QT QT QT QT Brugada Brugada Brugada Brugada Brugada Brugada QT QTQTc Torsade de pointes T-wave alternans Notched T wave in leads Circulation Journal Vol. 71, Suppl. IV, 2007 1257
TdP QT Na + ICD QT QT QT QT Brugada Brugada electrical storm QTBrugada QTSchwartz QT QTQT QT QT Brugada consensus conferencetype coved type Brugada type type type type QTBrugada QTBrugada 1258 Circulation Journal Vol. 71, Suppl. IV, 2007
QTBrugada QT QTQT Brugada Brugada A BC AHAACC QTLQTSQT torsade de pointestdp LQTS LQTS Romano-Ward Jervell-Lange Nielsen LQTS1 LQTS1 Romano-Ward LQTS QT QT Romano-Ward Jervell-Lange Nielsen QT QT H2 K + Mg 2+ Ca 2+ Circulation Journal Vol. 71, Suppl. IV, 2007 1259
Romano-Ward LQTLQT QT../ QTc msec ½ LQT LQT LQT LQTLQTLQT LQT LQTLQT QT QTTdP QTQT QT 1 TdP.. QT QT SNP LQTS KCNQ KCNHHERG SCNA LQTS QT RRQT TdP QTQT TdP QT TdP LQTS K + LQTNa + LQT Ca + LQT APD QT Monophasic action potentialmap QTMAP MAPD early afterdepolarizationead humptdp EAD MAPD spacial dispersion of repolarization SDRTdPSDR LQTS LQTSTdP LQTLQTLQT TdPTdP action potential durationapd Epi TdP TdPQRS M Endo EAD LQTSM APD TDRTdP SDR TDR 1 LQTSQT TdP 1260 Circulation Journal Vol. 71, Suppl. IV, 2007
Epi-M-Endo KCNQ1KCNH2SCN5AANKB KCNE1KCNE2KCNJ2CACNA1CCAV3SCN4B I Ks I Kr I K1 latei Na Ca 2 II Ca LQT15 APD LQT263 M APD LQT1-10 EAD QT QT Torsade de Pointes Miyasaka.mVST..Atarashi.mVcovedST..mVcovedST. Yamakawa.mV covedsaddlebackst. 2 2 Brugada* Priori** 144 268 144 190 48 152 139/5 251/17 120/24 135/55 152/48 51.2 53.4 41 19 15 34 72 29 33 46 33 66 51 29 12 5350/96 6397/154 41.50 VFEPS 7187/123 5265/125 73 33 VF/VTEPS 81 62 65 68 SAECG 7066/95 6389/141 VSA 2215/67 187/38 VFVTEPSSAECGVSA *Brugada J, et al. Circulation - **Priori S, et al. Circulation - Circulation Journal Vol. 71, Suppl. IV, 2007 1261
Brugada VFAF AF AF Na + STT ST EPS VF VT VF vs VF/././ VF/../. type EPS VF BrugadaEPS VF/ VT / ST/ PrioriVF/ VT type ST type SCNA type Brugada Na + SCNA SCNABrugada SCNA Na + Na + Na + trafficking defect Na + loss of function Na + loss of functionbrugada VF Antzelevitch Brugada 2 APD notch notchnotch notch K + I to I to notch Na + notchdome LCa + I to K + K + I k ATP K + Na + Ca + notch dome ST 2A Na + loss of functioni to notch spike and dome 1262 Circulation Journal Vol. 71, Suppl. IV, 2007
Antzelevitch C. J Cardiovasc Electrophysiol. Feb -. STJ APD APD saddle backst 2Bnotch STT 2CBrugada covedstnotch Ca + domeloss of domedome dome 2Dphase reentry2e QT Schwartz 3 QTc torsade de pointes TdPT-wave alternans notched T wavetdp T-wave alternans TdP notched T wave QT dispersion LQTS QTQT dispersion T QT Circulation Journal Vol. 71, Suppl. IV, 2007 1263
