急性死と左心房の関わりによる心律乱動性筋症
Chris Miles1, Gherardo Finocchiaro1, Michael Papadakis1
1Cardiology Clinical Academic Group, St George's University Hospitals' NHS Foundation Trust and Molecular and Clinical Sciences Institute, St George's University of London, United Kingdom (C.M., G.F., M.P., B.G., J.W., B.E., J.B., G.P.-W., E.P. C.P., A.M., A.A., M.T., S.S., E.R.B., M.N.S.).
Circulation
|February 1, 2019
まとめ
アリズム不全性心筋病 (ACM) は,主として男性やスポーツ選手において,予期せぬ突然心臓死 (SCD) を引き起こします. 解剖は左心房の関わりを 頻繁に明らかにし 現在の診断基準は 双心房ACMの症例を 見逃す可能性があることを示唆しています
科学分野:
- 心臓病科
- 病理学について
- 遺伝学
背景:
- 動脈動乱性心筋病 (ACM) は,繊維脂肪の置換と突然心臓死 (SCD) を引き起こす遺伝性心疾患である.
- 当初は右心室疾患と考えられていたが,現在は双心室疾患として認識されている.
- この研究は,突然の心臓死亡の大群における 病理的,遺伝的,および臨床的要因を調査しています.
研究 の 目的:
- 急性心臓死 (SCD) 症例における不律性心筋病 (ACM) の特徴を調査する.
- ACMにおける二室関わりの流行を評価する.
- ACMの現在の診断基準の有効性を評価する.
主な方法:
- 25年間にわたる 5205件の突然心臓死 (SCD) の分析.
- 専門家による心臓病学的検査と組織学的評価
- 臨床履歴,症状,競技スポーツへの参加の記録; 死亡後の遺伝子検査はサブセットで行われました.
主要な成果:
- 202例 (4%) がACMと診断され,主に男性 (82%) と若者 (平均年齢35. 4歳) であった.
- 死亡者の78%は心臓の症状がなかった. 20%は競争力のあるアスリートだった.
- 左心室の関わりは87%の症例で,二心室疾患は70%で,検査された症例の25%はACMに関連する遺伝子の病原性変異がありました.
結論:
- ACMによる突然心臓死 (SCD) は,主に男性に影響し,多くの場合,症状のない運動選手の運動中に起こります.
- 左心室の関わりは 解剖で診断されたACMで一般的です
- 現在の診断基準では,死前の双心筋ACMを適切に特定できない可能性があります.
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