba W, Andrews ML, Robinson JL, Locati EH, Ackerman MJ, Benhorin J, Kaufman ES, Napolitano C, Priori SG, Qi M, Schwartz PJ, Towbin JA, Vincent GM, Zhang L (2008) Risk factors for aborted cardiac arrest and sudden cardiac death in children with the congenital long-QT syndrome. Circulation 117:2184–2191CrossRefPubMedCentralPubMed Goldenberg I, Moss AJ, Peterson DR, McNitt S, Zareba W, Andrews ML, Robinson JL, Locati EH, Ackerman MJ, Benhorin J, Kaufman ES, Napolitano C, Priori SG, Qi M, Schwartz PJ, Towbin JA, Vincent GM, Zhang L (2008) Risk factors for aborted cardiac arrest and sudden cardiac death in children with the congenital long-QT syndrome. Circulation 117:2184–2191CrossRefPubMedCentralPubMed
4.
go back to reference Chockalingam P, Crotti L, Girardengo G, Johnson JN, Harris KM, Heijden JF, Hauer RN, Beckmann BM, Spazzolini C, Rordorf R, Rydberg A, Clur SA, Fischer M, Heuvel F, Kaab S, Blom NA, Ackerman MJ, Schwartz PJ, Wilde AA (2012) Not all beta-blockers are equal in the management of long QT syndrome types 1 and 2. J Am Coll Cardiol 60:2092–2099CrossRefPubMedCentralPubMed Chockalingam P, Crotti L, Girardengo G, Johnson JN, Harris KM, Heijden JF, Hauer RN, Beckmann BM, Spazzolini C, Rordorf R, Rydberg A, Clur SA, Fischer M, Heuvel F, Kaab S, Blom NA, Ackerman MJ, Schwartz PJ, Wilde AA (2012) Not all beta-blockers are equal in the management of long QT syndrome types 1 and 2. J Am Coll Cardiol 60:2092–2099CrossRefPubMedCentralPubMed
5.
go back to reference Chatrath R, Bell CM, Ackerman MJ (2004) Beta-blocker therapy failures in symptomatic probands with genotyped long-QT syndrome. Pediatr Cardiol 25:459–465CrossRefPubMed Chatrath R, Bell CM, Ackerman MJ (2004) Beta-blocker therapy failures in symptomatic probands with genotyped long-QT syndrome. Pediatr Cardiol 25:459–465CrossRefPubMed
6.
go back to reference Nakashima K, Kusakawa I, Yamamoto T, Hirabayashi S, Hosoya R, Shimizu W, Sumitomo N (2013) A left ventricular noncompaction in a patient with long QT syndrome caused by a KCNQ1 mutation: a case report. Heart Vessels 28:126–129CrossRefPubMed Nakashima K, Kusakawa I, Yamamoto T, Hirabayashi S, Hosoya R, Shimizu W, Sumitomo N (2013) A left ventricular noncompaction in a patient with long QT syndrome caused by a KCNQ1 mutation: a case report. Heart Vessels 28:126–129CrossRefPubMed
8.
go back to reference Schwartz PJ, Priori SG, Spazzolini C, Moss AJ, Vincent GM, Napolitano C, Denjoy I, Guicheney P, Breithardt G, Keating MT, Towbin JA, Beggs AH, Brink P, Wilde AA, Toivonen L, Zareba W, Robinson JL, Timothy KW, Corfield V, Wattanasirichaigoon D, Corbett C, Haverkamp W, Schulze-Bahr E, Lehmann MH, Schwartz K, Coumel P, Bloise R (2001) Genotype–phenotype correlation in the long-QT syndrome: gene-specific triggers for life-threatening arrhythmias. Circulation 103:89–95CrossRefPubMed Schwartz PJ, Priori SG, Spazzolini C, Moss AJ, Vincent GM, Napolitano C, Denjoy I, Guicheney P, Breithardt G, Keating MT, Towbin JA, Beggs AH, Brink P, Wilde AA, Toivonen L, Zareba W, Robinson JL, Timothy KW, Corfield V, Wattanasirichaigoon D, Corbett C, Haverkamp W, Schulze-Bahr E, Lehmann MH, Schwartz K, Coumel P, Bloise R (2001) Genotype–phenotype correlation in the long-QT syndrome: gene-specific triggers for life-threatening arrhythmias. Circulation 103:89–95CrossRefPubMed
9.
go back to reference Itoh H, Shimizu W, Hayashi K, Yamagata K, Sakaguchi T, Ohno S, Makiyama T, Akao M, Ai T, Noda T, Miyazaki A, Miyamoto Y, Yamagishi M, Kamakura S, Horie M (2010) Long QT syndrome with compound mutations is associated with a more severe phenotype: a Japanese multicenter study. Heart Rhythm 7:1411–1418CrossRefPubMed Itoh H, Shimizu W, Hayashi K, Yamagata K, Sakaguchi T, Ohno S, Makiyama T, Akao M, Ai T, Noda T, Miyazaki A, Miyamoto Y, Yamagishi M, Kamakura S, Horie M (2010) Long QT syndrome with compound mutations is associated with a more severe phenotype: a Japanese multicenter study. Heart Rhythm 7:1411–1418CrossRefPubMed
10.
go back to reference Bando S, Soeki T, Matsuura T, Niki T, Ise T, Yamaguchi K, Taketani Y, Iwase T, Yamada H, Wakatsuki T, Akaike M, Aiba T, Shimizu W, Sata M (2013) Congenital long QT syndrome with compound mutations in the KCNH2 gene. Heart Vessels. doi: 10.1007/s00380-013-0406-2 Bando S, Soeki T, Matsuura T, Niki T, Ise T, Yamaguchi K, Taketani Y, Iwase T, Yamada H, Wakatsuki T, Akaike M, Aiba T, Shimizu W, Sata M (2013) Congenital long QT syndrome with compound mutations in the KCNH2 gene. Heart Vessels. doi: 10.​1007/​s00380-013-0406-2
11.
