先天性QT延長症候群におけるelectrical stormに対するCa拮抗薬の使い方
先天性QT延長症候群（LQTS）の薬物治療としては，β遮断薬のほかに，内向き電流を減少させるIb群Na＋チャネル遮断薬やCa拮抗薬，外向き電流を増加させるK＋チャネル開口薬などが考えられる．Ca拮抗薬のベラパミルは，単相性活動電位（monophasic action potential ; MAP）記録を用いた臨床研究や，動脈灌流心室筋切片標本を用いた実験的LQTSモデルによる検討からその有効性が報告されている．当院では主にβ遮断薬治療による外来経過観察中に遺伝子型が同定された先天性LQTS患者193例中11例（5.7%）で，torsade de pointes（TdP）の反復によるelect...
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| Published in | 心電図 Vol. 30; no. 1; pp. 55 - 62 |
|---|---|
| Main Authors | , , , , , , , , , |
| Format | Journal Article |
| Language | Japanese |
| Published |
一般社団法人 日本不整脈心電学会
2010
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| Subjects | |
| Online Access | Get full text |
| ISSN | 0285-1660 1884-2437 |
| DOI | 10.5105/jse.30.55 |
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| Abstract | 先天性QT延長症候群（LQTS）の薬物治療としては，β遮断薬のほかに，内向き電流を減少させるIb群Na＋チャネル遮断薬やCa拮抗薬，外向き電流を増加させるK＋チャネル開口薬などが考えられる．Ca拮抗薬のベラパミルは，単相性活動電位（monophasic action potential ; MAP）記録を用いた臨床研究や，動脈灌流心室筋切片標本を用いた実験的LQTSモデルによる検討からその有効性が報告されている．当院では主にβ遮断薬治療による外来経過観察中に遺伝子型が同定された先天性LQTS患者193例中11例（5.7%）で，torsade de pointes（TdP）の反復によるelectrical storm（ES）を認めた．ESのリスク因子としては，女性，発端者，LQT2型，失神・心停止・TdPの既往，Schwartzスコアー≧6，安静時QT時間≧ 500msecが，直接的な誘因としては，低K血症や低Mg血症があげられた．ES急性期の治療として，Ca拮抗薬のベラパミルの静注と持続点滴が有効であった． |
|---|---|
| AbstractList | 先天性QT延長症候群（LQTS）の薬物治療としては，β遮断薬のほかに，内向き電流を減少させるIb群Na＋チャネル遮断薬やCa拮抗薬，外向き電流を増加させるK＋チャネル開口薬などが考えられる．Ca拮抗薬のベラパミルは，単相性活動電位（monophasic action potential ; MAP）記録を用いた臨床研究や，動脈灌流心室筋切片標本を用いた実験的LQTSモデルによる検討からその有効性が報告されている．当院では主にβ遮断薬治療による外来経過観察中に遺伝子型が同定された先天性LQTS患者193例中11例（5.7%）で，torsade de pointes（TdP）の反復によるelectrical storm（ES）を認めた．ESのリスク因子としては，女性，発端者，LQT2型，失神・心停止・TdPの既往，Schwartzスコアー≧6，安静時QT時間≧ 500msecが，直接的な誘因としては，低K血症や低Mg血症があげられた．ES急性期の治療として，Ca拮抗薬のベラパミルの静注と持続点滴が有効であった． |
| Author | 相原, 直彦 山田, 優子 清水, 渉 須山, 和弘 栗田, 隆志 里見, 和浩 岡村, 英夫 野田, 崇 鎌倉, 史郎 相庭, 武司 |
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| References | 7) Shimizu W, Kurita T, Matsuo K, Suyama K, Aihara N, Kamakura S, Towbin JA, Shimomura K : Improvement of repolarization abnormalities by a K+channel opener in the LQT1 form of con-genital long QT syndrome. Circulation, 1998 ; 97 : 1581-1588 12) Moss AJ, Shimizu W, Wilde AA, Towbin JA, Zareba W, Robinson JL, Qi M, Vincent GM, Ackerman MJ, Kaufman ES, Hofman N, Seth R, Kamakura S, Miyamato Y, Goldenberg I, Andrews ML, McNitt S : Clinical aspects of type-1 long-QT syndrome by location, coding type, and biophysical function of mutations involving the KCNQ1 gene. Circulation, 2007 ; 115 : 2481-2489 8) Kurita T, Ohe T, Shimizu W, Suyama K, Aihara N, Takaki H, Kamakura S, Shimomura K : Early afterdepolarization like activity in patients with class Ia-induced long QT syndrome and torsade de pointes. PACE, 1997 ; 20 : 695-705 13) Vincent GM, Schwartz PJ, Denjoy I, Swan H, Bithell C, Spazzolini C, Crotti L, Piippo K, Lupoglazoff JM, Villain E, Priori SG, Napolitano C, Zhang L : 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, 2009 ; 119 : 215-221 6) Shimizu W, Ohe T, Kurita T, Kawade M, Arakaki Y, Aihara N, Kamakura S, Kamiya T, Shi-momura K : Effects of verapamil and propranolol on early afterdepolarizations and ventricular arrhythmias induced by epinephrine in congenital long QT syndrome. J Am Coll Cardiol, 1995 ; 26 : 1299-1309 9) Shimizu W, Antzelevitch C : Sodium channel block with mexiletine is effective in reducing dis-persion of repolarization and preventing torsade de pointes in LQT2 and LQT3 models of the long QT