Table 1.
Clinical characteristics
| Single mutation | Multiple mutations | P-value | |
|---|---|---|---|
| N = | 346 | 57 | |
| Male, n (%) | 144 (41.6%) | 23 (40.4%) | 0.857 |
| QTc, msec | 480 ± 56 | 506 ± 72 | 0.003 |
| QTc > 500 msec, n (%) | 94 (27.2%) | 22 (38.6%) | 0.077 |
| QT, msec | 436 ± 86 | 446 ± 92 | 0.431 |
| RR, msec | 838 ± 238 | 787 ± 206 | 0.143 |
| Proband, n (%) | 125 (36.1%) | 20 (35.1%) | 0.880 |
| Age at 1st Syncope, years | 22.5 ± 13.9 | 19.5 ± 14.9 | 0.129 |
| Age at 1st ACA/LQTS death, years | 28.3 ± 13.1 | 27.3 ± 12.5 | 0.593 |
| Syncope, n (%) | 140 (40.5%) | 28 (49.1%) | 0.219 |
| ICD shock, n (%) | 10 (2.9%) | 5 (8.8%) | 0.030 |
| ACA, n (%) | 18 (5.2%) | 3 (5.3%) | 0.985 |
| LQTS death, n (%) | 1 (0.3%) | 2 (3.5%) | – |
| ACA, ICD shock or LQTS death, n (%) | 26 (7.5%) | 9 (15.8%) | 0.040 |
| Beta Blockers used, n (%) | 205 (59.3%) | 42 (73.7%) | 0.038 |
| ICD, n (%) | 64 (18.5%) | 9 (15.8%) | 0.623 |
| Pacemaker, n (%) | 21 (6.1%) | 3 (5.3%) | 0.812 |
| LCSD, n (%) | 4 (1.2%) | 0 (0.0%) | – |
Data are presented as number of patients (%) or mean ± SD
ACA = aborted cardiac arrest, ICD = implantable cardioverter defibrillator, JLNS = Jervell Lange-Nielsen Syndrome, LCSD = left cervical sympathetic denervation.