Brugada 11 QT 12 WPW sudden infant death syndrome :SIDS 1 QT 2 SCN5A WPW 2 3CPVT 4 QT LQT

2003 2004 Guidelines for Risks and Prevention of Sudden Cardiac Death (JCS 2005) 1 ECG 2 HRV 3 BRS 4 T TWA 5 LP 6 7 8 Circulation Journal Vol. 69, Suppl. IV, 2005 1209

2003 2004 1 2 3 4 5 6 7 8 9 10 Brugada 11 QT 12 WPW 13 14 15 1 2 3 1 2 1sudden infant death syndrome :SIDS 1 QT 2 SCN5A 3 4 5 2 1 WPW 2 3CPVT 4 QT LQTS 5 3 Commotio Cordis 4 5 6 1 1 30 40 5 10 % 1 2 75 80 % 3 3 4 5 6 7 1210 Circulation Journal Vol. 69, Suppl. IV, 2005

1 1075 4.9% QT 9.3% 3.8% 8.0% 28.7% Brugada 12.7% 14.0% 18.6% 80 % 30 % Brugada QT 8 9 2 3 1 Risk stratification 2 3-1 12 15 14 16 17 1 1 Circulation Journal Vol. 69, Suppl. IV, 2005 1211

2003 2004 1 ST-T QT QT dispersion WPW Brugada QT 1 Framingham T 5 33 % 21 % 14 2 ST-T ST T 2 18 T 19 3 QT QT QT QT Dispersion 12 QT QT 20 21 QT Dispersion T 22 23 QT Dispersion 40 50 msec 65 msec QT Dispersion 24 26 ACE 27 28 QT Dispersion 24 29 31 30 QT Dispersion 32 33 37 DIAMOND-CHF 38 ELITE 39 QT Dispersion 40 % QT Dispersion 35 msec 40 QT Dispersion 41 42 43 QT Dispersion 70 msec 44 45 65 msec 46 T T T peak-end Transmural Dispersion of Repolarization TDR 47 48 V5 V6 12 T peak-end TDR QT LQT1 Torsade de Pointes TdP 49 51 T peak-end 51 QT TDR TdP 52 53 4 2 54 1987 Kleiger 55 n 808 SDNN 24 NN 141 39 msec 100 msec 50 100 msec 50 mscc 3 4 SDNN 50 msec 100 msec 5.3 SDNN 1212 Circulation Journal Vol. 69, Suppl. IV, 2005

1 N 416 RR Triangular index 24 NN 37 15 msec 16 msec 16 19 msec 20 msec 3 20 20 msec 32 7 56 SDNN SDANN 5 NN 127 35 msec 57 21 SDNN 70 msec 5.3 58 ULF 0.0033 0.04 Hz VLF 0.04 Hz LF 0.04 0.15 Hz 1170 416 msec2 HF 0.15 0.40 Hz 975 203 msec2 total power 569 ULF VLF VLF 60 1 61 2 15 RR 62 SDANN pnn50 NN 50 ms 63 RR SDNN RMSSD NN 27 15 msec SDNN 100 100 64 65 68 3 58 69 73 BRS 74 75 LaRovere BRS 76 ATRAMI Autonomic Tone and Reflex After Myocardial Infarction 25 1284 80 recent MI BRS HRV LP 58 BRS 3.0 Infarct related artery IRA BRS 3.0 IRA BRS 77 BRS 3.0 SDNN 70 msec 35 % BRS 3.0 78 ATRAMI BRS 3.5 3.5 3 14.3 % 1.1 % p 0.05 BRS ACE 79 BRS 80 4 TWA T 1 TWA V TWA TWA EPSn 83 TWA EPS 81 EPS n 313 EPS TWA 82 n 248 TWA 83 8.1 Tapanainen 84 TWA 85 88 TWA 2 Circulation Journal Vol. 69, Suppl. IV, 2005 1213

