Radiofrequency catheter ablation of accessory pathways in patients with Ebstein's anomaly: At 8 years of follow-up.

BACKGROUND: Data regarding long-term follow-up of radiofrequency catheter ablation (RFCA) of accessory pathways (APs) in patients with Ebstein's anomaly (EA) are limited. The procedures are challenging due to multiple or wide APs. METHODS: Analysis was performed on clinical and periprocedural data of patients with EA referred to the centre in order to perform catheter ablation of AP. The group consisted of 22 patients (female 40.9%, mean age 33.6 ± 19.1 years). The follow-up utilized electrocardiogram and Holter monitoring. RESULTS: Twenty-two patients had 33 accessory pathways (8 patients had multiple APs, 11 patients broad AP). Twenty-nine different arrhythmias were ablated: 20 orthodromic atrioventricular reciprocating tachycardia (O-AVRT), 5 antidromic atrioventricular reciprocating tachycardia (A-AVRT), 3 slow/ fast atrioventricular nodal reentry tachycardia (s/f AVNRT) and 1 cavotricuspid-isthmus-dependent atrial flutter (CTI-AFL). In 3 (13.6%) patients multiple ablation targets for RFCA ablation were observed. The acute procedural success rate after the first RFCA performed was: 100% for AVNRT, 77.3% for APs and 50.0% for CTI-AFL ablation. Follow-up (mean 95.7 ± 49.8 months) was completed in 86.4% of patients. One patient had paroxysmal atrial fibrillation not targeted during ablation. One patient died due to heart failure 12 years after RFCA. Three patients who underwent RFCA of accessory pathways in the mid-1990s were lost in follow-up. CONCLUSIONS: Radiofrequency ablation in patients with EA is challenging but safe and have a high short-term as well as long-term success rate.

Features of accessory pathways in adult Ebstein's anomaly.

AIMS: The aim of the study was to describe the complex electrophysiological features of accessory pathways (APs) in adult Ebstein's anomaly (EA). METHODS AND RESULTS: We performed a retrospective study of 17 consecutive adult EA cases with APs who underwent electrophysiological study and radiofrequency catheter ablation (RFCA) from November 2011 to May 2013. There were a total of 24 atrioventricular reentrant tachycardias (AVRTs) due to 23 APs, including 20 (87.0%) non-decremental conducting, 2 (8.7%) decremental conducting, and 1 (4.3%) nodofascicular bundle. Six (6/17 = 35.3%) patients had two APs while others had only one. Twenty-one APs (91.3%) in 15 patients were manifested and 2 APs (8.7%) in 2 patients were concealed. Six APs (26.1%) were broad, while 17 APs (73.9%) were narrow in width. Two patients suffered from duodromic tachycardias mediated by two APs. Accessory pathways were mainly located on the posterior, posteroseptal, and posterolateral tricuspid annulus (TA). Right ventriculography confirmed that all APs were located on the anatomic TA. All the patients remained free from tachycardias during 11.9 ± 6.8 months of follow-up after RFCA. For the 15 patients with manifest APs, 10 patients' electrocardiograms (ECGs) after RFCA demonstrated morphologies of right bundle branch block, while 5 patients' ECGs were normal. CONCLUSIONS: Accessory pathways in EA are predominantly right-sided, manifest and localize to the lower half of the anatomic TA. A number of APs in EA have broad widths. The incidence of multiple APs is high in these patients and RFCA is effective.

Electroanatomic localization of a slowly conducting atrioventricular (Mahaim) accessory pathway.

We present a case of a 34-year-old female with Ebstein's anomaly and symptomatic wide complex tachyarrhythmia. Electrophysiologic evaluation demonstrated antidromic tachycardia utilizing a right-sided decremental, slowly conducting atrioventricular pathway. Distinct accessory pathway potentials along the length of the bypass tract were sequentially recorded to define the anatomic course of the pathway, as uniquely represented on a three-dimensional electroanatomic map, and to successfully guide catheter ablation.

