Table 5.
Patient | Sex | Event | Age With Event (y) | E1784K | H558R | PR | QRS | QTc | Ajmaline Test | ICD Implantation | CI (ms) | CI/Basic CL (%) |
---|---|---|---|---|---|---|---|---|---|---|---|---|
III,16 | Male | SCA, VF | 60.8 | + | + | 248 | 132 | 487 | Positive | Refused | N/A | N/A |
III,18 | Male | SCA, VF | 47.1 | + | + | 256 | 136 | 468 | Positive | Yes | 663 | 86.1% |
515 | 89.4% | |||||||||||
III,20 | Female | SCA, VF | 50 | + | + | 238 | 105 | 456 | Positive | Yes | 460 | 51.8% |
III,21 (proband) | Male | SCA, VF | 47.5 | + | + | 205 | 108 | 465 | Positive | Yes | 290 | 40.3% |
III,22 | Female | SCA, VF | 44.5 | + | + | 218 | 107 | 469 | Positive | Yes | 410 | 52.8% |
390 | 51.7% |
CI indicates coupling interval of the first beat of polymorphic VT; CL, cycle length; ICD, implantable cardioverter‐defibrillator; N/A, not available; SCA, sudden cardiac arrest; VF, ventricular fibrillation.