2003 2004 3 40 % 30 % TWA TWA TWA 90 95 % TWA 61 89 90 91 92 93 Brugada 94 95 96 5 QRS LP 97 101 filtered QRS f-qrs 40 msec Root Mean Square RMS40 40 V LAS40 LP 1980 102 105 LP EF LP 30 % LP 90 % LP 106 107 1990 LP ACE LP 108 109 CABGEF 35 % LP CABG Patch Trial 110 32 40 % MUSTT LP f-qrs 114 msec 5 1 LP 28 % 17 % 111 LP 30 % 21 % 36 % MUSTT LP LP 112 LP 113 LP LP 114 Brugada LP 94 6 115 1 10 20 % 10 116 118 QT 119 120 121 HV 2.3 % HV 100 msec 22 25 % 122 123 124 Wenckebach 150 His 125 2 1 3 80 % 115 126 127 130 2 1214 Circulation Journal Vol. 69, Suppl. IV, 2005

0.40 15 30 % 131 132 133 135 136 137 139 140 141 WPW 250 270 msec RR 200 msec 142 145 Brugada 1 3 50 80 % 146 152 147 150 146 149 153 46 4.7 18 154 7 155 157 158 159 20 mmhg 160 164 165 168 169 QT 170 171 174 175 176 168 8 QT LQTS 1 LQTS 8 LQT1-8 LQT1 2 3 3 177 178 LQT1 K Kinetic Iks LQT2 K Kinetic IKr LQT3 SCN5A Na LQT5 LQT6 KCNE1 KCNE2 K QT KCNJ2 K IK1 Jervell Lange-Nielsen LQT1 LQT5 179 181 182 185 LQT2 pore mutation 186 LQT1 C 187 Circulation Journal Vol. 69, Suppl. IV, 2005 1215

2003 2004 2 10 30 % SCN5A 146 188 SCN5A steady state inactivation curve intermediate inactivated state recovery kinetics 189 silent carriers 146 SCN5A 23 54 149 SCN5A 190 192 L567Q 3 CPVT FPVT ARVD2 3 RyR2 193 202 FKBP12.6 RyR2 FKBP SR Ca 2 Ca 2 Calsequestrin2 CASQ2 203 204 ARVD2 RyR2 FKBP12.6 194 RyR1 Priori CPVT 12 4 33 % hryr2 195 30 probandsfamily members 118 RyR2 proband 14 36 % family members 9 7.6 % 4 silent carrier 198 9 205 a b 1 30 30 30 QT 1216 Circulation Journal Vol. 69, Suppl. IV, 2005

1 1 2 80 % 10 11 116 118 31 79 % 128 130 206 207 208 9 205 AED 208 211 2 30 % 8 9 8 212 130l213 214 168 215 216 3 20 35 % 100 2 5 % 3 % a b VF SVT 1 2 EF 40 % 80 mmhg SVT SVT SVT EF 0.40 VF SVT EF VT VF Circulation Journal Vol. 69, Suppl. IV, 2005 1217

2003 2004 4 10 20 % 10 116 118 217 115 218 219 5 1 3 1 2 220 221 3 222 223 223 224 6 1.8 225 25 % 226 2 3 227 3 122 125 9 2 10-2 217 228 231 Bezold-Jarish 232 233 Head-up Tilting 2 80 % 15 % 20 % a b SVT VF SVT SVT VF SVT VF 1218 Circulation Journal Vol. 69, Suppl. IV, 2005

1 1 1 2 QRS 2 2 3 4 3 5 2 1 2 3 4 5 Brugada QT 3 NYHA 234 9 22 % 235 239 NYHA - NYHA - 235 239 240 1 LVEF 41 % 8 128 131 241 140 141 241 242 2 243 7294 2 4.7 % 20 % 244 245 231 246 242 MADITII 30 % 1,232 20 14.2 % 19.8 % 247 10 248 52 % 48 % NYHA - 35 % 3 SCD-HEFT 45.5 2 249 3 60 % 2 NYHA 10 % 70 % 240 30 % V-HeFT 250 25 % CHF-STAT 238 20 % GESICA 237 PROMISE 251 VPC 30 131 348 4 239 252 V-HeFT 253 SAVE 254 TRACE 255 ACE NYHA Circulation Journal Vol. 69, Suppl. IV, 2005 1219

