小児左心房非収縮症における死亡率と突然死 医療上の紹介センターにおける死亡率と突然死
Samuel T Brescia1, Joseph W Rossano, Ricardo Pignatelli
1Texas Children's Hospital/Section of Pediatric Cardiology, 6621 Fannin, Houston, TX 77030, USA. jjkim@texaschildrens.org
Circulation
|May 2, 2013
まとめ
子どもの左心房非収縮症 (LVNC) は高い死亡率を有しており,しばしば心律不全や心臓機能不全に関連しています. 小児のLVNC患者における正常な心臓機能と尺寸は,突然の心臓死のリスクが低いことを示しています.
科学分野:
- 心臓病学 心臓病学
- ペディアトリック・カルディオロジー
- 遺伝学 遺伝学とは
背景:
- 左心室非収縮症 (LVNC) は珍しい心筋病です.
- 過剰な左心房のトラベキュレーション,心筋機能不全,高い死亡率が特徴です.
- LVNCの臨床表現は多様で,不律性や突然の心臓死を含む.
研究 の 目的:
- 孤立した左心房非収縮症と診断された子供の臨床的特徴とアウトカムを調査する.
- この小児集団における死亡率と突然心疾患による死亡に関連する要因を特定する.
主な方法:
- 1990年1月から2009年1月までの間に孤立左心室非収縮 (LVNC) と診断された小児患者の遡及レビュー.
- 生まれながらの心臓病変を有する患者の除外.
- 臨床データの分析,心臓機能,心電図の異常,心律失調,死亡率を含む.
主要な成果:
- 242人の子どもが孤立したLVNCと診断されました.
- 死亡率は12.8%で,5.4%が移植を受けた.
- 心臓機能不全 (62%) と心律乱 (33.1%) は,死亡率の増加と強く関連していました.
- ECGの異常は87%に存在し,再偏振異常はより高い死亡率と関連していました.
- 心臓の突然死は6.2%の患者で発生し,主に心臓の異常寸法または機能不全を有する患者で発生しました.
結論:
- 左心室非収縮症 (LVNC) は,子供の死亡率が高い.
- 心臓機能不全と心室不律は,小児性LVNCにおける死亡率の有意な予測要因である.
- 前回の心律乱症のない正常な心臓の寸法と機能を持つ子供は,突然死のリスクが低い.
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