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Introduction: Long QT syndrome (LQTS) is a congenital ion channel disorder causing prolonged ventricular repolarization and presents on surface ECG with a prolonged QTc interval. This condition predisposes to ventricular arrhythmias and also sudden cardiac death. LQTS without appropriate therapy during pregnancy and the postnatal phase poses an additionally increased risk of sudden cardiac death due to physiological changes associated with gestation. We present a case report of a 30-year-old pregnant woman with known long QT syndrome Type 2 (LQT2) and discuss the management in cardiological practice., Competing Interests: Es bestehen keine Interessenskonflikte., (© 2024 Aerzteverlag medinfo AG.)

CME ECC 66: Torsade de Pointes: The Danger of a Rotating Heart Axis Abstract. Torsade de pointes tachycardia is a potentially life-threatening heart rhythm disorder, caused by prolongation of the QT-interval resulting in triggered activity. This QT-prolongation can be congenital or acquired. If acquired, it is mainly caused by pharmacological therapy. The hallmark of torsade de pointes is an undulating QRS axis with a twist of the QRS complex around the ECG's baseline. Often, this polymorphic ventricular tachycardia is self-limiting, but degeneration into ventricular fibrillation is possible, which makes torsade de pointes tachycardia dangerous. This article aims to provide insights into etiology, diagnostics, prevention and management of this heart rhythm disorder.

Aim: The aim of this study was to investigate the usefulness in providing diagnostic information about syncope by implantation of a loop recorder (ILR)., Methods and Results: The study population consisted of 48 consecutive patients (23 male, 25 female, mean age 42 +/- 17) with unexplained syncope who presented between 1998 and 2002 and underwent extensive cardiological screening and were followed with an implantable loop recorder (Reveal or Reveal Plus). The mean follow-up duration was 9 +/- 6 months. During this follow-up in 17 (35%) patients syncope recurred. Arrhythmia correlating with syncope was documented in 15 (88%) of these patients, in 2 (12%) patients an arrhythmia could be excluded. Of these 15 patients with arrhythmogenic cause of syncope 5 (33%) patients revealed higher degree AV-Block, 7 (47%) patients sinus bradycardia or sinus pauses, 4 (27%) due to sick sinus syndrome and 3 (20%) due to neurally mediated syncope, 3 (20%) patients had atrial tachycardias or atrial fibrillation with rapid AV-conduction. As a result of ILR findings 12 pacemakers were implanted and 2 radiofrequency ablations were performed., Conclusion: The ILR is a valuable and effective tool to establish an arrhythmic cause for unexplained syncope. In these cases they have an impact on subsequent clinical decision making. ILR can also be useful in ruling out arrhythmias as cause of syncope and presyncope.

Atrial fibrillation (AF) is the most common sustained arrhythmia and increases exponentially with age. The physiologic basis are certain triggers initiating multiple micro-reentry circuits, which require a certain amount of "myocardial mass" to be sustained. There are numerous predisposing factors for AF, mostly leading to dilatation or hypertrophy of the atrial myocardium. Lone AF, however, occurs in structurally normal hearts. In the management of AF it is mandatory to decide between medical or electrical cardioversion in persistent AF and rate control in permanent AF. Medical cardioversion or prophylaxis of recurrence can be performed with Class IA, IC or Class III antiarrhythmic drugs. The choice of drugs depends on the underlying cardiac pathology of the individual patient. Patients with long duration of poor rate control during AF are at risk for tachycardia-induced cardiomyopathy. Cardioversion is safe to be performed within 48 hours after the onset of AF without prior and--if there is no risk of recurrence--without consecutive anticoagulation. When AF persists longer than 48 hours, anticoagulation for three weeks is mandatory prior to attempted cardioversion, or alternatively, transesophageal echocardiography can be performed to exclude the presence of an intraatrial thrombus. Anticoagulation has to be maintained for a minimum of four weeks after the restoration of sinus rhythm. Anticoagulation is required for paroxysmal, persistent and permanent AF. Lone atrial fibrillation in patients under the age of 60 years is an exception to these rules and does not require anticoagulation. In case of refractory AF with poor rate control, catheter ablation of the AV node with pacemaker implantation is the treatment of last choice. Early attempts to provide a cure for AF included the surgical "Maze" procedure, followed by linear catheter ablation with the goal of reducing the atrial mass. Catheter ablation of the triggers of AF, which mainly originate at the pulmonary veins and the "substrate modification" have been introduced in the last couple of years and is performed increasingly in specialized EP centers.

Atrial fibrillation is the most frequent arrhythmia with a prevalence of 1.7% in the general population and 5% in the population over 65 year old [1], the prevalence in men over 65 years is even 9.1% [2]. Atrial fibrillation is divided dependent on its frequency in intermittent, persisting and chronic. The symptoms and the clinical course are individually very different. Responsible for the initiation of atrial fibrillation are triggers, which initiate atrial fibrillation, and a substrate, that means an atrial myocardium altered by remodelling, which maintains atrial fibrillation. A curative treatment was not available until recently. Different ablation techniques were developed: Linear, focal ablation techniques and the isolation of pulmonary veins. The observation that atrial fibrillation will maintain only in large atria, led to the linear ablation techniques with the goal of reducing the atria functionally. The linear ablations have however a small value because of low success rates and high complication rates. Arrhythmogenic foci are of crucial importance in the initiation of atrial fibrillation, these foci are mostly located in the pulmonary veins. This observation led to the focal ablation in the pulmonary veins, which was left again because of the limitations, however. For some years the isolation of pulmonary veins is performed at some large centers. This paper summarizes the most important techniques, success rates and complications of the pulmonary vein isolation. The present indication and the preinterventional diagnostics are discussed. Due to the constantly improving success rates and decreasing complication rates (pulmonary vein stenosis, thrombembolie, cardiac tamponades, mortality practically) the pulmonary vein isolation is an alternative possibility of treatment which can be considered for a subgroup of patients with drug-resistant, symptomatic atrial fibrillation.

In patients with ventricular tachycardias, various structural and functional conditions can be influenced by certain triggers, initiating the mechanism of the arrhythmia. Thorough understanding of these different factors is paramount for the appropriate choice of treatment. Several options are available for the management of ventricular tachycardias: antiarrhythmic drugs, ICD implantation and catheter ablation or a combination of the above. The prognosis of an individual patient is determined by the underlying disease, the mechanism of tachycardia and the selected therapy. The complexity of several conditions requires a specialized diagnostic approach and tailored medical management. Therefore the patients with the following diagnoses should be referred to a specialized electrophysiology center: survivors of sudden cardiac death old infarct scar, reduced ejection fraction and complex ectopy Cardiomyopathy with syncope or ventricular tachycardias Long QT-Syndrome Brugada Syndrome Right Ventricular Dysplasia recurrent syncopes of unclear etiology.