筋ジストロフィー症患者における心室遅延電位の検討: Duchenne型進行性筋ジストロフィー症と筋緊張性ジストロフィー症の比較
Duchenne型進行性筋ジストロフィー症 (DMD) 患者と筋緊張性ジストロフィー症 (MyD) 患者を対象とし, 平均加算心電図による心室遅延電位 (LP) の検出を試み, さらに心エコー図 (2-Decho) により左室障害の有無を検討した.標準12誘導心電図でQRS時間が100msec以下で脚ブロックの所見のないDMD19名 (17±6歳) とMyD9名 (39±19歳) では, 2-D echoでは左室収縮指標はMyDに比しDMDで有意に低値であり, 左室壁運動異常はDMDで有意に高頻度を示した.2-D echoの結果は一般にDMDに心不全が高頻度である事実と対応していた.DMDでは...
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| Published in | 心電図 Vol. 10; no. 6; pp. 814 - 820 |
|---|---|
| Main Authors | , , , , , , , , , , , |
| Format | Journal Article |
| Language | Japanese |
| Published |
一般社団法人 日本不整脈心電学会
1990
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| Subjects | |
| Online Access | Get full text |
| ISSN | 0285-1660 1884-2437 |
| DOI | 10.5105/jse.10.814 |
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| Abstract | Duchenne型進行性筋ジストロフィー症 (DMD) 患者と筋緊張性ジストロフィー症 (MyD) 患者を対象とし, 平均加算心電図による心室遅延電位 (LP) の検出を試み, さらに心エコー図 (2-Decho) により左室障害の有無を検討した.標準12誘導心電図でQRS時間が100msec以下で脚ブロックの所見のないDMD19名 (17±6歳) とMyD9名 (39±19歳) では, 2-D echoでは左室収縮指標はMyDに比しDMDで有意に低値であり, 左室壁運動異常はDMDで有意に高頻度を示した.2-D echoの結果は一般にDMDに心不全が高頻度である事実と対応していた.DMDではLPを全く検出せず, MyDでは4例 (44%) にLPを認め有意に高率であった.LP陽性のMyDでHolter心電図を施行したが, 心室頻拍 (VT) の所見は得られなかった.しかし, 文献上, MyDではVTやVTによる突然死の報告がある.LP陽性MyDの一部では, LPがVTの発生に関連する可能性があり, 今後の検討が必要である. |
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| AbstractList | Duchenne型進行性筋ジストロフィー症 (DMD) 患者と筋緊張性ジストロフィー症 (MyD) 患者を対象とし, 平均加算心電図による心室遅延電位 (LP) の検出を試み, さらに心エコー図 (2-Decho) により左室障害の有無を検討した.標準12誘導心電図でQRS時間が100msec以下で脚ブロックの所見のないDMD19名 (17±6歳) とMyD9名 (39±19歳) では, 2-D echoでは左室収縮指標はMyDに比しDMDで有意に低値であり, 左室壁運動異常はDMDで有意に高頻度を示した.2-D echoの結果は一般にDMDに心不全が高頻度である事実と対応していた.DMDではLPを全く検出せず, MyDでは4例 (44%) にLPを認め有意に高率であった.LP陽性のMyDでHolter心電図を施行したが, 心室頻拍 (VT) の所見は得られなかった.しかし, 文献上, MyDではVTやVTによる突然死の報告がある.LP陽性MyDの一部では, LPがVTの発生に関連する可能性があり, 今後の検討が必要である. |
| Author | 小竹, 寛 竹下, 研三 斎藤, 誠 都田, 裕之 長谷川, 純一 井後, 雅之 土井, 哲也 笠木, 重人 縄田, 隆浩 大野, 耕策 福木, 昌治 真柴, 裕人 |
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| References | 10) Hiromasa S, Ikeda T, Kubota K, Takada S, Hattori N, Nishimura M, Watanabe Y: A family with myotonic dystrophy associated with diffuse cardiac conduction disturbances as demonstrated by His bundle electrocardiography. Am Heart J 111: 85, 1986 11) Simson MB: Use of signals in the terminal QRS complex to identify patients with ventricular tachycardia after myocardial infarction. Circulation 64: 235, 1981 15) Rozanski JJ, Mortara D, Myerburg RJ, Castellanos A : Body surface detection of delayed depolarizations in patients with recurrent ventricular tachycardia and left ventricular aneurysm. Circulation 63: 1172, 1981 17) Pollak SJ, Kertes PJ, Bredlau CE, Walter PF : Influence of left ventricular function on signal averaged late potentials in patients with coronary artery disease with and without ventricular tachycardia. Am Heart J 110: 747, 1985 12) Denes P, Santarelli P, Hauser RG, Uretz EF: Quantitative analysis of the high-frequency components of the terminal portion of the body surface QRS in normal subjects and in patients with ventricular tachycardia. Circulation 67: 1129, 1983 1) 本多瑞枝: Duchenne型進行性筋ジストロフィー症の心機能の経年的観察: 死亡例と生存例の比較. 