go back to reference Villain E, Denjoy I, Lupoglazoff JM, Guicheney P, Hainque B, Lucet V, Bonnet D (2004) Low incidence of cardiac events with beta-blocking therapy in children with long QT syndrome. Eur Heart J 25:1405–1411CrossRefPubMed Villain E, Denjoy I, Lupoglazoff JM, Guicheney P, Hainque B, Lucet V, Bonnet D (2004) Low incidence of cardiac events with beta-blocking therapy in children with long QT syndrome. Eur Heart J 25:1405–1411CrossRefPubMed
12.
go back to reference Vincent GM, Schwartz PJ, Denjoy I, Swan H, Bithell C, Spazzolini C, Crotti L, Piippo K, Lupoglazoff J, Villain E, Priori SG, Napolitano C, Zhang L (2009) High efficacy of beta-blockers in long-QT syndrome type 1: contribution of noncompliance and QT-prolonging drugs to the occurrence of beta-blocker treatment “failures”. Circulation 119:215–221CrossRefPubMed Vincent GM, Schwartz PJ, Denjoy I, Swan H, Bithell C, Spazzolini C, Crotti L, Piippo K, Lupoglazoff J, Villain E, Priori SG, Napolitano C, Zhang L (2009) High efficacy of beta-blockers in long-QT syndrome type 1: contribution of noncompliance and QT-prolonging drugs to the occurrence of beta-blocker treatment “failures”. Circulation 119:215–221CrossRefPubMed
13.
go back to reference Bankston JR, Kass RS (2010) Molecular determinants of local anesthetic action of beta-blocking drugs: implication for therapeutic management of long QT syndrome variant 3. J Mol Cell Cardiol 48:246–253CrossRefPubMedCentralPubMed Bankston JR, Kass RS (2010) Molecular determinants of local anesthetic action of beta-blocking drugs: implication for therapeutic management of long QT syndrome variant 3. J Mol Cell Cardiol 48:246–253CrossRefPubMedCentralPubMed
14.
go back to reference Duff HJ, Mitchell LB, Wyse DG (1986) Antiarrhythmic efficacy of propranolol: comparison of low and high serum concentrations. J Am Coll Cardiol 8:959–965CrossRefPubMed Duff HJ, Mitchell LB, Wyse DG (1986) Antiarrhythmic efficacy of propranolol: comparison of low and high serum concentrations. J Am Coll Cardiol 8:959–965CrossRefPubMed
15.
go back to reference Aygul N, Ozdemir K, Duzenli MA, Aygul MU (2009) The comparative effects of long-term carvedilol versus bisoprolol therapy on QT dispersion in patients with chronic heart failure. Cardiology 112:168–173CrossRefPubMed Aygul N, Ozdemir K, Duzenli MA, Aygul MU (2009) The comparative effects of long-term carvedilol versus bisoprolol therapy on QT dispersion in patients with chronic heart failure. Cardiology 112:168–173CrossRefPubMed
16.
go back to reference Viitasalo M, Oikarinen L, Swan H, Vaananen H, Jarvenpaa J, Hietanen H, Karjalainen J, Toivonen L (2006) Effects of beta-blocker therapy on ventricular repolarization documented by 24-h electrocardiography in patients with type 1 long-QT syndrome. J Am Coll Cardiol 48:747–753CrossRefPubMed Viitasalo M, Oikarinen L, Swan H, Vaananen H, Jarvenpaa J, Hietanen H, Karjalainen J, Toivonen L (2006) Effects of beta-blocker therapy on ventricular repolarization documented by 24-h electrocardiography in patients with type 1 long-QT syndrome. J Am Coll Cardiol 48:747–753CrossRefPubMed
17.
go back to reference Extramiana F, Maison-Blanche P, Denjoy I, Jode PD, Messali A, Labbe JP, Leenhardt A (2013) Gene-specific effect of beta-adrenergic blockade on corrected QT interval in the long QT syndrome. Ann Noninvasive Electrocardiol 18:399–408CrossRefPubMed Extramiana F, Maison-Blanche P, Denjoy I, Jode PD, Messali A, Labbe JP, Leenhardt A (2013) Gene-specific effect of beta-adrenergic blockade on corrected QT interval in the long QT syndrome. Ann Noninvasive Electrocardiol 18:399–408CrossRefPubMed
18.
go back to reference Jons C, Moss AJ, Goldenberg I, Liu J, McNitt S, Zareba W, Qi M, Robinson JL (2010) Risk of fatal arrhythmic events in long QT syndrome patients after syncope. J Am Coll Cardiol 55:783–788CrossRefPubMed Jons C, Moss AJ, Goldenberg I, Liu J, McNitt S, Zareba W, Qi M, Robinson JL (2010) Risk of fatal arrhythmic events in long QT syndrome patients after syncope. J Am Coll Cardiol 55:783–788CrossRefPubMed
Metadata
Title
A high-risk patient with long-QT syndrome with no response to cardioselective beta-blockers
Authors
Naoki Toyota
Aya Miyazaki
Heima Sakaguchi
Wataru Shimizu
Hideo Ohuchi
Publication date
01-09-2015
Publisher
Springer Japan
Published in
Heart and Vessels / Issue 5/2015
Print ISSN: 0910-8327
Electronic ISSN: 1615-2573
DOI
https://doi.org/10.1007/s00380-014-0530-7

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