Rodzinny napadowy częstoskurcz węzłowy — obserwacja wielopokoleniowej rodziny

We report a three-generation family coming from southeastern region of Poland (Podkarpackie voivodship) with 6 women having normal hearts and presenting with a history of paroxysmal tachycardia with onset of symptoms in the adulthood. Recordings of clinical SVT, dual AVN electrophysiology, induction of typical AVNRT and results of RFCA are available. The history of this family shows the significance of a careful and detailed collection of medical history, and point towards the importance of family screening in AVNRT patients

Zmienność odstępu QTc. Czy jeden pomiar QTc wystarczy do podjęcia decyzji terapeutycznych i stratyfikacji ryzyka u chorych z zespołem długiego QT?

We described a case of 30 year-old woman with episodes of syncope primarily diagnosed as epilepsy, and finally recognisedas long QT syndrome. Based on QTc prolongation > 600 ms in series of electrocardiograms and Holter monitoring the patientwas implanted with cardioverter-defibrillator (ICD). During follow-up many appropriate ICD shocks due to ventricularfibrillation occurred

Congenital long QT syndrome of particularly malignant course connected with so far unknown mutation in the sodium channel SCN5A gene

This article presents the case of a 35 year-old male with long QT syndrome (LQTS) who suffered from sudden cardiac arrest. Even though asymptomatic LQTS had been diagnosed, the patient had not undergone any medical treatment. His two daughters, aged four and seven, were also diagnosed with LQTS. A new, previously unknown, mutation of the SCN5A gene has been found in the family. The older daughter died suddenly before implantable cardioverterdefibrillator (ICD) implantation, but the father and the younger daughter have been implanted with ICDs

Implantable cardioverter-defibrillators in patients with long QT syndrome: a multicentre study

Background: Implantable cardioverter-defibrillator (ICD) therapy has been proven effective in the prevention of sudden cardiac death, but data on outcomes of ICD therapy in the young and otherwise healthy patients with long QT syndrome (LQTS) are limited. Aim: We sought to collect data on appropriate and inappropriate ICD discharges, risk factors, and ICD-related complications. Methods: All LQTS patients implanted with an ICD in 14 centres were investigated. Demographic, clinical, and ICD therapy data were collected. Results: The study included 67 patients (88% female). Median age at ICD implantation was 31 years (12–77 years). ICD indication was based on resuscitated cardiac arrest in 46 patients, syncope in 18 patients, and malignant family history in three patients. During a median follow-up of 48 months, 39 (58%) patients received one or more ICD therapies. Time to first appropriate discharge was up to 55 months. Inappropriate therapies were triggered by fast sinus rhythm, atrial fibrillation, and T-wave oversensing. No predictors of inappropriate shocks were identified. Risk factors for appropriate ICD therapy were: (1) recurrent syncope despite b-blocker treatment before ICD implantation, (2) pacemaker therapy before ICD implantation, (3) single-chamber ICD, and (4) noncompliance to b-blockers. In 38 (57%) patients, at least one complication occurred. Conclusions: ICD therapy is effective in nearly half the patient population; however, the rates of early and late complica­tions are high. Although the number of unnecessary ICD shocks and reimplantation procedures may be lowered by modern programming and increased longevity of newer ICD generators, other adverse events are less likely to be reduced

Pediatric cardiology emergencies – the role of Long QT Syndrome among arrhythmias

Long QT syndrome (LQTS) is a hereditary disease with significant mortality, which might be reduced with appropriate management. This cardiac disorder is regarded as rare, but its prevalence remains unknown. The clinical course of LQTS is variable and syncope is a common first manifestation of LQTS. Therefore in each patient after syncope an ECG should be carried out. However, there is no universal QT value applying to all patients (especially infants and children), because it varies depending on age and sex. Genetic testing can be of great importance for the management of families with LQTS and early identification of patient relatives at risk of developing disease. We aimed to show that a very important part of treatment is not only pharmacotherapy, especially beta-blockers, but change of lifestyle plays a significant role