Cone repair for Ebstein’s anomaly and atrial fibrillation ablation in an adult patient

2021 ◽  
Keyword(s):  
Atrial Fibrillation ◽  
Tricuspid Valve ◽  
Pulmonary Veins ◽  
Maze Procedure ◽  
Right Atrial ◽  
Ebstein’S Anomaly ◽  
Ebstein's Anomaly ◽  
Previously Treated ◽  
Cone Repair ◽  
The Right

We present a 52-year-old woman with Ebstein’s anomaly not previously treated. In this subset of patients, there are no clear guidelines regarding the best surgical strategy for treating the tricuspid valve: replace it or repair it. In this case, extensive repair of the tricuspid valve and the right ventricle is achieved using the cone repair technique popularized by Dr. José Pedro Da Silva. Because the patient also presented with symptomatic paroxysmal atrial fibrillation, a right atrial maze procedure combined with isolation of the pulmonary veins was performed using both radiofrequency and cryotherapy. At the last follow-up, 2 years after the repair, the patient is asymptomatic and maintains sinus rhythm. The last echocardiogram showed mild tricuspid regurgitation with normal right ventricular function.

scholarly journals Ebstein's anomaly as a cause of paroxysmal atrial fibrillation

2008 ◽  
Vol 65 (11) ◽  
pp. 847-850
Author(s):  
Miodrag Damjanovic ◽  
Danijela Djordjevic-Radojkovic ◽  
Zoran Perisic ◽  
Svetlana Apostolovic ◽  
Goran Koracevic ◽  
...  
Keyword(s):  
Atrial Fibrillation ◽  
Right Ventricle ◽  
Tricuspid Valve ◽  
Paroxysmal Atrial Fibrillation ◽  
Ebstein’S Anomaly ◽  
Ebstein's Anomaly ◽  
Wpw Syndrome ◽  
Echocardiographic Examination ◽  
Electrophysiological Examination ◽  
The Right

Background. Ebstein's anomaly is characterized by a displacement of the tricuspid valve toward apex, because of anomalous attachment of the tricuspid leaflets. There are type B of Wolff-Parkinson-White (WPW) syndrome and paroxysmal arrhythmias in more than a half of all patients. Case report. We presented a female, 32-year old, with frequent paroxysms of atrial fibrillation. After conversion of rhythm an ECG showed WPW syndrome. Echocardiographic examination discovered normal size of the left cardiac chambers with paradoxical ventricular septal motion. The right ventricle was very small because of its atrialization. The origin of the tricuspid valve was 20 mm closer to apex of the right ventricle than the origin of the mitral valve. Electrophysiological examination showed a posterolateral right accesorial pathway. Atrial fibrillation was induced very easily in electrophysiological laboratory and a successful ablation of accessorial pathway was made. There were no WPW syndrome and paroxysms of atrial fibrillation after that. Conclusion. Ebstein's anomaly is one of the reasons of paroxysmal atrial fibrillation, especially in young persons with WPW syndrome.

Ebstein’s Anomaly

2007 ◽  
Vol 26 (3) ◽  
pp. 197-208 ◽  
Author(s):  
Sarah Pashia
Keyword(s):  
Heart Failure ◽  
Tricuspid Valve ◽  
Congenital Heart ◽  
Clinical Presentation ◽  
Ebstein’S Anomaly ◽  
Ebstein's Anomaly ◽  
Clinical Presentations ◽  
Wide Range ◽  
Pathologic Findings ◽  
The Right

EBSTEIN’S ANOMALY IS A RARE congenital heart defect characterized by displacement of the tricuspid valve leaflets into the right ventricle.1The defect was first described by Wilhelm Ebstein in 1866.2This anomaly of the tricuspid valve causes the right atrium to thin and become enlarged, resulting in a wide range of clinical presentations.3Clinical presentation depends on the severity of the pathologic findings, which vary considerably from patient to patient. Some infants may present with cyanosis, respiratory distress, heart failure, and even death, whereas others may not present with mild symptoms until adolescence or adulthood.

Cor triatriatum dexter masquerading as Ebstein's anomaly

2011 ◽  
Vol 21 (3) ◽  
pp. 354-356 ◽  
Author(s):  
Souheir Salam ◽  
David Gallacher ◽  
Orhan Uzun
Keyword(s):  
Tricuspid Valve ◽  
Transthoracic Echocardiography ◽  
Right Atrium ◽  
Cor Triatriatum ◽  
Ebstein’S Anomaly ◽  
Ebstein's Anomaly ◽  
Cor Triatriatum Dexter ◽  
The Right

AbstractWe report the case of a child with cor triatriatum dexter masquerading as Ebstein's anomaly on transthoracic echocardiography. This was attributed to a floppy membrane arising from the right atrium, protruding into the tricuspid valve and pushing the leaflets downwards, giving an impression of Ebstein's anomaly. The importance of recognising this pitfall will prevent misdiagnosis of Ebstein's anomaly.