2003 2004 ELITE 256 RALES 257 RAAS K 4 1 2.1 4.9 % 258 261 1 62 % 4 91 % 10 98 % 262 8 18.8 % 258 260 261 2.4 10.6 % 38 56 % 258 262 265 1 28 % 4 41 % 12 50 % 11 259 261 264 AED 2 10 40 % 1 4 2.4 48 2 % 265 266 Killip 3 90 n 87 EF 29 12 % 26 41 % 266 b a b SVT VF NSVTEF 40 % SVT VF NSVTEF 35 % SVT VF EF 40 % ACE VF NSVT SVT EF MADIT- 1220 Circulation Journal Vol. 69, Suppl. IV, 2005

a b SVT VF Ca 3 7.7 13.0 % 2.7 3.9 % 267 268 63 % 18.1 % 41.9 % 267 268 269 4 1 10 20 % 6 270 272 30 % 8 8 266 8 205 209 211 215 273 274 275 13 276 277 13 47 % 75 93 % 2 11 % 278 279 280 GISSI-2 6 10 278 278 281 282 10 25 42 % 81 88 % 2 13 % 270 278 283 30 40 % 4 8 18 40 % 278 284 285 MADIT 131 0.40 MADIT- 247 0.30 205 9 237 238 SCD-Heft 249 286 239 252 257 ACE 253 256 287 289 290 292 17 5 293 294 Yasue 254 80.5 10 93 % 12 7 Circulation Journal Vol. 69, Suppl. IV, 2005 1221

2003 2004 5 295 Nishizaki 73 2 25 % 294 5 296 297 T C 298 1 2 % 162 299 302 303 305 300 302 306 162 136 300 306 308 90 % 200 20 % 2 40 % 17 28 % 309 314 310 312 313 315 317 70 % 95 % 310 312 313 20 25 % HCM 1 % 8 9 % 310 314 318 319 HCM 311 320 321 30 mm 171 300 322 324 300 325 326 327 20 mmhg HCM 25 % 50 162 164 328 HCM 136 137 308 310 329 331 HCM 297 332 338 a b SVT VF NSVT SVT VF NSVT SVT VF NSVT 1 SVT VF 2 SVT VF NSVT NSVT5 10 NSVT 30 mm 20 mmhg 1 1222 Circulation Journal Vol. 69, Suppl. IV, 2005

6 7 339 341 14 342 346 343 350 342 343 351 352 353 Q ST-T 353 12 mm 60 mm 353 354 DCM 52 50 % 30 40 % 9 355 356 236 DCM 10 % 8 9 357 DCM 90 % 45 87 % 358 359 359 358 360 361 1.7 30 % 8 DCM a b SVT VF ACE EF 40 % SVT VF EF 40 % SVT VF EF 40 % SVT VF DEFINITE 147 SCD-Heft 148 Circulation Journal Vol. 69, Suppl. IV, 2005 1223

2003 2004 30 % 362 30 % 89 36 % 248 363 DCM 227 LP 364 TWA 90 91 365 367 9 40 % 369 374 141 374 4 8 376 20 34 2 1 3 5 7 49 % 30 % 26 % 26 % 374 3 373 1 8 9 ARVC 368 369 370 40 50 371 35 171 176 372 10 % 8 373 1 5 40 50 374 194 375 377 8 9 377 a b VF SVT LP SVT SVT LP 1224 Circulation Journal Vol. 69, Suppl. IV, 2005

