A case of Hypokinetic non-dilated cardiomyopathy, who was found in screening program at school
学校心臓検診の目的の一つは器質的疾患の早期発見である．今回我々は，学校心臓検診で指摘された心室性期外収縮（PVC）を契機にHypokinetic non-dilated cardiomyopathy（HNDC）の診断に至った症例を経験した． 症例は15歳男児，学校検診でPVCを指摘され精査目的に近医を受診した．近医で再検された12誘導心電図においてV1-V3でT波の陰転化を指摘された．心臓超音波検査（心エコー）で壁運動の低下と左室壁の軽度の菲薄化を認め，心筋症を疑われたため精査目的に当院紹介受診した．PVCは右室流出路起源で運動負荷にて消失した．心筋症精査のため行われた心筋生検で中等度の心筋の...
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| Published in | Shinzo Vol. 51; no. 9; pp. 941 - 948 |
|---|---|
| Main Authors | , , , , , , , , , , , |
| Format | Journal Article |
| Language | Japanese |
| Published |
Japan Heart Foundation
15.09.2019
公益財団法人 日本心臓財団 |
| Subjects | |
| Online Access | Get full text |
| ISSN | 0586-4488 2186-3016 |
| DOI | 10.11281/shinzo.51.941 |
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| Abstract | 学校心臓検診の目的の一つは器質的疾患の早期発見である．今回我々は，学校心臓検診で指摘された心室性期外収縮（PVC）を契機にHypokinetic non-dilated cardiomyopathy（HNDC）の診断に至った症例を経験した． 症例は15歳男児，学校検診でPVCを指摘され精査目的に近医を受診した．近医で再検された12誘導心電図においてV1-V3でT波の陰転化を指摘された．心臓超音波検査（心エコー）で壁運動の低下と左室壁の軽度の菲薄化を認め，心筋症を疑われたため精査目的に当院紹介受診した．PVCは右室流出路起源で運動負荷にて消失した．心筋症精査のため行われた心筋生検で中等度の心筋の線維化を認め，左室腔の拡大を伴わないHNDCの診断に至った．診断後はβブロッカーの投与を開始し，PVCは減少し心機能はおおむね横ばいで推移している．また原因検索のため患児，家族の73種の心筋症関連遺伝子について次世代シーケンサを用いて解析したが，有意な遺伝子変異を認めなかった．日常診療や検診の場面でPVCを発見した際は適切なフォローアップや介入の必要があると考えられた． |
|---|---|
| AbstractList | 学校心臓検診の目的の一つは器質的疾患の早期発見である．今回我々は，学校心臓検診で指摘された心室性期外収縮（PVC）を契機にHypokinetic non-dilated cardiomyopathy（HNDC）の診断に至った症例を経験した． 症例は15歳男児，学校検診でPVCを指摘され精査目的に近医を受診した．近医で再検された12誘導心電図においてV1-V3でT波の陰転化を指摘された．心臓超音波検査（心エコー）で壁運動の低下と左室壁の軽度の菲薄化を認め，心筋症を疑われたため精査目的に当院紹介受診した．PVCは右室流出路起源で運動負荷にて消失した．心筋症精査のため行われた心筋生検で中等度の心筋の線維化を認め，左室腔の拡大を伴わないHNDCの診断に至った．診断後はβブロッカーの投与を開始し，PVCは減少し心機能はおおむね横ばいで推移している．また原因検索のため患児，家族の73種の心筋症関連遺伝子について次世代シーケンサを用いて解析したが，有意な遺伝子変異を認めなかった．日常診療や検診の場面でPVCを発見した際は適切なフォローアップや介入の必要があると考えられた． |
| Author | Shiozawa, Ryousuke Yata, Soichirou Hayano, Satoshi Nishio, Tomohiro Iwashima, Satoru Kubota, Toshiko Kubota, Akira Jyoukou, Yumiko Morikawa, Shuji Sakurai, Shiki Kurebayashi, Nobutake Tanaka, Tomohiro |
| Author_FL | 久保田 晃 城向 裕美子 森川 修司 早野 聡 久保田 登志子 田中 智大 矢田 宗一郎 岩島 覚 櫻井 史記 塩澤 亮輔 西尾 友宏 紅林 伸丈 |
| Author_FL_xml | – sequence: 1 fullname: 田中 智大 – sequence: 2 fullname: 岩島 覚 – sequence: 3 fullname: 櫻井 史記 – sequence: 4 fullname: 早野 聡 – sequence: 5 fullname: 塩澤 亮輔 – sequence: 6 fullname: 西尾 友宏 – sequence: 7 fullname: 矢田 宗一郎 – sequence: 8 fullname: 久保田 登志子 – sequence: 9 fullname: 久保田 晃 – sequence: 10 fullname: 城向 裕美子 – sequence: 11 fullname: 紅林 伸丈 – sequence: 12 fullname: 森川 修司 |
| Author_xml | – sequence: 1 fullname: Yata, Soichirou organization: Department of Pediatrics, Chutoen General Medical Center – sequence: 1 fullname: Morikawa, Shuji organization: Department of Cardiology, Chutoen General Medical Center – sequence: 1 fullname: Kubota, Toshiko organization: Department of Pediatrics, Chutoen General Medical Center – sequence: 1 fullname: Jyoukou, Yumiko organization: Department of Cardiology, Chutoen General Medical Center – sequence: 1 fullname: Shiozawa, Ryousuke organization: