ST Brugada J (coved-shaped)st 0.2 T (negative T wave) (RBBB pattern) RBBB pattern J 0.2 ST (saddleback configuration) 0.1 (trough)st (biphasic)t J ST

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1 Brugada 前言 Brugada 1992 Pedro Brugada Josep Brugada (1) (syncope) (sudden cardiac death) (V1-V3) ST (right bundle branch block, RBBB) QT 1998 Brugada (chromosome 3p21) (cardiac sodium channel gene, SCN5A) (2) Brugada 1 中國醫藥大學附設醫院見習醫師 2 台大醫院家庭醫學部住院醫師 3 台大醫院家庭醫學部主任 Brugada syndrome, sudden cardiac death, ventricular fibrillation (sudden unexplained nocturnal death syndrome, SUNDS) SUNDS Brugada (3) Brugada 臨床表現及診斷 Brugada (polymorphic ventricular tachycardia, PVT) (ventricular fibrillation, VF) (nocturnal agonal respiration) (palpitations) (4) 家庭醫學與基層醫療第二期 47

2 ST Brugada J (coved-shaped)st 0.2 T (negative T wave) (RBBB pattern) RBBB pattern J 0.2 ST (saddleback configuration) 0.1 (trough)st (biphasic)t J ST 0.1 T Brugada Brugada ST (5) Brugada (autosomal dominant with incomplete penetrance) 20% (sodium channel) 10% L (L-type calcium channel) 1% -3- (glycerol-3-phosphate dehydrogenase-like peptide, GPD1-L) (ATP-sensitive potassium channel) (6) Brugada 8~10 (testosterone) (transient 表一 Brugada 症候群在心電圖上表現之三種型態第一型 ( 具診斷性 ) 第二型 ( 疑似病例 ) 第三型 ( 疑似病例 ) J 波振幅 0.2 毫伏特 0.2 毫伏特 0.2 毫伏特 ST 節段型態與振福拱形 0.2 毫伏特鞍背形 0.1 毫伏特拱形或鞍背形 <0.1 毫伏特 T 波型態負向正向或雙相正向心電圖右胸前導程之 ST 節段表現 ( 以 V2 為例 ) 參考來源 : 參考資料 5 48 家庭醫學與基層醫療第二期

3 表二 Brugada 症候群的診斷 1. 心電圖上觀察到至少一個右前胸導程出現第一型 ST 節段上升 2. 家族史家族成員在 45 歲之前發生心因性猝死家族成員在心電圖上表現第一型 ST 節段上升 3. 心律不整引起之相關症狀昏厥 (syncope) 癲癇 (seizures) 夜間瀕死狀呼吸 (nocturnal agonal respiration) 4. 心室性心律不整至少符合一項標準多形性心室心搏過速 (polymorphic ventricular tachycardia, PVT) 心室顫動 (ventricular fibrillation, VF) 參考來源 : 參考資料 4,5 outward potassium current, Ito) Brugada (4) Brugada (repolarization hypothesis) (depolarization hypothesis) (5,7,8) (repolarization hypothesis) Brugada (action potential, AP) (action potential notch) ST L (plateau phase) ST T J (heterogeneity) (phase 2 reentry) (ventricular extrasystoles) (PVT) (VF) (depolarization hypothesis) 家庭醫學與基層醫療第二期 49

4 表三 Brugada 症候群的亞型 (subtype) 分類亞型突變基因離子電流 / 蛋白質離子通道病變 BrS1 SCN5A Nav1.5 I Na BrS2 GPD1-L GPD1-L I Na BrS3 CACNA1C Cav1.2 I CaL BrS4 CACNB2b I CaL (β 次單元 ) I CaL BrS5 SCN1B I Na (β1 次單元 ) I Na BrS6 KCNE3 MiRP2 I to BrS7 SCN3B I Na (β3 次單元 ) I Na BrS8 KCNJ8 Kir6.1 I K-ATP BrS9 CACNA2D1 I CaL (α2δ1 次單元 ) I CaL BrS10 KCND3 Kv4.3 I to BrS11 MOG1 MOG1 I Na BrS12 ABCC9 SUR2A I K-ATP 註 :INa: 鈉離子通道電流 ;I CaL :L 型鈣離子通道電流 ;I to : 短暫鉀離子向外電流 ;I K-ATP : 腺苷三磷酸敏感性鉀離子通道電流參考來源 : 參考資料 6 Brugada PR (CBBB) (electrophysiology study, EPS) Brugada (H-V interval) / (mapping) H-V interval SCN5A Brugada (magnetic resonance imaging, MRI) (myocardial biopsies) (right ventricular outflow tract, RVOT) (RVOT) (electrical reentry) 治療及預後 Brugada 50 家庭醫學與基層醫療第二期