千葉医学61: 187, 1985 14) Kuchar DL, Thorburn CW, Sammel NL : Prediction of serious arrhythmic events after myocardial infarction : signal-averaged electrocardiogram, Holter monitoring and radionuclide ventriculography. JACC 9: 531, 1987 3) Hiromasa S, Ikeda T, Kubota K, Hattori N, Coto H, Maldonado C, Kupersmith J : Ventricular tachycardia and sudden death in myotonic dystrophy. Am Heart J 115: 914, 1988 16) Zimmermann M, Adamec R, Simonin P, Richez J : Prognostic significance of ventricular late potentials in coronary artery disease. Am Heart J 109: 725, 1985 8) 広正修一, 池田孝之, 久保田幸次, 高田重男, 山 本正和, 中村由紀夫, 中村暁, 森下大樹, 八木伸 治, 広瀬龍吉, 西田哲也, 網谷茂樹, 臼田和生, 服部信, 西村昌雄, 渡部良夫: 筋緊張性筋ジストロフィー症8例の心臓電気生理学的検討. 心臓 19: 168, 1987 2) Perloff JK, Stevenson WG, Roberts NK, Cabeen W, Weiss J : Cardiac involvement in myotonic muscular dystrophy (Steinert's disease) : A prospective study of 25 patients. Am J Cardiol 54: 1074, 1984 18) Ozawa Y, Yakubo S, Tanigawa N, Nagasawa M, Kojima R, Jinno L, Hibiya K, Watanabe I, Saito T, Saito S, Hatano M : The clinical evaluation of the late potentials in patients with ventricular arrhythmias. Jpn Circulation J 51: 230, 1987 21) Reeves WC, Griggs R, Nanda NC, Thomson K, Gramiak R: Echocardiographic evaluation of cardiac abnormalities in Duchenne's dystrophy and myotonic muscular dystrophy. Arch Neurol 37: 273, 1980 6) Melacini P, Buja G, Fasoli G, Angelini C, Armani M, Scognamiglio R, Volta SD: The natural history of cardiac involvement in myotonoc dystrophy : an eightyear follow-up in 17 patients. Clin Cardiol 11: 231, 1988 19) Yotsukura M, Miyagawa M, Kanamaru S, Okada M, Ishihara T, Ishikawa K : Late potentials in patients with Duchenne's progressive muscular dystrophy. Circulation (suppl II) 78: II-52, 1988 23) Mehra R, Zeiler RH, Gough WB, El-Sherif N: Reentrant ventricular arrhythmias in the late myocardial period. 9. Electrophysiologic -anatomic correlation of reentrant circuits. Circulation 67: 11, 1983 13) 小沢友紀雄: Late potential. 心臓ペーシング5: 285, 1989 22) Boineau JP, Cow JL : Slow ventricular activation in acute myocardial infarction. A source of re-entrant premature ventricular contractions. Circulation 48: 702, 1973 24) 檜澤一夫: シンポジウム1: 筋緊張性ジストロフィー症. 5. 筋緊張性ジストロフィーの心筋病理を中心に. 臨床神経学23: 1082, 1983 7) Cannon DS, Wyman MG, Goldreyer BN : Clinical and induced ventricular tachycardia in a patient with myotonic dystrophy. JACC 4: 625, 1984 25) Hartwig GB, Rao KR, Radoff FM, Coleman RE, Jones RH, Roses AD : Radionuclide angiocardiographic analysis of myocardial function in myotonic muscular dystrophy. Neurology 33: 657, 1983 9) Clements SD Jr, Colmers RA, Hurst JW: Myotonia Dystrophia. Ventricular arrhythmias, intraventricular conduction abnormalities, atrioventricular block and Stokes-Adams attacks successfully