Department of Pediatrics, Chutoen General Medical Center – sequence: 1 fullname: Nishio, Tomohiro organization: Department of Pediatrics, Chutoen General Medical Center – sequence: 1 fullname: Kubota, Akira organization: Department of Pediatrics, Chutoen General Medical Center – sequence: 1 fullname: Tanaka, Tomohiro organization: Department of Pediatrics, Chutoen General Medical Center – sequence: 1 fullname: Sakurai, Shiki organization: Department of Pediatrics, Chutoen General Medical Center – sequence: 1 fullname: Kurebayashi, Nobutake organization: Department of Cardiology, Chutoen General Medical Center – sequence: 1 fullname: Iwashima, Satoru organization: Department of Pediatrics, Chutoen General Medical Center – sequence: 1 fullname: Hayano, Satoshi organization: Department of Pediatrics, Chutoen General Medical Center |
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| References | 9) 北田実男，中島節子，中川 正，ほか：基礎心疾患を認めない不整脈患者の長期予後．日小循誌1993；9：420-430 8) Tsuji A, Nagashima M, Hasegawa S, et al：Long-term followup of idiopathic ventricular arrhythmias in otherwise normal children. Jpn Circ J 1995；59：654-662 1) Pettersen MD, Du W, Skeens ME, Humes RA：Regression equations for calculation of z scores of cardiac structures in a large cohort of healthy infants, children, and adolescents：an echocardiographic study. J Am Soc Echocardiogr 2008；21(8)：922-934 4) Abelmann WH, Lorell BH：The challenge of cardiomyopathy. J Am Coll Cardiol 1989；13(6)：1219-1239 14) Lipshultz SE, Orav EJ, Wilkinson JD, et al；Pediatric Cardiomyopathy Registry Study Group：Risk stratification at diagnosis for children with hypertrophic cardiomyopathy：an analysis of data from the Pediatric Cardiomyopathy Registry. Lancet 2013；382：1889-1897 13) Felker GM, Thompson RE, Hare JM, et al：Underlying causes and long-term survival in patients with initially unexplained cardiomyopathy. N Engl J Med 2000；342(15)：1077-1084 6) Pinto YM, Elliott PM, Arbustini E, et al：Proposal for a revised definition of dilated cardiomyopathy, hypokinetic non-dilated cardiomyopathy, and its implications for clinical practice：a position statement of the ESC working group on myocardial and pericardial diseases. Eur Heart J 2016；37(23)：1850-1858 17) Tandri H, Castillo E, Ferrari VA, et al：Magnetic resonance imaging of arrhythmogenic right ventricular dysplasia：sensitivity, specificity, and observer variability of fat detection versus functional analysis of the right ventricle. J Am Coll Cardiol 2006；48：2277-2284 7) 太田八千雄，三浦正次，浜田 勇，ほか：札幌市学校心音心電図検診で発見された心筋症7例の検討．日小循誌1999；15(1)：33-39 19) 市田蕗子，佐地 勉，梶野浩樹，ほか：平成21年度稀少疾患サーベイランス調査結果．日小循誌2010；26：348-350 2) Buechel EV, Kaiser T, Jackson C, et al：Normal right- and left ventricular volumes and myocardial mass in children measured by steady state free precession cardiovascular magnetic resonance. J Cardiovasc Magn Reson 2009；11：19 10) 長嶋正實：学校検診で検出される不整脈の特徴とその対策．