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Japanese

Title 遺伝性不整脈の診断と治療 : QT延長症候群, QT短縮症候群, CPVT, Brugada症候群について
Subtitle Review 【シリーズ : 不整脈】
Authors 鈴木博
Authors (kana)
Organization 新潟大学医歯学総合病院魚沼地域医療教育センター
Journal 日本小児循環器学会雑誌
Volume 35
Number 4
Page 249-263
Year/Month 2019 / 11
Article 報告
Publisher 日本小児循環器学会
Abstract 遺伝性不整脈は, 心筋活動電位を形成するイオンチャネルとこれに関連する蛋白などをコードする遺伝子変異によって発症する疾患の総称である. 1957年にQT延長症候群が初めて報告され, 現在ではカテコラミン誘発多形性心室頻拍, Brugada症候群, QT短縮症候群, 早期再分極症候群, 進行性心臓伝導障害も遺伝性不整脈とみなされている. 若年突然死の主要な原因であるが, 早期発見と介入により予防しうる. 遺伝子解析の進歩により, 原因不明であった失神, 突然死に遺伝性不整脈の診断がつき, 個々により適した管理, 治療が行われるようになってきている. 近年, 日本循環器学会のガイドラインも改訂された. これも踏まえて本稿では, QT延長症候群, QT短縮症候群, カテコラミン誘発多形性心室頻拍, Brugada症候群について述べる.
Practice 医学一般
Keywords inherited arrhythmias, long-QT syndrome, short-QT syndrome, catecholaminergic polymorphic ventricular tachycardia, Brugada syndrome

English

Title Diagnosis and Management of Inherited Arrhythmias : Long- and Short-QT Syndromes, Catecholaminergic Polymorphic Ventricular Tachycardia, and Brugada Syndrome
Subtitle Reviews
Authors Hiroshi Suzuki
Authors (kana)
Organization Uonuma Institute of Community Medicine, Niigata University Medical and Dental Hospital
Journal Pediatric Cardiology and Cardiac Surgery
Volume 35
Number 4
Page 249-263
Year/Month 2019 / 11
Article Report
Publisher Japanese Society of Pediatric Cardiology and Cardiac Surgery
Abstract Inherited arrhythmia defines a group of diseases caused by mutations on genes encoding for ion-channel proteins and proteins that regulate ion channels. In 1957, long-QT syndrome was first reported and described as an inherited arrhythmia. Currently, catecholaminergic polymorphic ventricular tachycardia, Brugada syndrome, short-QT syndrome, early repolarization syndrome, and progressive cardiac conduction disturbance are also considered to be inherited arrhythmias. Although inherited arrhythmia is a major cause of sudden cardiac death in young individuals, it can be prevented by earlier diagnosis and clinical management. Progress in genetic analysis has enabled the diagnosis of inherited arrhythmias in patients with syncope and/or cardiac arrest of unknown origin and facilitated more suitable management of inherited arrhythmias. Guidelines for diagnosis and management of inherited arrhythmias have recently been published by the Japanese Circulation Society. Here on the basis of the guidelines, I review inherited arrhythmias with a particular focus on long- and short-QT syndromes, catecholaminergic polymorphic ventricular tachycardia, and Brugada syndrome.
Practice General medicine
Keywords inherited arrhythmias, long-QT syndrome, short-QT syndrome, catecholaminergic polymorphic ventricular tachycardia, Brugada syndrome
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