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アリズム発生性右心室不形成症:アメリカでの経験
Darshan Dalal1, Khurram Nasir, Chandra Bomma
1Division of Cardiology, The Johns Hopins University School of Medicine, Baltimore, MD, USA.
Circulation
|December 14, 2005
まとめ
アリズム発生性右心室不形成症 (ARVD) は,若年成人ではしばしば心拍数や昏睡を呈する. 早期診断とインプラント可能な心動除細動器 (ICD) 治療により,ARVD患者の死亡率が著しく低下します.
科学分野:
- 心臓病学 心臓病学
- 遺伝学 遺伝学とは
- 遺伝性疾患は,遺伝的に受け継がれる病気です.
背景:
- アリズム発生性右心室不形成症 (ARVD) は,遺伝性心筋病です.
- 進行的な右心室機能障害と心室不律が特徴です.
- 遺伝的異質性は,疾患の呈現と進行の多様性に寄与する.
研究 の 目的:
- ARVDの臨床表現,特徴,生存率,自然経歴を記述する.
- 米国を拠点とする大規模な患者コホートにおけるアウトカムを分析する.
- ARVDの臨床管理と遺伝カウンセリングを伝えるために.
主な方法:
- 米国で100人のARVD患者の遡及的分析.
- 患者の人口統計,症状の提示,診断の時間表,治療方法のレビュー.
- カプラン・マイヤー生存分析と急性心臓死 (SCD) と心臓移植を含むアウトカムの評価.
主要な成果:
- 発症時の平均年齢は26歳であり,32%の症例で家族的なパターンがみられる.
- 常見の症状には,心拍数 (27%),昏睡 (26%),突然心死 (SCD) (23%) が含まれていた.
- 平均生存期間は60歳であり,死亡率は早期診断とインプラント可能な心臓変容器-除細動器 (ICD) のインプラントで低かった.
結論:
- ARVDは,通常,人生の2~5十年の間に発現します.
- 遅れた診断は一般的ですが,ICD治療は死亡率を低下させます.
- ARVDの呈現と経過の有意な変動は,遺伝的要因の重要性を強調しています.
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