テストステロン減少が病態の顕性化に寄与したと考えられた高齢発症のQT延長症候群の1例

27歳時に片側睾丸摘出の既往がある61歳男性.母親とその姉妹2人が20歳頃に突然死している.失神や多形性心室頻拍(TdP)の既往はなかったが,50歳頃より検診でQT延長を指摘されていた.今回,運転中に初めて失神し搬送された.この際,著明なQT延長(QTc=666ms)とTdP頻発を認めた.器質的心疾患は認めず,家族歴から先天性QT延長症候群と考えられたが,本例では60歳代での心原性失神の初発であった.検診心電図では,QT延長は段階的に顕性化していた.本例では数日前からエフェドリン含む感冒薬の内服歴がありTdP発症の要因となったと考えられた.さらに本例では明らかなテストステロン低値を認めており,...

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Published inShinzo Vol. 49; no. SUPPL.1; pp. S1_43 - S1_49
Main Authors 飯嶋, 賢一, 八木原, 伸江, 佐藤, 光希, 和泉, 大輔, 大槻, 総, 南野, 徹, 長谷川, 祐紀, 池主, 雅臣, 坂口, 裕太
Format Journal Article
LanguageJapanese
Published 公益財団法人 日本心臓財団 28.08.2017
Japan Heart Foundation
Subjects
Online AccessGet full text
ISSN0586-4488
2186-3016
DOI10.11281/shinzo.49.S1_43

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Abstract 27歳時に片側睾丸摘出の既往がある61歳男性.母親とその姉妹2人が20歳頃に突然死している.失神や多形性心室頻拍(TdP)の既往はなかったが,50歳頃より検診でQT延長を指摘されていた.今回,運転中に初めて失神し搬送された.この際,著明なQT延長(QTc=666ms)とTdP頻発を認めた.器質的心疾患は認めず,家族歴から先天性QT延長症候群と考えられたが,本例では60歳代での心原性失神の初発であった.検診心電図では,QT延長は段階的に顕性化していた.本例では数日前からエフェドリン含む感冒薬の内服歴がありTdP発症の要因となったと考えられた.さらに本例では明らかなテストステロン低値を認めており,QT延長症候群の高齢での顕性化に寄与したと考えられた.QT延長例や潜在的素因を持つ例に内分泌的異常や薬剤など幅広い修飾因子が加わることで,高齢で初回発作をきたす例があり注意を要する.
AbstractList 27歳時に片側睾丸摘出の既往がある61歳男性.母親とその姉妹2人が20歳頃に突然死している.失神や多形性心室頻拍(TdP)の既往はなかったが,50歳頃より検診でQT延長を指摘されていた.今回,運転中に初めて失神し搬送された.この際,著明なQT延長(QTc=666ms)とTdP頻発を認めた.器質的心疾患は認めず,家族歴から先天性QT延長症候群と考えられたが,本例では60歳代での心原性失神の初発であった.検診心電図では,QT延長は段階的に顕性化していた.本例では数日前からエフェドリン含む感冒薬の内服歴がありTdP発症の要因となったと考えられた.さらに本例では明らかなテストステロン低値を認めており,QT延長症候群の高齢での顕性化に寄与したと考えられた.QT延長例や潜在的素因を持つ例に内分泌的異常や薬剤など幅広い修飾因子が加わることで,高齢で初回発作をきたす例があり注意を要する.
