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Features of accessory pathways in adult Ebstein's anomaly

Published on Nov 1, 2014in Europace6.1
· DOI :10.1093/europace/euu028
Wei Wei5
Estimated H-index: 5
(Guangdong General Hospital),
Xianzhang Zhan6
Estimated H-index: 6
(Guangdong General Hospital)
+ 5 AuthorsShulin Wu13
Estimated H-index: 13
(Guangdong General Hospital)
Abstract
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.
  • References (19)
  • Citations (20)
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References19
Newest
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Last. Emanuela R. Valsangiacomo Buechel (Boston Children's Hospital)H-Index: 18
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OBJECTIVES: To assess clinical presentation, treatment, and outcome of children with Ebstein's anomaly. BACKGROUND: Data on long-term outcome of children with Ebstein's anomaly are scarce. METHODS: Retrospective analysis of all children with Ebstein's anomaly treated between February, 1979 and January, 2009 in a single tertiary institution. Primary outcomes included patient survival and need for intervention, either cardiac surgery or catheter intervention. RESULTS: A total of 42 patients were d...
13 CitationsSource
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#2Fang PhH-Index: 1
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#1Laurent Roten (University of Bern)H-Index: 3
#2Peter LukacH-Index: 1
Last. Etienne Delacretaz (University of Bern)H-Index: 2
view all 8 authors...
In patients with Ebstein's anomaly (EA) arrhythmias are frequently encountered. Although most arrhythmias can be targeted with catheter ablation, specific issues render the procedure more challenging in EA. This study examines the mechanisms of the different arrhythmias related to EA and the outcome after catheter ablation.
58 CitationsSource
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#2William D. Edwards (Mayo Clinic)H-Index: 99
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Abstract Background Ebstein's anomaly has been described extensively in autopsy material. However, there have been no large surgical pathology series of this malformation. Objective To review clinical and surgical pathologic features of a large number of cases of Ebstein's anomaly from a single institution. Methods Review of medical histories, surgical reports, and surgical pathology reports at the Mayo Clinic (2000–2005). Results Among 104 patients, the mean age was 31 years (2 months–79 years)...
11 CitationsSource
Ebstein’s anomaly was first described by Wilhelm Ebstein in 1866. At autopsy of a young man with a history of palpitations’ and dyspnoea followed—before his death—by cyanosis, cardiomegaly and systolic murmur, the Berlin based doctor described a tricuspid valve abnormality. This congenital malformation is characterised by any degree of inferior displacement of the proximal attachments of the septal and posterior leaflets of the tricuspid valve from the atrioventricular ring. It occurs in about 1...
68 CitationsSource
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Background Severely symptomatic neonates and young infants with Ebstein's anomaly usually die without surgical intervention. The relative risks and benefits of single-ventricle palliation versus a two-ventricle repair are uncertain. In a recent series, 69% early survival with single-ventricle palliation was reported in 16 neonates with Ebstein's anomaly. Our institutional bias has been to do a two-ventricle repair in all such patients. Methods We reviewed our entire surgical experience with a tw...
47 CitationsSource
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“The treatment of congenital heart disease is unsatisfactory. As a rule, nothing can be done to improve patients symptomatically; in some instances digitalis may be of help.” — —L. Emmett Holt, MD, 1933 On August 26, 1938, Dr Robert Gross performed the first successful surgery for a congenital heart defect by closing a patent ductus arteriosus in a 7-year-old girl. The event marked the beginning of an interventional approach to congenital heart disease (CHD) that forever banished the sort of pes...
131 CitationsSource
Ebstein's anomaly is a rare congenital cardiac disease initially described by Wilhelm Ebstein in 1866. The primary pathology involves significant apical displacement of the septal tricuspid valve leaflet and the presence of a redundant, elongated, anterior tricuspid valve leaflet. This congenital malformation has multiple known associated morphological and electrophysiological cardiac pathologies. In the present article, we report, to the best of our knowledge for the first time in the literatur...
6 CitationsSource
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Four cases of Ebstein's anomaly are described, revealing that the clinical features permit diagnosis in the majority of instances and that cardiac catheterization affords precise confirmation of the diagnosis. The occurrence of this anomaly in acyanotic adults is pointed out, and the similarity to acquired valvular rheumatic heart disease is stressed.
345 CitationsSource
#1Pedro IturraldeH-Index: 1
#2Santiago NavaH-Index: 2
Last. Mario D. Gonzalez (UF: University of Florida)H-Index: 1
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UNLABELLED The abnormal development of the tricuspid valve in patients with Ebstein's anomaly results in several activation abnormalities including delayed intraatrial conduction, right bundle branch block (RBBB), and ventricular preexcitation. The aim of the present study was to define the ECG characteristics before and after ablation of an accessory A-V pathway (AP) in patients with Ebstein's anomaly. METHODS A series of 226 consecutive patients with Ebstein's anomaly was studied. Sixty-four p...
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Newest
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