小児科臨床2000；53：1949-1957 20) Miura K, Nakagawa H, Morikawa Y, et al：Epidemiology of idiopathic cardiomyopathy in Japan：results from a nationwide survey, Heart 2002；87：126-130 21) Poole-Wilson PA, Swedberg K, Cleland JG, et al；Carvedilol Or Metoprolol European Trial Investigators：Comparison of carvedilol and metoprolol on clinical outcomes in patients with chronic heart failure in the Carvedilol Or Metoprolol European Trial(COMET)：randomised controlled trial. Lancet 2003；362：7-13 22) Kantor PF, Abraham JR, Dipchand AI, et al：The impact of changing medical therapy on transplantation-free survival in pediatric dilated cardiomyopathy. J Am Coll Cardiol 2010；55：1377-1384 23) 小野安生，福嶌教偉：診断と治療 最近の進歩 先天性心疾患 小児期拡張型心筋症の自然予後：心臓移植対象例における検討．Annual Review循環器2008年版．東京：中外医学社；2008．p.297-301 11) Driscoll D, Allen HD, Atkins DL, et al：Guidelines for evaluation and management of common congenital cardiac problems in infants, children, and adolescents. A statement for healthcare professionals from the Committee on Congenital Cardiac Defects of the Council on Cardiovascular Disease in the Young, American Heart Association. Circulation 1994；90：2180-2188 15) Jefferies JL, Towbin JA：Dilated cardiomyopathy. Lancet 2010；375：752-762 5) 西川俊郎，宮沢要一朗：心筋症；高尾篤良，門間和夫，中澤 誠，中西敏雄・編：臨床発達心臓病学 改訂3版．東京：中外医学社；2001．p.704-717 16) 松岡留美子：小児の心筋炎，心筋症 心筋症の遺伝子診断．小児科2008；49：803-808 18) 福嶌教偉：心筋疾患 若年期拡張型心筋症．別冊循環器症候群Ⅲ(第2版)その他の循環器疾患を含めて．東京：日本臨牀社；2008．p.134-138 3) Dec GW, Fuster V：Idiopathic dilated cardiomyopathy. N Engl J Med 1994；331(23)：1564-1575 12) 住友直方，石川広己，泉田直己，ほか：日本循環器学会/日本小児循環器学会合同ガイドライン．2016年版 循環器ガイドラインシリーズ．学校心臓検診のガイドライン．Guidelines for Heart Disease Screening in Schools(JCS 2016/JSPCCS 2016)http://www.j-circ.or.jp/guideline/pdf／JCS2016_sumitomo_h.pdf(2016.11.25既刊 |
| References_xml | – reference: 12) 住友直方，石川広己，泉田直己，ほか：日本循環器学会/日本小児循環器学会合同ガイドライン．2016年版 循環器ガイドラインシリーズ．学校心臓検診のガイドライン．Guidelines for Heart Disease Screening in Schools(JCS 2016/JSPCCS 2016)http://www.j-circ.or.jp/guideline/pdf／JCS2016_sumitomo_h.pdf(2016.11.25既刊) – reference: 15) Jefferies JL, Towbin JA：Dilated cardiomyopathy. Lancet 2010；375：752-762 – reference: 9) 北田実男，中島節子，中川 正，ほか：基礎心疾患を認めない不整脈患者の長期予後．日小循誌1993；9：420-430 – reference: 6) Pinto YM, Elliott PM, Arbustini E, et al：Proposal for a revised definition of dilated cardiomyopathy, hypokinetic non-dilated cardiomyopathy, and its implications for clinical practice：a position statement of the ESC working group on myocardial and pericardial diseases. Eur Heart J 2016；37(23)：1850-1858 – reference: 21) Poole-Wilson PA, Swedberg K, Cleland JG, et al；Carvedilol Or Metoprolol European Trial Investigators：Comparison of carvedilol and metoprolol on clinical outcomes in patients with chronic heart failure in the Carvedilol Or Metoprolol European Trial(COMET)：randomised controlled trial. Lancet 2003；362：7-13 – reference: 5) 西川俊郎，宮沢要一朗：心筋症；高尾篤良，門間和夫，中澤 誠，中西敏雄・編：臨床発達心臓病学 改訂3版．東京：中外医学社；2001．p.704-717 – reference: 13) Felker GM, Thompson RE, Hare JM, et al：Underlying causes and long-term survival in patients with initially unexplained cardiomyopathy. N Engl J Med 2000；342(15)：1077-1084 – reference: 19) 市田蕗子，佐地 勉，梶野浩樹，ほか：平成21年度稀少疾患サーベイランス調査結果．