幼児期における長期的結果 左心室非収縮性心筋症: 全国の人口ベースの研究の結果
William Y Shi1,2,3,4, Margarita Moreno-Betancur3,4, Alan W Nugent5
1Department of Cardiac Surgery (W.Y.S.).
Circulation
|March 9, 2018
まとめ
幼児期左心房非収縮症 (LVNC) は,主に乳児期に広がった心疾患として現れます. 長期的には 拡張性心筋病の子どもと比べると 悪い結果が出ます
科学分野:
- 小児心臓科
- 心血管研究
- 心臓 疾患 の 遺伝
背景:
- 幼児期左心房非収縮症 (LVNC) は,長期的な予後が不確実な稀な先天性心疾患である.
- LVNCの早期診断と理解は,効果的な管理と患者の成果の改善に不可欠です.
研究 の 目的:
- 左心室非収縮 (LVNC) と診断された子供の長期的なアウトカムを調査する.
- 拡張性心筋病 (LVNC- D) の患者と拡張性心筋病患者のアウトカムを比較する.
主な方法:
- オーストラリア国立小児心筋病研究 (1987年−1996年) のデータを活用した.
- リスク調整のための傾向スコア分析を用いた,拡張性心筋病症のLVNC患者の比較結果.
- 死亡率と移植リスクを評価するために競合リスク分析とコックス回帰を用いた.
主要な成果:
- LVNCは9. 2%の心筋病変で,診断時の平均年齢は0. 3歳でした.
- 閉塞性心不全は,LVNC症例の83%で主要な症状であり,93%が拡張性フェノタイプ (LVNC- D) を表した.
- 診断から15年後には,死亡または移植のリスクはLVNCの45%で,LVNC- Dは死亡または移植のリスクが有意に増加していた (HR2. 3,P=0. 0012).
結論:
- LVNCの症状のある子供は,典型的には幼児期に膨張した表型を示します.
- 幼児期LVNCの長期的アウトカム,特に拡張性フェノタイプは,拡張性心筋病のマッチング症例よりも悪い.
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