Table 5.
Patient | Sex | QTc, msa | Initial Symptom | Genotype | Age, y | Activityb | QT+Rxc | β‐Blocker | ICD | Outcome |
---|---|---|---|---|---|---|---|---|---|---|
#1d | Female | 500 | Exertional syncope | Negative | 10 | Walking in a winter storm | No | Nadolol (compliant) | Yes—3 shocks for TdP | Mexiletine added |
#2 | Female | 513 | Syncope | KCNH2 | 17 | Walking | No | Nadolol/labetalol (compliant) | No | ICD implanted |
18 | Auditory stimulation | No | Nadolol/labetalol (compliant) | Yes—1 shock for TdP | BB dose increased | |||||
#3 | Female | 450 | Syncope | KCNQ1 | 23 | Using copy machine | No | BB unknown (noncompliant) | No | ICD implanted |
#4 | Female | 590 | Syncope | KCNH2 | 14 | Giving speech at school | No | Nadolol (compliance unknown) | Yes—TdP self‐terminated | Mexiletine added |
#5 | Female | 585 | Syncope | KCNQ1 | 4 | Shopping | Unknown | Atenolol (noncompliant) | Yes—TdP storme with ICD shock | Change BB |
5 | Using bathroom | Unknown | Nadolol (compliance unknown) | Yes—TdP storm with ICD shock | LCSD | |||||
#6 | Male | 553 | TdP during surgery | KCNH2 | 7 | Running from car to garage | No | Propranolol (compliant) | Yes—TdP storm with ICD shock | Changed BB |
8 | Post ICD revision | No | Atenolol (compliant) | Yes—TdP storm with ICD shock | Changed BB |
ADL indicates activities of daily living; BB, β‐blocker; CE, cardiac events; ICD, implantable cardioverter‐defibrillator; LCSD, left cardiac sympathetic denervation; QTc, corrected QT interval; TdP, torsades des pointes.
Highest pretreatment QTc reported.
Reported activity at the time of cardiac event.
QT+Rx, QT prolonging medications.
Patient #1 is the same as patient #3 in Table 4.
TdP storm defined as 3 or more ICD therapies within a 24‐h period.