points 1 ABazettQT 0.48sec 1/2 3 0.46-0.47sec 1/2 2 0.45sec 1/2 1 Btorsade de pointes* 2 CT 1 D3 notched T 1 E 0.5 2 A* 2 1 B 0.5 3 Adefinite LQTS 1 B30 0.5 high probabilityor definite intermediate probability low probability *torsade de pointes * Schwartz PJ,et al. Circulation. ; : -. Bifid T wavet T T T Notched T wave Romano- Ward QTRR BazettQT/RR / BazettQTQT QTQT RRFridericia QRS QT TdP TdP T T-wave alternans T-wave alternans QT LQTQT LQT QT QTc QTc msec ½ 4 5 1 QT QTc 440msecQT 2 QT 3 a 1 2 LQT1LQT2 b QT 1264 Circulation Journal Vol. 71, Suppl. IV, 2007
QTQT msec QTT QT dispersion T peak-endqt RR HRV TdP QT QTT QT T-wave alternans T-wave alternans TdP TWA QT b 1 QT QTc 440msecQT 2 QT shortead monophasic action potentialmap QT MAP EAD short-long- QT PESTdP APD R-Rshort-longshort PES QTPES MAP QTT EAD EAD 6 QTLQTS Romano-Ward loss of functiongain of functionapd QT LQTLQTKCNQ KCNE LQTLQTKCNH a 1 QT b 1 C 2Torsade de pointestdp B 3 TdP QT C 1 QT QT C Circulation Journal Vol. 71, Suppl. IV, 2007 1265
KCNE K + I K I Ks I Kr I Ks I Kr LQT SCNANa + late Na + I Na LQTS LQTS Romano-Ward LQT LQT LQTLQTLQTLQT LQTLQTLQT T torsade de pointes LQTS LQTLQT KCNQKCNH QT TdP TdP QT TdP QT TdP TdPK + TdP QT LQT 7 Ca + LQT TimothyEAD QTI Ks I Kr K + K + QT K + QTQT torsade de pointestdp TdP TdP QT LQT1LQT2 a QT b 1266 Circulation Journal Vol. 71, Suppl. IV, 2007
SCNALQT LQT QT QT Torsade de PointesTdP QT ICD QT TdP ICD 9 b 1 ICD 2 torsade de pointes B 8ICD TdP A Torsade de pointestdp 3 ab TdP a a a a b b TdP ab QT LQT b QT torsades de pointestdp QT 1 QT K + BazettQTQTc. sec ½ QT QTtorsades de pointes QT A g mg/ B Circulation Journal Vol. 71, Suppl. IV, 2007 1267
/ B / B.mmol/L C mg QTQT TdP Na + /K + K + QT EADtorsade de pointes TdP QTTdP Vaughan- WilliamsATdP QTc msec ½ TdP TdPT long-shortsequence EAD QTT /QT TdP /QT TdP ICD QT QT TdP QT g Wildeconsensus report V-V J mm STtype STcovedtype type saddle backst mm mm consensus conferencetype STcovedsaddle back Brugadatype type type type 1268 Circulation Journal Vol. 71, Suppl. IV, 2007
Brugada Brugadatype type Brugada Brugada Vaugahn WilliamsIA IC Na + ST coved ST J.mV mg/kg mg/kg mg/kg Brugada SAECG LPLP LP Brugada Brugada 10 BrugadaNa + SCNA SCNAQTLQT PCCD Na + BrugadaSCNA Brugada BrugadaSCNA PQ HV Brugada.. BrugadaBrugada Brugada SCNA SCNA 1 covedbrugada 2 covedbrugada a 1 saddle backbrugada 2 saddle backbrugada 3 Brugada coved saddle back B b 1 Brugada coved saddle back 2 Brugada coved saddle back Circulation Journal Vol. 71, Suppl. IV, 2007 1269
Brugada ICDBrugada ICD ICD.g/Kg/min C mg/ mg/c I Ca I to mg/ Ca + I to K + mg/ Brugada ICD ICD ICD 11 ICD 1 2 Brugada coved 3 a b VF a a a a b b b a b Brugada Brugadasaddle back 1270 Circulation Journal Vol. 71, Suppl. IV, 2007