10 Brugada V1 V3 ST 378 0.15 % 0.014 % Na SCN5A 140 188 17 % 6 % 141 149 0.5 4 % 141 149 Brugada 153 0.2 mv coved ST 6 % 141 142 coved saddle back 379 QRS EPS 151 152 380 V1 V3 1 coved ST 0.2 mv ST 380 Na 60 90 % ST 380 ST EPS 60 80 % LP LP EPS 94 381 382 Brugada 2 3 50 80 % 150 152 381 5 % ST 7 % 14 % 147 150 146 149 153 SCN5A 149 LP QT dispersion TWA 94 383 384 385 386 a b VF Na Coved ST Brugada VF VF VF VF VF VF Circulation Journal Vol. 69, Suppl. IV, 2005 1225

2003 2004 11 Romano-Ward Jervell and Lange-Nielsen QT Torsade de pointes TdP 387 390 1 1,000 1 100 391 8,000 LQTS 10 % 390 391 LQTS 8 LQT1 LQT8 LQT1 2 3 3 171 179 3 3 182 186 392 LQT1 LQT2 LQT3 393 LQT1 112 LQT2 72 LQT3 62 40 63 % 46 % 18 % LQT1 LQT2 4 % LQT3 20 % 180 TdP LQT2 QTc 500 ms LQT3 180 394 185 TdP LQT1 LQT2 LQT3 181 395 LQT2 pore TdP 186 LQT1 187 LQTS QT 33 % 178 K TdP 12 WPW 1 2 0.02 0.15 % 3 4 396 398 WPW 9 205 399 a b VF TdP QTc 500 m LQT1 LQT2 QTc 500 ms LQT3 QT 1226 Circulation Journal Vol. 69, Suppl. IV, 2005

a b VF RR 250 ms 270 ms Ia Ic 145 400 145 400 401 7 145 401 WPW 3 8 3 400 Kent 20 40 % 400 Ebstein 145 403 404 WPW 158 398 399 270 msec RR 250 msec 155 158 402 9 205 399 13 CPVT 169 405 407 7 10 QT 350 400 RyR2 193 202 Calsequestrin CASQ2 203 204 8 81 39 42 6 RyR2 43 family a b VF Ca RyR2 CASQ2 NSVT Ca Circulation Journal Vol. 69, Suppl. IV, 2005 1227

2003 2004 member carrier 199 43 28 65 % 1 19 CPVT RyR2 4.2 RyR2 198 RyR2 CPVT 169 Ca 169 RyR2 CASQ2 198 14 Brugada 1 Brugada 409 416 417 418 Brugada Brugada 419 avf Brugada ST Brugada variant 2 Leenhardt 250 msec 420 3 421 4 QT 422 15 1 60 15 20 % 423 70 44 424 Schwartz 425 9 7 Olshausen 426 Holter 61 7 7 6 2 2 TdP 2 a b SVT VF SVT VF 1228 Circulation Journal Vol. 69, Suppl. IV, 2005

a b SVT VF 8 34 % 427 428 0 5 % 429 430 425 Bezold-Jarisch reflex 431 Holter 432 433 434 2 3 QT Dispersion QTD 70 ms 48 49 LP 435 LP 436 437 8 44 % 438 439 440 2 441 2 5 % 442 443 444 445 446 447 50 100 1.8 % 447 448 449 453 453 QT Dispersion QTD 3 St Jude Medical 0.5 2.4 % 454 455 Hall 456 457 0.2 1.0 % 458 459 460 X 461 1 462 463 Circulation Journal Vol. 69, Suppl. IV, 2005 1229

