Clinical spectrum and long-term outcome of Ebstein’s anomaly based on a 26-year experience in an Asian cohort

Chang, Ya-Mei; Wang, Jou‐Kou; Chiu, Shuenn–Nan; Lin, Ming Tai; Wu, En‐Ting; Huang, Shu‐Chien; Chen, Yih‐Sharng; Chang, Chung I.; Chiu, Ing Sh; Lin, Jiunn Lee; Lai, Ling‐Ping; Wu, Mei

doi:10.1007/s00431-008-0820-0

Cited by 21 publications

(17 citation statements)

References 25 publications

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“…Supraventricular arrhythmia was seen in 80 patients. A recent Asian cohort study of Ebstein’s anomaly found SVT in half of the patients [10]. Other series show an incidence of WPW varying from 10 to 29% of patients with Ebstein’s anomaly [3,4,11].…”

Section: Discussionmentioning

confidence: 99%

Behavior of Ebstein’s Anomaly: Single-Center Experience and Midterm Follow-Up

Legius

Bruaene²,

Deyk³

et al. 2010

Cardiology

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Objectives: Ebstein’s anomaly, characterized by an apical displacement of the tricuspid valve into the right ventricle, occurs in approximately 1/200,000 live births. Because long-term follow-up data of adults with Ebstein’s anomaly are scarce, we evaluated the outcome of our Ebstein’s anomaly patients. Methods: All patients >16 years and registered in the congenital heart disease database of our hospital with isolated Ebstein’s anomaly were selected for the study. Records were reviewed for outcome. Results: Forty-nine patients (21 males, mean age at diagnosis 29.1 ± 20.7 years) were followed for a mean time of 11.4 years (range 1.1–32.4). Twenty-five patients (51%) underwent tricuspid valve surgery (16 valvuloplasty and 9 valve replacement). Eight patients (32%) required redo tricuspid valve surgery. Twenty-six patients (52.1%) exhibited supraventricular arrhythmia, and the typical Wolff-Parkinson-White syndrome occurred in 15 patients (31.2%). Seventeen patients (34.7%) underwent ablation therapy and 5 patients (10.4%) required pacemaker implantation. Conclusions: Half of the patients with Ebstein’s anomaly needed tricuspid valve surgery and redo surgery was not uncommon. Supraventricular arrhythmia occurred frequently and ablation therapy was often indicated. Careful follow-up is obligate, as some complications occur for the first time in adulthood.

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Section: Discussionmentioning

confidence: 99%

Behavior of Ebstein’s Anomaly: Single-Center Experience and Midterm Follow-Up

Legius

Bruaene²,

Deyk³

et al. 2010

Cardiology

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“…Our data show that ≈50% of adult patients with EA experienced arrhythmic complications, which is consistent with previous studies. 2,4,5,16 In addition, we found the presence of fQRS to be independently associated with overall arrhythmic events, even after adjusting for relevant clinical and echocardiographic parameters.…”

Section: New Findingsmentioning

confidence: 71%

“…Although there have been some studies dealing with arrhythmic complications in adult patient groups, they only described the prevalence of a few types of arrhythmias in a limited number of patients. [2][3][4][5] Most of the previous data focused primarily on the characteristics or management of AVRT with a bypass tract. [14][15][16] In contrast, we evaluated the association between fQRS and arrhythmic risk as well as the severity of the structural abnormality.…”

Section: New Findingsmentioning

confidence: 99%

“…1 Therefore, the most common clinical presentations in adolescents and adults with this malformation are arrhythmic events and right-sided heart failure. [2][3][4][5] Moreover, these complications are expected to become more frequent with the improvement in long-term survival of patients. 5,6 However, there have been few studies investigating the risk factors for arrhythmic complications, especially in adults with EA.…”

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confidence: 99%

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Fragmented QRS Complex in Adult Patients With Ebstein Anomaly and Its Association With Arrhythmic Risk and the Severity of the Anomaly