[Atrioventricular nodal and atrioventricular reentrant tachycardia in a patient with Ebsteinanomaly--the role of percutaneous RF ablation, surgery and Amplatzer septal occluder]. / Dwie postacie czestoskurczu nawrotnego u chorej z zespolem Ebsteina przyczyna ekwiwalentów zespolu MAS.Rola przezskórnej ablacji, operacji i zapinki Amplatza.

We present a case of a twelve-year-old girl with Ebstein's anomaly, atrial septal defect and two forms of tachycardia. From the first year the patient suffered from episodes of tachycardia. A few months prior to admission, episodes of palpitations became more frequent, periodically incessant and difficult to interrupt, despite amiodarone treatment. During the tachycardia the patient deteriorated with severe cyanosis, hypotension and often with syncope. During sinus rhythm ECG showed RA enlargement and preexcitation. During the documented tachycardia with rate 160-170/min, the ECG showed right bundle branch block (QRS=160 ms) with right axis deviation. An echocardiogram demonstrated dislodgment of septal tricuspid leaflet 30 mm with a high degree of right ventricle atrialisation, moderate tricuspid insufficiency, and ASD with bidirectional shunt. During the EP study two forms of tachycardia were induced: orthodromic atrioventricular reentrant tachycardia, and atrioventricular nodal reentry tachycardia. Both tachycardias were successfully ablated. Four months later the patient underwent TV replacement with aortic homograft, Glenn anastomosis and ASD closure with fenestration. During the next 18 months, the patient was in better general condition, but still was cyanotic especially during exercise (SAT 76%). An Amplatzer occluder was implanted on the 'residual' atrial septal defect. During the 24-month follow-up period, the patients has been in a good general condition, showing good exercise tolerance with no cyanosis or arrhythmia.

Supraventricular tachyarrhythmias in Ebstein anomaly: management and outcome.

OBJECTIVE: This study was undertaken to review the role of electrophysiology testing and to determine the early and late results of medical and surgical management of supraventricular tachyarrhythmias in Ebstein anomaly. METHODS: We reviewed 130 patients between 1990 and 2001 with Ebstein anomaly and history of tachyarrhythmia with a median age of 25 years (mean age 27.5 years); 106 underwent electrophysiologic testing and 24 had documented atrial flutter or fibrillation. We excluded 21 patients: negative results of testing (n = 18), ventricular tachycardia (n = 2), and junctional tachycardia (n = 1). The remaining 109 patients had more than one mechanism: accessory pathway-mediated tachycardia (n = 49), atrioventricular nodal reentrant tachycardia (n = 10), and atrial flutter/fibrillation (n = 70). RESULTS: Eighty-three patients underwent at least one arrhythmia procedure combined with surgery for Ebstein anomaly. Early mortality was 4.8%. Forty-one patients underwent surgical ablation of an accessory pathway without recurrent accessory pathway-mediated tachycardia at a mean follow-up of 48 months. Seven patients underwent surgical perinodal cryoablation for atrioventricular nodal reentrant tachycardia without recurrence at a mean follow-up of 57 months. Forty-eight patients underwent surgical intervention for atrial flutter or fibrillation (right-sided maze procedure, n = 38, and cryoablation of the atrial isthmus, n = 10). Freedom from recurrent atrial flutter or fibrillation was 75% at a mean follow-up of 34 months. CONCLUSION: Concomitant arrhythmia procedures can be performed without increase in early mortality and should be added to Ebstein repairs for all patients who have supraventricular tachyarrhythmias. Surgical procedures for accessory pathway-mediated tachycardia and atrioventricular nodal reentrant tachycardia give excellent (100%) freedom from recurrence of those arrhythmias. Surgical intervention for atrial flutter/fibrillation yields freedom from late recurrence in 75% of cases.

Ebstein's anomaly, atrial paralysis and atrio-ventricular block: an uncommon association.

We report here the case of a 22-year-old female patient with an incomplete Ebstein's anomaly, complete heart block and atrial standstill. Atrial paralysis associated with Ebstein's anomaly is the most important feature, since there is a report of familial Ebstein's anomaly associated with atrial standstill but isolated cases have not been described. The patient presented with atypical chest pain and a symptomatic bradycardia of 37 beats per minute. A VVIR pacemaker was implanted. She has subsequently been symptom free.

