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BACKGROUND Lown-Ganong-Levine syndrome, includes a short PR interval, normal QRS complex, and paroxysmal tachycardia. The pathophysiology of this syndrome includes an accessory pathway connecting the atria and the atrioventricular (AV) node (James fiber), or between the atria and the His bundle (Brechenmacher fiber). Similar features are seen in enhanced atrioventricular nodal conduction (EAVNC), with the underlying pathophysiology due to a fast pathway to the AV node, and with the diagnosis requiring specific electrophysiologic criteria. CASE REPORT A 17-year-old man presented with a history of recurrent narrow-complex and wide-complex tachycardia on electrocardiogram (ECG). An electrophysiologic study showed an unusually short atrial to His (AH) conduction interval and a normal His to ventricle (HV) interval, without a delta wave. Two stable AH intervals coexisted in the same atrial pacing cycle length. In the recovery curve study, this pathway had a flat conduction curve without an AH increase until the last 60 ms, before reaching the effective refractory period. These ECG changes did not respond to an adenosine challenge. When this pathway became intermittent, there was a paradoxical response to adenosine challenge with conduction via a short AH interval, but without conduction block. Catheter ablation of the AV nodal region resulted in a normalized AH interval, decremental conduction properties, and resulted in a positive response to an adenosine challenge. CONCLUSIONS In this case of Lown-Ganong-Levine syndrome, electrophysiologic studies supported the role of the accessory pathway of James fibers.

There is insufficient knowledge concerning long-QT (LQT) 3 in the pediatric population to determine whether recommendations for more aggressive therapy in these patients are appropriate. An international multicenter review of 43 children with cardiac sodium channel (SCN5A) mutations and clinical manifestations of LQT syndrome without overlap of other SCN5A syndromes was undertaken to describe the clinical characteristics of LQT3 in children. Patients were aged 7.6 ± 5.9 years at presentation and were followed for 4.7 ± 3.9 years. There was significant intrasubject corrected QT interval (QTc) variability on serial electrocardiography. Forty-two percent presented with severe symptoms or arrhythmia and exhibited longer QTc intervals compared to asymptomatic patients. None of the 14 patients who underwent primary prevention implantable cardioverter-defibrillator (ICD) implantation received appropriate shocks in 41 patient-years of follow-up, while 2 of 6 patients who underwent secondary prevention ICD implantation received appropriate shocks in 30 patient-years of follow-up. Half of patients who underwent ICD implantation experienced inappropriate shocks or ICD-related complications. Mexiletine significantly shortened the QTc interval, and QTc shortening was greater in patients with longer pretreated QTc intervals. Two ICD patients with frequent appropriate ICD shocks showed immediate clinical improvement, with elimination of appropriate ICD shocks after mexiletine loading. In conclusion, severe symptoms are common in children with LQT3 and are associated with longer QTc intervals. ICD implantation is associated with significant morbidity. Mexiletine shortens the QTc interval, and it may be beneficial., (Copyright © 2012 Elsevier Inc. All rights reserved.)

An 11-year-old boy, who underwent bicaval orthotopic heart transplantation for idiopathic dilated cardiomyopathy, had a focal atrial tachycardia originating from the donor superior vena cava. The pathogenesis of this tachycardia may be related to transplant rejection or transplant vasculopathy. Radiofrequency catheter ablation can eliminate this unique tachycardia and result in hemodynamic improvement.

Introduction: Fasciculoventricular (FV) fiber is a rare cause for ventricular preexcitation. It is usually described as an innocent bystander pathway. There is only limited data on FV fiber in children. Hence we evaluated the clinical and electrophysiological features of FV fiber in a group of 11 children., Methods and Results: Of 215 children with manifest preexcitation who had electrophysiological studies at the University of Miami, 11 (5.1%) had characteristics of FV fiber. FV fiber was not directly responsible for any arrhythmias in these children. Three children had supraventricular tachycardia due to associated left sided Kent fiber and FV fiber was identified after the ablation of Kent fiber. One child had associated hypertrophic cardiomyopathy, another child had atrial septal defect and a third child had ventricular septal defect. The electrophysiological testing of FV fiber revealed AH interval of 40-95 ms and H-delta interval of 15-40 ms. Mapping study showed that the FV fiber was located on the right side of the heart in all patients: right anteroseptal in 9, right midseptal in 1 and it could not be mapped well in 1 as the delta wave was intermittent. We identified a discrete FV fiber depolarization spike in 9 patients, with a local FV fiber depolarization to delta wave interval of 5-20 ms (11+4 ms)., Conclusion: FV fiber is an uncommon cause for ventricular preexcitation in children and it can be associated with other conduction abnormalities and heart defects. FV fibers are commonly located at the right anteroseptal region and are not usually involved in any tachycardia. Discrete local FV fiber potential can be identified during electrophysiological testing of these patients.