Park

Chung

et al. 2013

Circ: Arrhythmia and Electrophysiology

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Background-Fragmented QRS complex (fQRS) on 12-lead ECG, a marker of myocardial scar, is a predictor of arrhythmic events in patients with ischemic and nonischemic cardiomyopathy. We investigated whether the presence of fQRS is associated with the severity of the anomaly and with increased arrhythmic events in adult patients with Ebstein anomaly (EA). Methods and Results-In 51 consecutive adult patients with EA (median age, 37 years; 18 males), the severity index of EA calculated from echocardiographic data and clinical arrhythmic events were analyzed. The extent of fQRS in each patient was measured by counting the number of ECG leads showing fQRS. There were 35 (68.6%) patients with fQRS (fQRS group) and 16 (31.4%) patients without fQRS (non-fQRS group). fQRS was observed more frequently in the inferior (n=26) and precordial (n=25) leads versus the lateral leads (n=5). The patients in the fQRS group had a worse functional class, greater cardiothoracic ratios, more severe tricuspid regurgitation, larger atrialized right ventricular areas, higher EA severity scores, and more frequent arrhythmic events compared with those in the non-fQRS group. The atrialized right ventricular area showed a positive correlation with the fQRS extent (r=0.51; P<0.001). In multivariable Cox regression models, the presence of fQRS was independently associated with arrhythmic events (P=0.036). Conclusions-Fragmented

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“…However, despite appropriate treatment, mortality proved to be very high in the first years of life, as has been reported previously in EA patients. 24 The cluster analysis of EA comorbidities identified 2 clearly …”

Section: Discussionmentioning

confidence: 99%

Identification of clinically relevant phenotypes in patients with Ebstein anomaly

Cabrera

Miranda-Fernández

Huertas-Quiñones

et al. 2018

Clinical Cardiology

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BackgroundEbstein anomaly (EA) is a heterogeneous congenital heart defect (CHD), frequently accompanied by diverse cardiac and extracardiac comorbidities, resulting in a wide range of clinical outcomes.HypothesisPhenotypic characterization of EA patients has the potential to identify variables that influence prognosis and subgroups with distinct contributing factors.MethodsA comprehensive cross‐sectional phenotypic characterization of 147 EA patients from one of the main referral institutions for CHD in Colombia was carried out. The most prevalent comorbidities and distinct subgroups within the patient cohort were identified through cluster analysis.ResultsThe most prevalent cardiac comorbidities identified were atrial septal defect (61%), Wolff‐Parkinson‐White syndrome (WPW; 27%), and right ventricular outflow tract obstruction (25%). Cluster analysis showed that patients can be classified into 2 distinct subgroups with defined phenotypes that determine disease severity and survival. Patients in cluster 1 represented a particularly homogeneous subgroup with a milder spectrum of disease, including only patients with WPW and/or supraventricular tachycardia (SVT). Cluster 2 included patients with more diverse cardiovascular comorbidities.ConclusionsThis study represents one of the largest phenotypic characterizations of EA patients reported. The data show that EA is a heterogeneous disease, very frequently associated with cardiovascular and noncardiovascular comorbidities. Patients with WPW and SVT represent a homogeneous subgroup that presents with a less severe spectrum of disease and better survival when adequately managed. This should be considered when searching for genetic causes of EA and in the clinical setting.

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Clinical spectrum and long-term outcome of Ebstein’s anomaly based on a 26-year experience in an Asian cohort

Cited by 21 publications

References 25 publications

Behavior of Ebstein’s Anomaly: Single-Center Experience and Midterm Follow-Up

Behavior of Ebstein’s Anomaly: Single-Center Experience and Midterm Follow-Up

Fragmented QRS Complex in Adult Patients With Ebstein Anomaly and Its Association With Arrhythmic Risk and the Severity of the Anomaly

Identification of clinically relevant phenotypes in patients with Ebstein anomaly

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