5 (implantable cardioverter defibrillator, ICD) ICD Brugada ICD Brugada J et al (5, 9) Brugada / (radiofrequency catheter ablation, RFCA) 78% / 89% (10) ICD (I to ) / (I Na / I CaL ) Quinidine I to (11) Brugada ST SCN5A (tachyarrhythmia) (12) 45 Brugada Brugada Brugada / (5) (genetic testing) Brugada (13) SCN5A Brugada (mutation-specific genetic testing) 結語 Brugada Brugada 30% 70% SCN2B (missense mutation) 家庭醫學與基層醫療第二期 51

6 Brugada (14) Brugada 參考資料 1. Brugada P, Brugada J: Right bundle branch block, persistent ST segment elevation and sudden cardiac death: a distinct clinical and electrocardiographic syndrome: a multicenter report. J Am Coll Cardiol 1992; 20: Chen Q, Kirsch GE, Zhang D et al: Genetic basis and molecular mechanism for idiopathic ventricular fibrillation. Nature 1998; 392: Vatta M, Dumaine R, Varghese G et al: Genetic and biophysical basis of sudden unexplained nocturnal death syndrome (SUNDS), a disease allelic to Brugada syndrome. Hum Mol Genet 2002; 11: Shimizu W: Clinical features of Brugada syndrome. Journal of Arrhythmia 2013; 29: Berne P, Brugada J: Brugada syndrome Circ J 2012; 76: Kusano KF: Brugada syndrome: recent understanding of pathophysiological mechanism and treatment. Journal of Arrhythmia 2013; 29: Bébarová M: Arrhythmogenesis in Brugada syndrome: Impact and constrains of current concepts. Int J Cardiol In Press, corrected proof, Juang JMJ, Chen CY, Liu YB et al: Prevalence of atrial fibrillation in patients with Brugada syndrome in Taiwan. Acta Cardiol Sin 2013; 29: Brugada J, Brugada R, Brugada P: Determinants of sudden cardiac death in individuals with the electrocardiographic pattern of Brugada syndrome and no previous cardiac arrest. Circ J 2003; 108: Nademanee K, Veerakul G, Chandanamattha P et al: Prevention of ventricular fibrillation episodes in Brugada syndrome by catheter ablation over the anterior right ventricular outflow tract epicardium. Circ J 2011; 123: Campuzano O, Sarquella-Brugada G, Brugada R et al: Brugada syndrome. els. John Wiley & Sons, Ltd (February 2013). 12. Probst V, Veltmann C, Eckardt L et al: Longterm prognosis of patients diagnosed with Brugada syndrome: Results from the FINGER Brugada syndrome registry. Circ 2010; 121: Ackerman MJ, Priori SG, Willems S et al: HRS/EHRA expert consensus statement on the state of genetic testing for the channelopathies and cardiomyopathies: this document was developed as a partnership between the Heart Rhythm Society (HRS) and the European Heart Rhythm Association (EHRA). Europace 2011; 13: Riuró H, Beltran-Alvarez P, Tarradas A et al: A missense mutation in the sodium channel 2 Subunit reveals SCN2B as a new candidate gene for Brugada syndrome. Hum Mutat 2013 (4 April 2013 in Wiley Online Library). 52 家庭醫學與基層醫療第二期

家庭醫業圖1 Brugada syndrome之心電圖表現過去BS的心電圖特徵分為三型(A)型I EKG (B)型II EKG (C)型III EKG 2012年最新之標準只有型I(穹隆型)及型II(鞍背型) 且主要以型I做為診斷資料來源參考資料6 圖也有類似的變化直到1992年Br

家庭醫業圖1 Brugada syndrome之心電圖表現過去BS的心電圖特徵分為三型(A)型I EKG (B)型II EKG (C)型III EKG 2012年最新之標準只有型I(穹隆型)及型II(鞍背型) 且主要以型I做為診斷資料來源參考資料6 圖也有類似的變化直到1992年Br 家庭醫業布魯格達氏症候群 (Brugada Syndrome) 蔡崇煌1 黃素雲2 醫師發現有此疾患者亦應安排其他家庭前言成員做篩選以防心因性猝死突發性心跳停止(sudden cardiac arrest)大多起因於心室顫動(ventricular fibrillation, VF) 而VF絕大部分是由冠狀流行病學動脈疾病(coronary artery disease, CAD)所