2003 2004 8 4 QT 3 3 WPW 2 2 1 1 462 35 16 6 % 464 Maron 35 175 Drory 162 40 20 32 22 % 465 2 1 SIDS 1 34,442 1 1 QT 466 SIDS24 QTc 435 45 msec 400 20 msec SIDS 393 24 msec SIDS24 12 QTc 440 msec SIDS QTc 440 msec QTc 440 msec SIDS QTc 2 6 467 SIDS 1 2 3 4 466 89 apparent lifethreatening event ALTE QT dispersion SIDS 0.0318 0.020 sec 0.026 0.017 sec 468 469 QTc 440 msec SIDS QT Dispersion 25 93 SIDS 2 SCN5A a b SIDS QTc 440 ms SIDS QTc 440 ms QTc 440 ms a b 1230 Circulation Journal Vol. 69, Suppl. IV, 2005

a b CPT- Translocase LCAD VLCAD L- L- LCHAD L- 190 192 SCN5A SIDS 3 SIDS 470 His 471 4 acylcarnitine SIDS 472 5 SIDS 473 ALTE 474 476 474 475 SIDS 2 1 400 15 % 250 msec 250 msec 30 % 143 400 144 2 5 40 6 15 % 477 Silka 11.2 15 478 8 a b Circulation Journal Vol. 69, Suppl. IV, 2005 1231

2003 2004 a b Mg Mg QT LQT2 LQT3 Torsades de Pointes TdP 6 2 3 3 CPVT 7 10 169 4405 408 15 182 4 LQTS QT 1,200 1 479 1 10 LQT2 LQT3 480 LQTS 481 LQTS 482 487 LQTS prospective study 488 LQT1 LQTS 5 489 493 2.9 % 489 493 494 490 492 3 Commotio Cordis 495 496 Commotio Cordis QRS 1232 Circulation Journal Vol. 69, Suppl. IV, 2005

a b 497 498 KATP 499 495 496 10 % Commotio Cordis 28 % 496 500 509 482 486 510 511 9 205 21 % 25 % 482 483 484 510 508 512 4 5 501 502 503 507 QRS 497 QRS QRS 504 transannular patch 11.7 505 outflow tract patch 506 right ventricular patch 12 1 2 3 QRS 180 ms 4 40 % 507 3 508 QRS 120 msec HCM 173 175 513 518 515 518 302 318 519 523 14 30 174 3 120 bpm 26 6 4.35 318 18 HCM 99 4.8 19 3.75 519 14 30 HCM 174 30 mm 3.5 318 66 HCM 19 6 12 520 QTc Dispersion 20 msec myocardial bridging 519 517 520 3 515 5 23 mg kg day 26 Circulation Journal Vol. 69, Suppl. IV, 2005 1233

2003 2004 a b VT Ca ACE Ca 518 0.8 4 mg kg day 140 9 520 482 486 ACC AHA NASPE 9 205 HCM 520 521 Ca 519 523 2 mg kg 5 23 mg kg day 519 519 515 517 520 6 525 526 527 2003 D 1 E a EbA D 1234 Circulation Journal Vol. 69, Suppl. IV, 2005

126 b a b a Ca ACE tissue plasminogen activator Regression 30 30 1 1 a b Circulation Journal Vol. 69, Suppl. IV, 2005 1235

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33 Davey P. QT interval and mortality from coronary artery disease. Prog Cardiovasc Dis. 2000; 42: 359-384 34 Zabel M., Klingenheben T., Franz MR. et al. Assessment of QT dispersion for prediction of mortality or arrhythmic events after myocardial infarction: re-sults of a prospective, long-term follow-up study. Circulation. 1998; 97: 2543-2550 35 Bodi V., Sanchis J., Navarro A. et al. QT dispersion within the first 6 months after an acute myocardial infarction: relationship with systolic function, left ventricular volumes, infarct related artery status and clinical outcome. Int J Cardiol. 2001; 80: 37-45 36 Kirchhof P., Eckardt L., Arslan O. et al. Prolonged QRS duration increases QT dispersion but does not relate to arrhythmias in survivors of acute myocardial infarction. Pacing Clin Electrophysiol. 2001; 24: 789-795 37 Fiol M., Marrugat J., Bergada J.et al. QT dispersion and ventricular fibrillation in acute myocardial infarction. 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