treated with permanent transvenous pacemaker. Am J Cardiol 37: 933, 1976 20) Swinyard CA, Deaver GG, Greenspan L : Gradients of functional ability of importance in rehabilitation of patients with progressive muscular and neuromuscular diseases. Arch Phys Med 38: 574, 1957 5) Grigg LE, Chan W, Mond HG, Vohra JK, Downey WF : Ventricular tachycardia and sudden death in myotonic dystrophy : clinical, electrophysiologic and pathologic features. JACC 6: 254, 1985 4) Nguyen HH, Wolfe III JT, Holmes DR Jr, Edwards WD : Pathology of the cardiac conduction system in myotonic dystrophy : a study of 12 cases. JACC 11: 662, 1988 |
| References_xml | – reference: 3) Hiromasa S, Ikeda T, Kubota K, Hattori N, Coto H, Maldonado C, Kupersmith J : Ventricular tachycardia and sudden death in myotonic dystrophy. Am Heart J 115: 914, 1988 – reference: 16) Zimmermann M, Adamec R, Simonin P, Richez J : Prognostic significance of ventricular late potentials in coronary artery disease. Am Heart J 109: 725, 1985 – reference: 4) Nguyen HH, Wolfe III JT, Holmes DR Jr, Edwards WD : Pathology of the cardiac conduction system in myotonic dystrophy : a study of 12 cases. JACC 11: 662, 1988 – reference: 15) Rozanski JJ, Mortara D, Myerburg RJ, Castellanos A : Body surface detection of delayed depolarizations in patients with recurrent ventricular tachycardia and left ventricular aneurysm. Circulation 63: 1172, 1981 – reference: 6) Melacini P, Buja G, Fasoli G, Angelini C, Armani M, Scognamiglio R, Volta SD: The natural history of cardiac involvement in myotonoc dystrophy : an eightyear follow-up in 17 patients. Clin Cardiol 11: 231, 1988 – reference: 1) 本多瑞枝: Duchenne型進行性筋ジストロフィー症の心機能の経年的観察: 死亡例と生存例の比較. 千葉医学61: 187, 1985 – reference: 13) 小沢友紀雄: Late potential. 心臓ペーシング5: 285, 1989 – reference: 19) Yotsukura M, Miyagawa M, Kanamaru S, Okada M, Ishihara T, Ishikawa K : Late potentials in patients with Duchenne's progressive muscular dystrophy. Circulation (suppl II) 78: II-52, 1988 – reference: 21) Reeves WC, Griggs R, Nanda NC, Thomson K, Gramiak R: Echocardiographic evaluation of cardiac abnormalities in Duchenne's dystrophy and myotonic muscular dystrophy. Arch Neurol 37: 273, 1980 – reference: 9) Clements SD Jr, Colmers RA, Hurst JW: Myotonia Dystrophia. Ventricular arrhythmias, intraventricular conduction abnormalities, atrioventricular block and Stokes-Adams attacks successfully treated with permanent transvenous pacemaker. Am J Cardiol 37: 933, 1976 – reference: 11) Simson MB: Use of signals in the terminal QRS complex to identify patients with ventricular tachycardia after myocardial infarction. Circulation 64: 235, 1981 – reference: 22) Boineau JP, Cow JL : Slow ventricular activation in acute myocardial infarction. A source of re-entrant premature ventricular contractions. Circulation 48: 702, 1973 – reference: 2) Perloff JK, Stevenson WG, Roberts NK, Cabeen W, Weiss J : Cardiac involvement in myotonic muscular dystrophy (Steinert's disease) : A