syndrome. Circulation, 1997 ; 96 : 2038-2047 4) Ohe T, Kurita T, Aihara N, Kamakura S, Matsuhisa M, Shimomura K : Electrocardiographic and electrophysiologic studies in patients with torsades de pointes : Role of monophasic action potentials. Jpn Circ J, 1990 ; 54 : 1323-1330 14) Aiba T, Shimizu W, Inagaki M, Noda T, Miyoshi S, Ding WG, Zankov DP, Toyoda F, Matsuura H, Horie M, Sunagawa K : Cellular and ionic mechanism for drug-induced long-QT syndrome and effectiveness of verapamil. J Am Coll Cardiol, 2005 ; 45 : 300-307 2) Schwartz PJ, Moss AJ, Vincent GM, Crampton RS : Diagnostic criteria for the long QT syn-drome : An update. Circulation, 1993 ; 88 : 782-784 5) Shimizu W, Ohe T, Kurita T, Takaki H, Aihara N, Kamakura S, Matsuhisa M, Shimomura K : Early afterdepolarizations induced by isoproterenol in patients with congenital long QT syndrome. Circulation, 1991 ; 84 : 1915-1923 3) Shimizu W : Clinical impact of genetic studies in lethal inherited cardiac arrhythmias. Circ J, 2008 ; 72 : 1926-1936 1) Shimizu W : The long QT syndrome : Therapeutic implications of a genetic diagnosis. Cardiovasc Res, 2005 ; 67 : 347-356 16) Shimizu W, Ohe T, Kurita T, Tokuda T, Shimomura K : Epinephrine-induced ventricular premature complexes due to early afterdepolarizations and effects of verapamil and propranolol in a patient with congenital long QT syndrome. J Cardiovasc Electrophysiol, 1994 ; 5 : 438-444 10) Shimizu W, Antzelevitch C : Cellular basis for the electrocardiographic features of the LQT1 form of the long QT syndrome : Effects of &beta adrenergic agonists, antagonists and sodium channel blockers on transmural dispersion of repolarization and torsade de pointes. Circulation, 1998 ; 98 : 2314-2322 11) Shimizu W, Antzelevitch C : Effects of a K+channel opener to reduce transmural disper-sion of repolarization and prevent torsade de pointes in LQT1, LQT2 and LQT3 models of the long-QT syndrome. Circulation, 2000 ; 102 : 706-712 15) Franz MR : Long-term recording of monophasic action potentials from human endocardium. Am J Cardiol, 1983 ; 51 : 1629-1634 17) Shimizu W, Noda T, Satomi K, Suyama K, Kurita T, Aihara N, Horie M, Priori SG, Kamakura S : Clinical characteristics and acute therapy for electrical storm of torsade de pointes in genotyped patients with congenital long QT syndrome. Circulation, 2005 ; 112 : II 493 |
| References_xml | – reference: 15) Franz MR : Long-term recording of monophasic action potentials from human endocardium. Am J Cardiol, 1983 ; 51 : 1629-1634 – reference: 10) Shimizu W, Antzelevitch C : Cellular basis for the electrocardiographic features of the LQT1 form of the long QT syndrome : Effects of &beta adrenergic agonists, antagonists and sodium channel blockers on transmural dispersion of repolarization and torsade de pointes. Circulation, 1998 ; 98 : 2314-2322 – reference: 9) Shimizu W, Antzelevitch C : Sodium channel block with mexiletine is effective in reducing dis-persion of repolarization and preventing torsade de pointes in LQT2 and LQT3 models of the long QT syndrome. Circulation, 1997 ; 96 : 2038-2047 – reference: 14) Aiba T, Shimizu W, Inagaki M, Noda T, Miyoshi S, Ding WG, Zankov DP, Toyoda F, Matsuura H, Horie M, Sunagawa K : Cellular and ionic mechanism for drug-induced long-QT syndrome and effectiveness of verapamil. J Am Coll Cardiol, 2005 ; 45 : 300-307 – reference: 3) Shimizu W : Clinical impact of genetic studies in lethal inherited cardiac arrhythmias. Circ J, 2008 ; 72 : 1926-1936 – reference: 5) Shimizu W, Ohe T, Kurita T, Takaki H, Aihara N, Kamakura S, Matsuhisa M, Shimomura K : Early afterdepolarizations induced by isoproterenol in patients with congenital long QT syndrome. Circulation, 1991 ; 84 : 1915-1923 – reference: 4) Ohe T, Kurita T, Aihara N, Kamakura S, Matsuhisa M, Shimomura K : Electrocardiographic and electrophysiologic studies in patients with torsades de pointes : Role of monophasic action potentials. Jpn Circ J, 1990 ; 54 : 1323-1330 – reference: 13) Vincent GM, Schwartz PJ, Denjoy I, Swan H, Bithell C, Spazzolini C, Crotti L, Piippo K, Lupoglazoff JM, Villain E, Priori SG, Napolitano C, Zhang L : 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, 2009 ; 119 : 215-221 – reference: 6) Shimizu W, Ohe T, Kurita T, Kawade M, Arakaki Y, Aihara N, Kamakura S, Kamiya T, Shi-momura K : Effects of verapamil and propranolol on early afterdepolarizations and ventricular arrhythmias induced by epinephrine in congenital long QT syndrome. J Am Coll Cardiol, 1995 ; 26 : 1299-1309 – reference: 7) Shimizu W, Kurita T, Matsuo K, Suyama K, Aihara N, Kamakura S, Towbin JA, Shimomura K : Improvement of repolarization abnormalities by a K+channel opener in the LQT1 form of con-genital long QT syndrome. Circulation, 1998 ; 97 : 1581-1588 – reference: 12) Moss AJ, Shimizu W, Wilde AA, Towbin JA, Zareba W, Robinson JL, Qi M, Vincent GM, Ackerman MJ, Kaufman ES, Hofman N, Seth R, Kamakura S, Miyamato Y, Goldenberg I, Andrews ML, McNitt S : Clinical aspects of type-1 long-QT syndrome by location, coding type, and biophysical function of mutations involving the KCNQ1 gene. Circulation, 2007 ; 115 : 2481-2489 – reference: 16) Shimizu W, Ohe T, Kurita T, Tokuda T, Shimomura K : Epinephrine-induced ventricular premature complexes due to early afterdepolarizations and effects of verapamil and propranolol in a patient with congenital long QT syndrome. J Cardiovasc Electrophysiol, 1994 ; 5 : 438-444 – reference: 2) Schwartz PJ, Moss AJ, Vincent GM, Crampton RS : Diagnostic criteria for the long QT syn-drome : An update. Circulation, 1993 ; 88 : 782-784 – reference: 17) Shimizu W, Noda T, Satomi K, Suyama K, Kurita T, Aihara N, Horie M, Priori SG, Kamakura S : Clinical characteristics and acute therapy for electrical storm of torsade de pointes in genotyped patients with congenital long QT syndrome. Circulation, 2005 ; 112 : II 493 – reference: 1) Shimizu W : The long QT syndrome : Therapeutic implications of a genetic diagnosis. Cardiovasc Res, 2005 ; 67 : 347-356 – reference: 8) Kurita T, Ohe T, Shimizu W, Suyama K, Aihara N, Takaki H, Kamakura S, Shimomura K : Early afterdepolarization like activity in patients with class Ia-induced long QT syndrome and torsade de pointes. PACE, 1997 ; 20 : 695-705 – reference: 11) Shimizu W, Antzelevitch C : Effects of a K+channel opener to reduce transmural disper-sion of repolarization and prevent torsade de pointes in LQT1, LQT2 and LQT3 models of the long-QT syndrome. Circulation, 2000 ; 102 : 706-712 |
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| Snippet | 先天性QT延長症候群（LQTS）の薬物治療としては，β遮断薬のほかに，内向き電流を減少させるIb群Na＋チャネル遮断薬やCa拮抗薬，外向き電流を増加させるK＋チャネル開口薬などが考えられる．Ca拮抗薬のベラパミルは，単相性活動電位（monophasic action potential ;... |
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| SubjectTerms | Ca拮抗薬 QT延長症候群 イオンチャネル 遺伝子 |
| Title | 先天性QT延長症候群におけるelectrical stormに対するCa拮抗薬の使い方 |
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