左心室内静脈機能不全による心筋縮症: SHaRe レジストリからの洞察
Peter Marstrand1,2, Larry Han3, Sharlene M Day4
1Cardiovascular Division, Brigham and Women's Hospital, Boston, MA (P.M., N.K.L., C.Y.H.).
Circulation
|April 2, 2020
まとめ
左心室内静脈機能不全 (HCM- LVSD) による多動性心筋病は,患者の8%に影響する. 75%が有害事象を経験し, 35%が8. 4年以内に複合的な結果に直面しています.
科学分野:
- 心臓病科
- 遺伝学
- 心不全 の 研究
背景:
- 左心室収縮機能不全 (HCM-LVSD) を伴う高縮性心筋症は,エジェクション分数 < 50% で定義される.
- HCM-LVSDの予後と自然史は,その希少性のために十分に理解されていません.
研究 の 目的:
- HCM-LVSDの自然史を記述する.
- HCM-LVSDの予後と発生の予測要因を特定する.
主な方法:
- 11の国際専門センター (SHaRe Registry) の6793人のHCM患者のデータを利用した.
- 結果とリスク要因を分析するためにコックスの比例リスクモデルを使用した.
主要な成果:
- HCM- LVSDはコホートの8% (553人の患者) で発生した.
- HCM- LVSD患者の75%が有害事象を経験し,35%が複合的な結果 (死亡,移植,LVAD) を達成し,平均期間は8. 4年であった.
- 複合的なアウトカムを予測する要因には,マルチサルコメリック変種,心房細動,およびLVEF<5%が含まれていた. 発生したHCM- LVSDの予測要因には,LVサイズ,壁の厚さ,LVEF50-60%,遅いガドリニウム増強,およびサーコメリック変異が含まれています.
結論:
- HCM- LVSDは,HCM患者の大部分に影響を与え,しばしば深刻な結果をもたらします.
- 遺伝的要因,特にサルコメリック変種 (複数または薄いフィラメント) は,予後と疾患の進行の両方に重要な役割を果たします.
- HCM- LVSDにおける有害事象と疾患の発症の重要な予測要因は,臨床的特徴と心臓画像検査の結果である.
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