Author 飯嶋, 賢一
佐藤, 光希
大槻, 総
池主, 雅臣
長谷川, 祐紀
坂口, 裕太
和泉, 大輔
八木原, 伸江
南野, 徹
Author_FL Sato Akinori
Otsuki Sou
Chinushi Masaomi
Minamino Tohru
Sakaguchi Yuta
Hasegawa Yuki
Yagihara Nobue
Izumi Daisuke
Iijima Kenichi
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DocumentTitleAlternate Low levels of testosterone contributed to the first development of cardiac event in an elderly patient with congenital long QT syndrome: a case report
DocumentTitle_FL Low levels of testosterone contributed to the first development of cardiac event in an elderly patient with congenital long QT syndrome: a case report
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References 4 Silvia G.Priori, Peter J.Schwartz, Carlo Napolitano, et al: Risk Stratification in the Long-QT Syndrome. N Engl J Med 2003;348:1866-1874
12 van Noord C, Dörr M, Sturkenboom MC, et al: The association of serum testosterone levels and ventricular repolarization. Eur J Epidemiol 2010;25:21-28
3 Hashiba K: Hereditary QT prolongation syndrome in Japan: genetic analysis and pathological findings of the conducting system. Jap Curc J 1978;42:133-150
16 Vicente J, Johannesen L, Galeotti L, et al: Mechanisms of sex and age differences in ventricular repolarization in humans. Am Heart J. 2014;168(5):749-756
13 Pecori Giraldi F, Toja PM, Filippini B, et al: Increased prevalence of prolonged QT interval in males with primary or secondary hypogonadism: a pilot study. Int J Androl 33(2010), e132-138
7 Locati EH, Zareba W, Moss AJ, et al: Age- and sex-related differences in clinical manifestations in patients with congenital long-QT syndrome: findings from the International LQTS Registry. Circulation 1998;97:2237-2244
11 Jie Zhao, Chaoqiang Jiang, Tai Hing Lam, et al: Genetically predicted testosterone and electrocardiographic QT interval duration in Chinese: a Mendelian randomization analysis in the Guangzhou Biobank Cohort Study. International Journal of Epidemiology 2015, 613-620
17 Thai V. Pham, Eugene A. Sosunov, Ravil Z. Gainullin, et al: Impact of Sex and Gonadal Steroids on Prolongation of Ventricular Repolarization and Arrhythmias Induced by IK-Blocking Drugs. Circulation. 2001;103:2207-2212
1 Morita H, Wu J, Zipes DP: The QT syndromes: long and short. Lancet. 2008;372(9640):750-763
15 Bai CX, Kurokawa J, Tamagawa M, et al: Nontranscriptional regulation of cardiac repolarization currents by testosterone. Circulation. 2005;112(12):1701-1710
5 J Sauer AV, Moss AJ, McNitt S, et al: Long QT syndrome in adults. Am Coll Cardiol. 2007;49(3):329-337
10 Iwamoto T, Yanase T, Koh E, et al: Reference ranges of total serum and free testosterone in Japanese male adults. Nihon Hinyokika Gakkai Zasshi. 2004;95(6):751-760
18 Yang Lin , Yicheng Fu: Repolarization reserve, arrhythmia and new drug development. Acta Pharmaceutica Sinica B 2013;3(3):150–153
14 Bidoggia H, Maciel JP, Capalozza N, et al: Sex differences on the electrocardiographic pattern of cardiac repolarization: possible role of testosterone. Am Heart J. 2000;140(4):678-683
8 Imboden M, Swan H, Denjoy I, et al: Female predominance and transmission distortion in the long-QT syndrome. N Engl J Med. 2006;355(26):2744-2751
9 William J. Bremner, Michael V. Vitiello, Patricia N. Prinz: Loss of Circadian Rhythmicity in Blood Testosterone Levels with Aging in Normal Men. J Clin Endocrinol Metab 56:1278-1983
2 Schouten EG1, Dekker JM, Meppelink P, et al: QT interval prolongation predicts cardiovascular mortality in an apparently healthy population. Circulation. 1991;84(4):1516-1523
