Introduction-One form of the hereditary long QT-syndrome, LQT3-ΔKPQ, is associated with sustained inward sodium current during membrane depolarization. Ranolazine reduces late sodium channel current, and we hypothesized that ranolazine would have beneficial effects on electrical and mechanical cardiac function in LQT3 patients with the SCN5A-ΔKPQ mutation.
Venous complications of pacemaker/ implantable cardioverter defibrillator (ICD) system implantation rarely cause immediate clinical problems. The challenge starts when patients come for system revision or upgrade. Numerous reports of venous complications such as stenosis, occlusions, and superior vena cava syndrome have been published. We reviewed current knowledge of these complications, management, and their impact on upgrade/revision procedures. One study has suggested that intravenous lead infection promotes local vein stenosis. Another found that the presence of a temporary wire before implantation is associated with an increased risk of stenosis. Although data for ICD leads is based only on three studies-it suggests that the rate of venous complications is very similar to that of pacing systems, and probably data from pacing leads can be extrapolated to ICD leads. Despite 40 years of experience with transcutaneous implanted intravenous pacing systems and dozens of studies, we were unable to identify clear risk factors (confirmed by independent studies) that lead to venous stenosis. Neither the hardware (lead size, number and material) nor the access site choice (cephalic cut down, subclavian or axillary puncture) appears to affect rate of venous complications. A few factors were proposed as predictors of severe venous stenosis/occlusion: presence of multiple pacemaker leads (compared to a single lead), use of hormone therapy, personal history of venous thrombosis, the presence of temporary wire before implantation, previous presence of a pacemaker (ICD as an upgrade) and the use of dual-coil leads. Anticoagulant therapy (for other reasons than pacemaker lead) seemed to have protective antithrombotic effect.
Chronic low-dose flecainide significantly shortens the QTc interval in LQT-3 subjects with the DeltaKPQ mutation. No major adverse drug effects were observed with flecainide during this trial, but the sample size is not large enough to evaluate the safety of flecainide therapy in patients with this mutation.
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