Successful radiofrequency current catheter ablation of accessory atrioventricular pathway in Ebstein's anomaly using electroanatomic mapping.

Ebstein's anomaly is highly associated with atrioventricular reciprocating tachycardia (AVRT) due to an atrioventricular accessory pathway (AP). This case report describes a case of a 30-year-old man with Ebstein's anomaly who had been suffering from recurrent palpitation caused by AVRT due to the right-sided AP. Conventional mapping technique failed to ablate his AP successfully. In baseline electrophysiological study, orthodromic AVRT with a right posterior AP were induced. The AP was mapped using an electroanatomic mapping system. RF current was successfully delivered at the posterior site. RFCA using an electroanatomic mapping system seems to be useful for A VRT patients in Ebstein's anomaly.

Invasive electrophysiology in children: state of the art.

While noninvasive techniques, including esophageal recording and pacing, clearly have advantages in the pediatric population; they also have significant limitations. Invasive electrophysiology (EP) in children now encompasses the use of many advanced engineering applications, which contribute to the efficacy of such procedures. This is particularly true with respect to the performance of radiofrequency catheter ablation. First, microcatheters (eg, Cardima) offer advantages as diagnostic catheters in small hearts, and allow pacing and recording from both atrium and ventricle in even the smallest infants using limited venous access. In addition, there is the possibility of mapping the distal coronary sinus, and the right atrioventricular groove via the right coronary artery. Second, electroanatomic mapping using the CARTO system (Biosense Webster) allows complex maps to be constructed in patients with congenital heart disease and a history of cardiac surgery. Although somewhat laborious to construct, such maps offer great detail to guide ablation, and also provide voltage information to guide identification of patches and scars. Similarly, non-contact mapping systems (eg, EnSite, Endocardial Solutions) are available which record far-field potentials and employ solutions to the inverse problem to reconstruct endocardial potentials. Three-dimensional mapping based on a single beat is possible, and the success of ablation in creating conduction block in unique channels can be evaluated. Both of these three-dimensional mapping systems have the potential to improve outcomes in patients with complex disease undergoing ablation. Finally, patients are now undergoing combined procedures with interventional catheterization (eg, device closure, stenting) along with EP procedures (eg, ablation, device implant) in the same laboratory session. The state of the art in invasive pediatric EP increasingly involves the use of advanced technology adapted for use in pediatric and congenital heart disease applications.

Surgical treatment of Wolf-Parkinson-White syndrome during plastic operations in patients with Ebstein's anomaly.

OBJECTIVE: Ebstein's anomaly is the most common pathology associated with the accessory conduction pathways. METHODS: From January 1990 to August 1999 48 patients underwent surgical repair of Ebstein's anomaly by various plastic techniques. The pathways were identified and characterized at preoperative electrophysiologic mapping in 17 (34.5%) patients. There were seven males and ten females. The patients age ranged 6-35 years (mean 12.7+/-2.1 years). Five patients were in NYHA class II and were in 12 in NYHA class III. The cardiothoracic ratio ranged from 0.59 to 0.69 (mean 0.65+/-0.08). Tachycardia was present in 15 patients with Wolf-Parkinson-White (WPW) syndrome. Atrial septal defect was present in 12 patients (70. 6%). Accessory conduction pathways were in the right posterior septal area in seven patients, in right posterior septal area and free wall in seven patients and in right free wall in three. The pathways were successfully ablated in all patients during cardiopulmonary bypass. RESULTS: There were no deaths, no relapses. Follow-up ranged from 4 months to 7 years (mean 4.7+/-1.1 years). There were no late deaths, but one patient required successful tricuspid valve replacement because of severe tricuspid insufficiency. At follow-up 57.8% of patients were in NYHA class I. CONCLUSIONS: Preoperative electrophysiologic study allows to identify the accessory conduction pathways in patients with Ebstein's anomaly. The combined approach of tricuspid valve repair and surgical ablation of accessory atrioventricular connections has been proved safe and effective. This operative procedure allows to improve functional results in patients with Ebstein's malformation and concomitant accessory conduction pathways.