Wide QRS complex supraventricular tachycardia with negative precordial concordance: Electrocardiographic clues for Mahaim pathway with Ebstein anomaly.

Ebstein's anomaly is often accompanied by either Wolff-Parkinson-White syndrome or atriofascicular Mahaim. These bypass tracts give rise to antidromic atrioventricular (AV) re-entrant tachycardias, in which the bypass tract serves as the anterograde limb of the circuit and the AV node as the retrograde limb of the reentrant circuit. Since the antidromic AV reentrant tachycardia over a Mahaim fibre has a typically left bundle braunch block (LBBB) morphology, it is easy to make a misdiagnosis of supraventricular tachycardia with functional LBBB or even of ventricular tachycardia particularly in the presence of negative concordance. Some electrocardiographic clues might prevent misdiagnosis of ventricular tachycardia and inadvertent ICD implantation.

[Wide spectrum of arrhythmias, with inductions and terminations of atrio-ventricular replica-nodal tachycardia by automaticity during successful radiofrequency ablation of long anterogradely slow conducting atrio-fasciculo-ventricular accessory pathway]. / Kanonada arytmii, w tym czestoskurcz nawrotnyz udzialem repliki wezla p−k w czasieskutecznej ablacji dlugiego szlakuprzedsionkowo−peczkowo−komorowego.

Mahaim syndrome is still a challenge. The specific characteristics of the pathway, the fact that it seldom occurs, and ambiguities about the pathophysiology and nomenclature make it, for electrophysiologists, still difficult to ablate. We present a case of a patient with anterogradely slow conducting accessory pathway, which has been effectively ablated although M potential was not observed nor was a mechanical conducting block of the accessory pathway. The accessory pathway showed a wide spectrum of electrophysiological characteristics of the "AV node physiology" including inductions and terminations of atrio-ventricular replica-nodal tachycardia by local automaticity, not only during electrophysiological study, but during successful application. The accessory pathway presented signs of two types of pseudo Mahaim fibers: atrio-fascicular and slow conducting atrio-ventricular connection.

Postablation-acquired short atrioventricular Mahaim-type fibers: observations on their clinical, electrocardiographic, and electrophysiologic profile.

BACKGROUND: The electrophysiologic characteristics of decrementally conducting accessory pathways (APs) are well described; however, little is known about decrementally conducting APs caused by the radiofrequency ablation of a rapidly conducting AP. OBJECTIVE: To report the clinical, electrocardiographic, and electrophysiologic characteristics of 6 patients who developed a decremental AP after an attempt at ablation. METHODS: We compared the clinical and electrophysiologic characteristics of 295 consecutive patients with the Wolff-Parkinson-White syndrome who underwent radiofrequency ablation of 311 manifest APs (group A) with those of 6 patients with the Wolff-Parkinson-White syndrome in whom a decrementally conducting AP was detected after an attempt at ablation. RESULTS: The AP ablation site in group B patients was at the coronary sinus ostium region in 3 patients, middle cardiac vein in 2 patients, and left posteroseptal region in 1 patient. Sixty-two bypass tracts in group A patients and all 6 in group B patients were ablated at these locations, while 249 bypass tracts in group A patients and none in group B patients were ablated elsewhere (P = .0001). Five of the 6 patients (83%) with acquired Mahaim physiology had an AP located in the venous system. The odds for developing an acquired decremental antegrade atrioventricular AP when it was located inside the venous system were 1 in 6. All group B decremental APs were sensitive to adenosine, but none in 85 group A patients (P <.0001). CONCLUSIONS: The risk for developing decremental conduction after the ablation of a rapidly conducting AP is greater for APs inside the coronary venous system. Acquired decremental antegrade atrioventricular APs are electrophysiologically similar to de novo ones. They are capable of being part of an arrhythmia circuit and, therefore, should be targeted for ablation.

Nodoventricular pathway associated with twin AV nodes: complexity of ablation in single ventricle physiology.

We report the case of a patient with heterotaxy syndrome including complex single ventricular morphology and interrupted IVC in association with twin conduction systems and a nodoventricular accessory pathway connection. The presence of 3 distinct QRS morphologies was inadvertently discovered during a hemodynamic catheterization study and prompted formal EP testing prior to hepatic venous inclusion into the Fontan circuit and loss of access to the atrial chamber for testing and therapy. This patient underscores the importance of close surveillance and high index of suspicion of arrhythmia mechanisms in patients with heterotaxy syndrome in conjunction with single ventricle morphology.

Wide-QRS tachycardia inducible by both atrial and ventricular pacing.

We describe an interesting case of an atriofascicular re-entrant tachycardia due to a Mahaim pathway. The differential diagnosis is discussed and a review of the relevant literature is presented.

Clinical and electrophysiological characteristics of fasciculoventricular fibers in children.

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.

Regular narrow QRS complex tachycardias in the Manitoba Follow-up Study (1948-88).

OBJECTIVE: To determine the incidence, risk factors and prognosis of regular narrow QRS complex tachycardia (NQT), which develops in the absence of pre-excitation in subjects free from ischemic heart disease in the Manitoba Follow-up Study. DESIGN AND SETTING: The Manitoba Follow-up Study is a longitudinal cardiovascular study of 3983 initially healthy men (primarily living in Canada) followed prospectively for 40 years. Risk factors and prognosis were assessed in a nested case-control study. MAIN RESULTS: Twenty-two individuals were diagnosed with NQT before clinical and/or electrocardiographic manifestation of ischemic heart disease (145,408 person-years of observation). Between the ages of 30 and 80, the incidence of NQT was one per 6000 person-years and increased with age. History of childhood diseases, valvular disease, smoking, elevated blood pressure and body mass index did not increase the likelihood for NQT development. NQT was diagnosed concurrently with a serious noncardiac condition in seven cases; excess mortality resulted as six of these subjects died within one year of NQT diagnosis while only two subjects without concurrent disease at NQT diagnosis died during follow-up. In comparison with 2% of control subjects, 27% of subjects with NQT subsequently developed electrocardiographical evidence of atrial fibrillation (relative risk was 12 with lower 95% confidence limit of 1.8). CONCLUSIONS: NQT in an otherwise healthy individual is a benign condition and increases the likelihood of atrial fibrillation development.