prospective study of 25 patients. Am J Cardiol 54: 1074, 1984 – reference: 7) Cannon DS, Wyman MG, Goldreyer BN : Clinical and induced ventricular tachycardia in a patient with myotonic dystrophy. JACC 4: 625, 1984 – reference: 25) Hartwig GB, Rao KR, Radoff FM, Coleman RE, Jones RH, Roses AD : Radionuclide angiocardiographic analysis of myocardial function in myotonic muscular dystrophy. Neurology 33: 657, 1983 – reference: 8) 広正修一, 池田孝之, 久保田幸次, 高田重男, 山 本正和, 中村由紀夫, 中村暁, 森下大樹, 八木伸 治, 広瀬龍吉, 西田哲也, 網谷茂樹, 臼田和生, 服部信, 西村昌雄, 渡部良夫: 筋緊張性筋ジストロフィー症8例の心臓電気生理学的検討. 心臓 19: 168, 1987 – reference: 24) 檜澤一夫: シンポジウム1: 筋緊張性ジストロフィー症. 5. 筋緊張性ジストロフィーの心筋病理を中心に. 臨床神経学23: 1082, 1983 – reference: 10) Hiromasa S, Ikeda T, Kubota K, Takada S, Hattori N, Nishimura M, Watanabe Y: A family with myotonic dystrophy associated with diffuse cardiac conduction disturbances as demonstrated by His bundle electrocardiography. Am Heart J 111: 85, 1986 – reference: 17) Pollak SJ, Kertes PJ, Bredlau CE, Walter PF : Influence of left ventricular function on signal averaged late potentials in patients with coronary artery disease with and without ventricular tachycardia. Am Heart J 110: 747, 1985 – reference: 5) Grigg LE, Chan W, Mond HG, Vohra JK, Downey WF : Ventricular tachycardia and sudden death in myotonic dystrophy : clinical, electrophysiologic and pathologic features. JACC 6: 254, 1985 – reference: 12) Denes P, Santarelli P, Hauser RG, Uretz EF: Quantitative analysis of the high-frequency components of the terminal portion of the body surface QRS in normal subjects and in patients with ventricular tachycardia. Circulation 67: 1129, 1983 – reference: 14) Kuchar DL, Thorburn CW, Sammel NL : Prediction of serious arrhythmic events after myocardial infarction : signal-averaged electrocardiogram, Holter monitoring and radionuclide ventriculography. JACC 9: 531, 1987 – reference: 20) Swinyard CA, Deaver GG, Greenspan L : Gradients of functional ability of importance in rehabilitation of patients with progressive muscular and neuromuscular diseases. Arch Phys Med 38: 574, 1957 – reference: 18) Ozawa Y, Yakubo S, Tanigawa N, Nagasawa M, Kojima R, Jinno L, Hibiya K, Watanabe I, Saito T, Saito S, Hatano M : The clinical evaluation of the late potentials in patients with ventricular arrhythmias. Jpn Circulation J 51: 230, 1987 – reference: 23) Mehra R, Zeiler RH, Gough WB, El-Sherif N: Reentrant ventricular arrhythmias in the late myocardial period. 9. Electrophysiologic -anatomic correlation of reentrant circuits. Circulation 67: 11, 1983 |
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| Snippet | Duchenne型進行性筋ジストロフィー症 (DMD) 患者と筋緊張性ジストロフィー症 (MyD) 患者を対象とし, 平均加算心電図による心室遅延電位 (LP) の検出を試み, さらに心エコー図 (2-Decho)... |
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| SubjectTerms | Duchenne型進行性筋ジストロフィー症 左室障害 心室遅延電位 突然死 筋緊張性ジストロフィー症 |
| Title | 筋ジストロフィー症患者における心室遅延電位の検討: Duchenne型進行性筋ジストロフィー症と筋緊張性ジストロフィー症の比較 |
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