6 Kurokawa J, Kodama M, Clancy CE, et al: Sex hormonal regulation of cardiac ion channels in drug-induced QT syndromes. Pharmacology & Therapeutics 168(2016) 23-28
References_xml – reference: 15 Bai CX, Kurokawa J, Tamagawa M, et al: Nontranscriptional regulation of cardiac repolarization currents by testosterone. Circulation. 2005;112(12):1701-1710
– reference: 2 Schouten EG1, Dekker JM, Meppelink P, et al: QT interval prolongation predicts cardiovascular mortality in an apparently healthy population. Circulation. 1991;84(4):1516-1523
– reference: 9 William J. Bremner, Michael V. Vitiello, Patricia N. Prinz: Loss of Circadian Rhythmicity in Blood Testosterone Levels with Aging in Normal Men. J Clin Endocrinol Metab 56:1278-1983
– reference: 3 Hashiba K: Hereditary QT prolongation syndrome in Japan: genetic analysis and pathological findings of the conducting system. Jap Curc J 1978;42:133-150
– reference: 8 Imboden M, Swan H, Denjoy I, et al: Female predominance and transmission distortion in the long-QT syndrome. N Engl J Med. 2006;355(26):2744-2751
– reference: 5 J Sauer AV, Moss AJ, McNitt S, et al: Long QT syndrome in adults. Am Coll Cardiol. 2007;49(3):329-337
– reference: 6 Kurokawa J, Kodama M, Clancy CE, et al: Sex hormonal regulation of cardiac ion channels in drug-induced QT syndromes. Pharmacology & Therapeutics 168(2016) 23-28
– reference: 18 Yang Lin , Yicheng Fu: Repolarization reserve, arrhythmia and new drug development. Acta Pharmaceutica Sinica B 2013;3(3):150–153
– reference: 7 Locati EH, Zareba W, Moss AJ, et al: Age- and sex-related differences in clinical manifestations in patients with congenital long-QT syndrome: findings from the International LQTS Registry. Circulation 1998;97:2237-2244
– reference: 11 Jie Zhao, Chaoqiang Jiang, Tai Hing Lam, et al: Genetically predicted testosterone and electrocardiographic QT interval duration in Chinese: a Mendelian randomization analysis in the Guangzhou Biobank Cohort Study. International Journal of Epidemiology 2015, 613-620
– reference: 1 Morita H, Wu J, Zipes DP: The QT syndromes: long and short. Lancet. 2008;372(9640):750-763
– reference: 17 Thai V. Pham, Eugene A. Sosunov, Ravil Z. Gainullin, et al: Impact of Sex and Gonadal Steroids on Prolongation of Ventricular Repolarization and Arrhythmias Induced by IK-Blocking Drugs. Circulation. 2001;103:2207-2212
– reference: 13 Pecori Giraldi F, Toja PM, Filippini B, et al: Increased prevalence of prolonged QT interval in males with primary or secondary hypogonadism: a pilot study. Int J Androl 33(2010), e132-138
– reference: 16 Vicente J, Johannesen L, Galeotti L, et al: Mechanisms of sex and age differences in ventricular repolarization in humans. Am Heart J. 2014;168(5):749-756
– reference: 10 Iwamoto T, Yanase T, Koh E, et al: Reference ranges of total serum and free testosterone in Japanese male adults. Nihon Hinyokika Gakkai Zasshi. 2004;95(6):751-760
– reference: 12 van Noord C, Dörr M, Sturkenboom MC, et al: The association of serum testosterone levels and ventricular repolarization. Eur J Epidemiol 2010;25:21-28
– reference: 14 Bidoggia H, Maciel JP, Capalozza N, et al: Sex differences on the electrocardiographic pattern of cardiac repolarization: possible role of testosterone. Am Heart J. 2000;140(4):678-683
– reference: 4 Silvia G.Priori, Peter J.Schwartz, Carlo Napolitano, et al: Risk Stratification in the Long-QT Syndrome. N Engl J Med 2003;348:1866-1874
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SubjectTerms HERG
QT延長
テストステロン
多形性心室頻拍
Title テストステロン減少が病態の顕性化に寄与したと考えられた高齢発症のQT延長症候群の1例
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