A centrally inserted venous catheter may cause atrial ectopic tachycardia. The association of atrial ectopic tachycardia with spontaneous reverse alternating Wenckebach periodicity has rarely been reported. We describe a 4-year-old boy with tetralogy of Fallot who developed atrial ectopic tachycardia with reverse alternating Wenckebach periods postoperatively after central venous catheter placement. All such episodes emerged from a 3:2 atrioventricular block, followed by runs of 2:1 atrioventricular block with progressive shortening of the conducted PR intervals. Normal sinus rhythm returned after the catheter was withdrawn to the superior vena cava. Reverse alternating Wenckebach periodicity may be a tachycardia-dependent physiologic phenomenon.

1. (-)-Caryachine, isolated from the plant (Cryptocarya chinensis), increased the contractility of atrial and right ventricular strips and significantly suppressed the reperfusion arrhythmias in adult rabbit heart (ED50 = 1.27 microM). 2. Data obtained by the whole-cell voltage clamp technique has shown that (-)-caryachine causes a negative shift of the steady-state Na channel inactivation and a slower rate of recovery from inactivation. The maximal Na current amplitude decreased to 67 +/- 7%, 29 +/- 8% and 12 +/- 5% after 0.5, 1.5 and 4.5 microM (-)-caryachine, respectively. 3. This agent also had effects on the time- and voltage-dependent K currents. (-)-Caryachine markedly suppressed the 4-AP-sensitive transient outward current (I10). However, it produced very little voltage-dependent shift in inactivation. After 0.5, 1.5 and 4.5 microM of the compound, the respective value of I10 elicited at +60 mV was 80 +/- 7%, 45 +/- 8% and 15 +/- 3%. At higher concentrations, the inward rectifier K current (IK1) was also inhibited but to a much smaller extent. Its slope conductance after 0.5, 1.5 and 4.5 microM (-)-caryachine was reduced to 71 +/- 9%, 51 +/- 12% and 42 +/- 11%, respectively. The outward hump of inward rectification was not changed. 4. In contrast, the L-type Ca current was not significantly changed by (-)-caryachine. 5. Electrophysiological studies in perfused whole heart preparations revealed that (-)-caryachine increased the intra-atrial conduction interval and also prolonged the atrial refractory period. No proarrhythmic effects were induced during the infusion of this compound (up to 13.5 microM). 6. We conclude that (-)-caryachine predominantly blocks the Na and I10 currents. These changes alter the electrophysiological properties of the heart and terminate the induced ventricular arrhythmias. The relatively selective I10 inhibition, safety margin of Ik1 suppression and lack of effect on Ica-L will provide an opportunity to develop an effective antiarrhythmic agent with positive inotropy as well as low proarrhythmic potential.

To determine the utility of preoperative screening electrocardiograms (ECGs) among ambulatory surgery patients, we reviewed the charts of 751 consecutive adult patients who underwent ambulatory surgery. Data were collected on demographic characteristics, coexisting medical problems, American Society of Anesthesiologists physical status score, preoperative ECG results, adverse intraoperative cardiovascular events, and postoperative cardiovascular complications. In our study population of relatively healthy outpatients, preoperative ECGs were abnormal in 42.7% of patients. Age, increased physical status score, and male gender were associated with a greater incidence of abnormal preoperative ECGs. There were 12 adverse cardiovascular perioperative events among the 751 patients (1.6%), and the preoperative ECG may have been clinically useful in six of these 12 patients. Neither preoperative ECGs nor results of preoperative screening questionnaires were predictive of adverse cardiovascular perioperative events. These findings question the utility of preoperative ECGs in the ambulatory surgery setting, especially among younger, relatively healthy patients.