ECG & EP CASES 38 Journal of Cardiac Arrhythmia 서론 브루가다 증후군은 심전도의 우측 전흉부 유도(right precordial leads)(V 1 ~V 3 )에서 ST분절의 상승 소견과 더불 어, 임상경과 중 심실세동(ventricular fibrillation)에 의한 심인성 급사라는 매우 위중한 심장사건(cardiac event)을 보이는 증례들이 보고되면서 주목을 받게 되었다. 1 브루가 다 증후군은 심근 세포막(myocardial cell membrane)에 존재하는 나트륨 통로(Na + channel)의αsubunit를부호화 (encoding)하는 SCN5A, 3q21 유전자의 돌연변이와 연관 성이 확인되었으며, 2 이들 이상 나트륨 통로들은 생리적 범 위보다 높은 온도에서 그 기능 이상이 심화되는 특징을 지 녀, 이들 환자에서 고열이 동반되는 질환이 발생할 경우 브 루가다 증후군의 특징적 심전도 소견이 더 잘 나타나는 것 으로 알려져 있다. 3,4 증례 44세 남성 환자가 내원 3시간 전부터 시작된 우측 전흉 부 흉통을 호소하며 응급실로 내원하였다. 현 병력: 내원 전 저녁 소주 1병을 마시고 난 후 자정부 브루가다 증후군 가톨릭대학교 의과대학 내과학교실 이만영 A case of brugada syndrome presented as chest pain ABSTRACT Brugada syndrome was described in 1992 as a new clinical entity characterized by electrocardiographic ST- segment elevation in the right precordial leads (V1~V3) and the occurrence of sudden cardiac death (SCD) during its clinical course. Brugada syndrome is known to be linked with the mutations of SCN5A, the gene encoding α subunit of the cardiac sodium channel. Furthermore, these abnormal cardiac sodium channels were found to show variable degrees of dysfunctioning in relation to changes in body temperature and electrocardiogram (ECG) findings of Brugada syndrome sometimes happen to reveal with the presence of high fever. We experienced a 44-year-old man with Brugada syndrome in whom the diagnosis of acute coronary syndrome had been mistakenly made because of an initial clinical presentation with complains of severe chest pain and accompanying ST segment elevations. The ST segment elevations in leads I and aVL, which extended the right precordial leads, led the physician to suspect transient pericarditis caused by coexisting pneumonia. In this case, a diagnosis of idiopathic Brugada syndrome was reconfirmed by a flecainide challenge test. Key words: ■ brugada syndrome ■ ECG ■ fever ■ chest pain
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CASES 3 > îÐ 1 µe-arrhythmia.org/upload/pdf/arrhythmia-11-2-38.pdf1 ECG pattern (B), and with normal temperature on HD 7: normal ECG with resolved ST-segment elevation (C). A B
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Transcript
ECG
& E
P C
ASES
38 Journal of Cardiac Arrhythmia
서론
브루가다 증후군은 심전도의 우측 전흉부 유도(right
precordial leads)(V1~V3)에서 ST분절의상승소견과더불
어, 임상경과중심실세동(ventricular fibrillation)에의한
심인성 급사라는 매우 위중한 심장사건(cardiac event)을
보이는 증례들이 보고되면서 주목을 받게 되었다.1 브루가
다 증후군은 심근 세포막(myocardial cell membrane)에
존재하는나트륨통로(Na+ channel)의αsubunit를부호화
(encoding)하는 SCN5A, 3q21 유전자의 돌연변이와 연관
성이확인되었으며,2 이들이상나트륨통로들은생리적범
위보다높은온도에서그기능이상이심화되는특징을지
녀, 이들환자에서고열이동반되는질환이발생할경우브
루가다증후군의특징적심전도소견이더잘나타나는것
으로알려져있다.3,4
증례
44세남성환자가내원 3시간전부터시작된우측전흉
부흉통을호소하며응급실로내원하 다.
현병력: 내원전저녁소주1병을마시고난후자정부
브루가다증후군
가톨릭대학교 의과대학 내과학교실 이 만
A case of brugada syndrome presented as chest pain
ABSTRACTBrugada syndrome was described in 1992 as a new clinical entity characterized by electrocardiographic ST-
segment elevation in the right precordial leads (V1~V3) and the occurrence of sudden cardiac death (SCD)
during its clinical course. Brugada syndrome is known to be linked with the mutations of SCN5A, the gene
encoding αsubunit of the cardiac sodium channel. Furthermore, these abnormal cardiac sodium channels
were found to show variable degrees of dysfunctioning in relation to changes in body temperature and
electrocardiogram(ECG) findings of Brugada syndrome sometimes happen to reveal with the presence of high
fever. We experienced a 44-year-old man with Brugada syndrome in whom the diagnosis of acute coronary
syndrome had been mistakenly made because of an initial clinical presentation with complains of severe chest
pain and accompanying ST segment elevations. The ST segment elevations in leads I and aVL, which extended
the right precordial leads, led the physician to suspect transient pericarditis caused by coexisting pneumonia.
In this case, a diagnosis of idiopathic Brugada syndrome was reconfirmed by a flecainide challenge test.
Figure 1. The twelve-lead ECG on admission: marked ST segment elevations were noted on the right precordial leads(V1-V4) and subtle ST segment elevations also noted in lead 1 and aVL (A), with a fever of 38.5℃ on HD 2: Brugada Type1 ECG pattern (B), and with normal temperature on HD 7: normal ECG with resolved ST-segment elevation (C).
A
B
C
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Figure 2. Chest CT scan on admission showed pulmonary infiltration of the right middle lobe lateral segment, andpulmonary infiltration of the right lower lobe anterobasal segment with pleural involvement.
Figure 3. On flecainide provocation test, the baseline electrocardiaram(ECG) showed normal (A), and after 5 minutes,the ECG showed the Brugada Type 1 ECG pattern (B). V5, V6 leads were placed 1 intercoastal space above leads V1 andV2 respectively.
A
A
B
B
ECG
& E
P C
ASES
42 Journal of Cardiac Arrhythmia
행하 으나, 심실성빈맥은유발되지않았다.
진단 및 예후 평가 차원에서 심장전기생리 검사의 역할
및가치에대해서는아직논란이많은상태이나, 본환자의
경우처럼급사의가족력이없고, 무증상의환자는주의하며
추적관찰하는진료지침에따라외래에서경과관찰하기로
하고환자는퇴원하 다.
References
1. Brugada P, Brugada J. Right bundle branch block, persistent STsegment and sudden cardiac death: a distinct clinical and
electrocardiographic syndrome. A multicenter report. J Am CollCardiol. 1992;20:1391-1396.
2. Chen Q, Kirsch GE, Zhang D, Brugada R, Brugada J, Brugada P,Potenza D, Moya A, Borggrefe M, Breithardt G, Oritz-Lopez R,Wang Z. Genetic basis and molecular mechanisms for idiopathicventricular fibrillation. Nature. 1998;392:293-296.
3. Dumaine R, Towbin JA, Brugada P, Vatta M, Nesterenko DV,Nesterenko VV, Brugada J, Brugada P, Antzelevitch C. Ionicmechanisms responsible for the electrocardiographic phenotypeof the Brugada syndrome are temperature dependent. Circ Res.1999;85:803-809.
4. Antzelevitch C. The Brugada syndrome: ionic basis andarrhythmia mechanisms. J Cardiovasc Electrophysiol.2001;12:268-272.