Background-A rapidly growing number of long-QT syndrome (LQTS) patients are being treated with an implantable cardioverter-defibrillator (ICD). ICDs may pose problems, especially in the young. We sought to determine the characteristics of the LQTS patients receiving an ICD, the indications, and the aftermath. Methods and Results-The study population included 233 patients. Beginning in 2002, data were collected prospectively.Female patients (77%) and LQT3 patients (22% of genotype positive) were overrepresented; mean QTc was 516Ϯ65 milliseconds; mean age at implantation was 30Ϯ17 years; and genotype was known in 59% of patients. Unexpectedly, 9% of patients were asymptomatic before implantation. Asymptomatic patients, almost absent among LQT1 and LQT2 patients, represented 45% of LQT3 patients. Patients with cardiac symptoms made up 91% of all study participants, but only 44% had cardiac arrest before ICD implantation. In addition, 41% of patients received an ICD without having first been on LQTS therapy. During follow-up, 4.6Ϯ3.2 years, at least 1 appropriate shock was received by 28% of patients, and adverse events occurred in 25%. Appropriate ICD therapies were predicted by age Ͻ20 years at implantation, a QTc Ͼ500 milliseconds, prior cardiac arrest, and cardiac events despite therapy; within 7 years, appropriate shocks occurred in no patients with none of these factors and in 70% of those with all factors. Conclusions-Reflecting previous concepts, ICDs were implanted in some LQTS patients whose high risk now appears questionable. Refined criteria for implantation, reassessment of pros and cons, ICD reprogramming, and consideration for other existing therapeutic options are necessary. (Circulation. 2010;122:1272-1282.)

Moderate increases in air pollution appear to be associated with ventricular arrhythmias in ICD patients already after 2 h, although future studies including larger numbers of events are required to confirm these findings. Representative geographical exposure classification seems important in studies of these effects.

Among survivors of OHCA, a substantial proportion was found in a non-shockable rhythm and this occurred more frequently in women than in men. The proportion of survivors found in a shockable rhythm has not changed markedly over time. Survivors found in a shockable rhythm had a better cerebral performance than survivors found in a non-shockable rhythm. The proportion of survivors who were bystander-witnessed and found in a shockable rhythm and defibrillated early is still remarkably low.

Noise reversion and undersensing might prevent the ICD from delivering shock when it should and the interpretation as VT/VF could result in inappropriate shocks. Because of the potentially serious consequences of interference we do not recommend the use of TENS in patients with ICD.