日小循誌2010；26：348-350 – reference: 3) Dec GW, Fuster V：Idiopathic dilated cardiomyopathy. N Engl J Med 1994；331(23)：1564-1575 – reference: 17) Tandri H, Castillo E, Ferrari VA, et al：Magnetic resonance imaging of arrhythmogenic right ventricular dysplasia：sensitivity, specificity, and observer variability of fat detection versus functional analysis of the right ventricle. J Am Coll Cardiol 2006；48：2277-2284 – reference: 11) Driscoll D, Allen HD, Atkins DL, et al：Guidelines for evaluation and management of common congenital cardiac problems in infants, children, and adolescents. A statement for healthcare professionals from the Committee on Congenital Cardiac Defects of the Council on Cardiovascular Disease in the Young, American Heart Association. Circulation 1994；90：2180-2188 – reference: 20) Miura K, Nakagawa H, Morikawa Y, et al：Epidemiology of idiopathic cardiomyopathy in Japan：results from a nationwide survey, Heart 2002；87：126-130 – reference: 7) 太田八千雄，三浦正次，浜田 勇，ほか：札幌市学校心音心電図検診で発見された心筋症7例の検討．日小循誌1999；15(1)：33-39 – reference: 10) 長嶋正實：学校検診で検出される不整脈の特徴とその対策．小児科臨床2000；53：1949-1957 – reference: 14) Lipshultz SE, Orav EJ, Wilkinson JD, et al；Pediatric Cardiomyopathy Registry Study Group：Risk stratification at diagnosis for children with hypertrophic cardiomyopathy：an analysis of data from the Pediatric Cardiomyopathy Registry. Lancet 2013；382：1889-1897 – reference: 16) 松岡留美子：小児の心筋炎，心筋症 心筋症の遺伝子診断．小児科2008；49：803-808 – reference: 2) Buechel EV, Kaiser T, Jackson C, et al：Normal right- and left ventricular volumes and myocardial mass in children measured by steady state free precession cardiovascular magnetic resonance. J Cardiovasc Magn Reson 2009；11：19 – reference: 4) Abelmann WH, Lorell BH：The challenge of cardiomyopathy. J Am Coll Cardiol 1989；13(6)：1219-1239 – reference: 18) 福嶌教偉：心筋疾患 若年期拡張型心筋症．別冊循環器症候群Ⅲ(第2版)その他の循環器疾患を含めて．東京：日本臨牀社；2008．p.134-138 – reference: 1) Pettersen MD, Du W, Skeens ME, Humes RA：Regression equations for calculation of z scores of cardiac structures in a large cohort of healthy infants, children, and adolescents：an echocardiographic study. J Am Soc Echocardiogr 2008；21(8)：922-934 – reference: 22) Kantor PF, Abraham JR, Dipchand AI, et al：The impact of changing medical therapy on transplantation-free survival in pediatric dilated cardiomyopathy. J Am Coll Cardiol 2010；55：1377-1384 – reference: 8) Tsuji A, Nagashima M, Hasegawa S, et al：Long-term followup of idiopathic ventricular arrhythmias in otherwise normal children. Jpn Circ J 1995；59：654-662 – reference: 23) 小野安生，福嶌教偉：診断と治療 最近の進歩 先天性心疾患 小児期拡張型心筋症の自然予後：心臓移植対象例における検討．Annual Review循環器2008年版．東京：中外医学社；2008．p.297-301 |
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| Snippet | 学校心臓検診の目的の一つは器質的疾患の早期発見である．今回我々は，学校心臓検診で指摘された心室性期外収縮（PVC）を契機にHypokinetic non-dilated cardiomyopathy（HNDC）の診断に至った症例を経験した．... |
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| SubjectTerms | DCM Hypokinetic non-dilated cardiomyopathy PVC 学校検診 心室性期外収縮(PVC) 拡張型心筋症(DCM) |
| Title | A case of Hypokinetic non-dilated cardiomyopathy, who was found in screening program at school |
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