Effectiveness of Flecainide in a Catecholaminergic Polymorphic Ventricular Tachycardia Patient with Cardiac Ryanodine Receptor Gene（RyR2） Mutation： A Case Report

Name: Work Video:
Duration: 3 min 30 s
Masataka Fukuoka; Yoko Yoshida; Shintaro Kishimoto; Tsugutoshi Suzuki; Mitsuhiro Fujino; Yasuhiro Hirano; Yuki Ozawa; Eiji Ehara; Yosuke Murakami; Yoshihide Nakamura
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'https://openalex.org/W1988998632', 'https://openalex.org/W2018074436', 'https://openalex.org/W2035699845', 'https://openalex.org/W2055928084', 'https://openalex.org/W2068482636', 'https://openalex.org/W2072499242', 'https://openalex.org/W2081524260', 'https://openalex.org/W2097312037', 'https://openalex.org/W2100477955', 'https://openalex.org/W2119956887', 'https://openalex.org/W2166519632'], 'related_works': ['https://openalex.org/W4243824770', 'https://openalex.org/W4221093914', 'https://openalex.org/W2797995848', 'https://openalex.org/W2791495462', 'https://openalex.org/W2324073567', 'https://openalex.org/W2122971014', 'https://openalex.org/W2055928084', 'https://openalex.org/W2053430609', 'https://openalex.org/W1989056964', 'https://openalex.org/W1965719501'], 'abstract_inverted_index': {'患者は特記すべき既往歴・家族歴のない10歳男児で，運動時の失神を主訴に受診した．トレッドミル運動負荷心電図検査では上室頻拍と多形性心室期外収縮が誘発され，ホルター心電図検査では多形性心室頻拍が記録され，カテコラミン誘発多形性心室頻拍（CPVT）と診断．遺伝子検査で筋小胞体カルシウム放出チャネルの心臓リアノジン受容体遺伝子（RyR2）に既報のミスセンス変異が同定された．運動制限の後，プロプラノロール（3mg/kg/': [0], '日）とフレカイニド（100mg/m2/': [1], '日）の併用を開始，プロプラノロールは著しい徐脈のため減量（2mg/kg/': [2], '日），フレカイニドは段階的に増量した（最終投与量150mg/m2/': [3], '日）．治療により上室不整脈は消失し，フレカイニドは用量依存性に心室不整脈を抑制した．治療開始後1年の経過観察期間で失神の再発はない．フレカイニドはナトリウムチャネル遮断作用に加え，心臓リアノジン受容体への直接作用を有し，細胞内カルシウム過負荷による撃発活動を抑制する薬剤である．フレカイニドはβ遮断薬との併用によりCPVT': [4], '治療において重要な役割を果たすと考えられた．': [5]}, 'cited_by_api_url': 'https://api.openalex.org/works?filter=cites:W2324073567', 'counts_by_year': [], 'updated_date': '2024-12-15T16:25:02.653160', 'created_date': '2016-06-24'}
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