The Right Ventricular Infundibulum in Ebstein's Anomaly of the Tricuspid Valve

Radiology ◽  
1967 ◽  
Vol 89 (4) ◽  
pp. 694-700 ◽  
Author(s):  
Larry P. Elliott ◽  
Alexis F. Hartmann
Keyword(s):  
Right Ventricular ◽  
Tricuspid Valve ◽  
Ebstein’S Anomaly ◽  
Ebstein's Anomaly ◽  
The Right

scholarly journals Successful One and One-half Ventricular Circulation with Bioprosthetic Tricuspid Valve Replacement and Total Chordal Preservation in a Patient with Ebstein’s Anomaly: A Video Presentation

2021 ◽  
Vol 5 (02) ◽  
pp. 147-150
Author(s):  
Ujjwal K. Chowdhury ◽  
Sukhjeet Singh ◽  
Niwin George ◽  
Lakshmi Kumari Sankhyan ◽  
Sandeep Sharan ◽  
...  
Keyword(s):  
Right Ventricle ◽  
Tricuspid Valve ◽  
Ebstein’S Anomaly ◽  
Anterior Leaflet ◽  
Tricuspid Annulus ◽  
Tricuspid Valve Replacement ◽  
Rotational Displacement ◽  
Atrioventricular Junction ◽  
Ebstein's Anomaly ◽  
The Right

AbstractEbstein’s anomaly is a rare congenital malformation of the right ventricle and tricuspid valve which is characterized by several features that can exhibit an infinite spectrum of malformation.The abnormalities include: i) adherence of the tricuspid leaflets to the underlying myocardium (failure of delamination); ii) anterior and apical rotational displacement of the functional tricuspid annulus (septal > posterior > anterior leaflet); iii) dilatation of the right atrioventricular junction (the true tricuspid annulus) with variable degrees of hypertrophy and thinning of the wall; iv) dilatation of the “atrialized” portion of the right ventricle; and v) redundancy, fenestration, and tethering of the anterior leaflet.

scholarly journals Atrial Fibrillation Drivers: Redefining the Electrophysiological Substrate

2020 ◽  
Author(s):  
Markus Rottmann ◽  
Anna Pfenniger ◽  
Shin Yoo ◽  
David Johnson ◽  
Gail Elizabeth Geist ◽  
...  
Keyword(s):  
Atrial Fibrillation ◽  
Pulmonary Veins ◽  
Persistent Atrial Fibrillation ◽  
Right Atrial ◽  
Atrial Pacing ◽  
New Paradigm ◽  
Density Mapping ◽  
Right Atrial Appendage ◽  
The Right ◽  
Activation Delay

Background: We performed high-density mapping of persistent atrial fibrillation (AF) in animals and patients (1) to test that AF is due to greater than or equal 1 reentry, and (2) to characterize activation delay and reentries pre/ post pulmonary vein isolation (PVI). We determined electrophysiological characteristics that may predispose to the induction, maintenance, and reduction of AF. Methods and Results: This study includes 48 dogs and nine patients. 43 AF- and five sinus/ paced rhythm dogs (3-14 weeks rapid atrial pacing) were studied at open chest surgery with 117 epicardial electrograms (EGMs) (2.5mm dist.) in 6 bi-atrial regions. Rotational activity automatically detected with a new algorithm tracking the earliest and latest activation in all regions (5+/-2 per region) were stable over 424+/-505ms [120-4940ms]. Reentry stability was highest in the right atrial appendage (RAA) (405+/-219ms) and the posterior left atrium (PLA) (267+/-115ms) and anchored between >=3 zones of activation delay (15+/-5ms, median 13ms) defined as >10ms per 2.5mm. Cycle length (CL) and degree of focal fibrosis were highest in the PLA and left atrial free wall (LAFW) with 94+/-7ms, 96+/-5ms, and 49+/-14%, 47+/-19%. Fiber crossing density correlated with the stability of rotational activity (R=0.6, P<0.05). Activation delay was 2x higher in AF compared to sinus rhythm/paced rhythm (interval 200-500ms). Activation delay zones > 10ms were at the same locations, but increased 4x during AF vs. SR and were located at fiber crossings, fibrosis/ fat zones. Stability of rotational activity correlated with Organization Index (OI), Fraction Index (FI), Shannon's Entropy (ShEn), and CL (R>0.5, p< 0.0001). PVI in five hearts increased CL [2-14%] and reduced stability of rotational activity in nearly all regions remote to the pulmonary veins (PVs). Also in the clinical evaluation in nine patients using the HD-catheter (16 electrodes, 3mm dist.) activation delay at the reentrant trajectory was 2x higher at edges with maximal delay (20.5+/-8.1ms, median 19.6ms) vs (9.3+/-8.8ms, median 9.2ms) and 1.4 x higher during AF (13.0+/-18.7ms, median 7.2ms) compared to SR/ CS-pacing (18.0+/-11.6ms, median 17.7ms). Conclusion: Rotational activities in all bi-atrial regions anchored between small frequency-dependent activation delay zones in AF. PVI led to beneficial remodeling in bi-atrial regions remote to the PVs. These data may identify a new paradigm for persistent AF.

Radiofrequency Ablation of Atrial Fibrillation in Patients with Ebstein's Anomaly: A Two-Case Report

Cardiology ◽  
2017 ◽  
Vol 139 (1) ◽  
pp. 33-36 ◽  
Author(s):  
Konstantinos E. Iliodromitis ◽  
Marc Bonsels ◽  
Rolf Borchard ◽  
Anja Dorszewski
Keyword(s):  
Atrial Fibrillation ◽  
Radiofrequency Ablation ◽  
Right Ventricle ◽  
Ebstein’S Anomaly ◽  
Atrioventricular Nodal Reentrant Tachycardia ◽  
Reentrant Tachycardia ◽  
Ebstein's Anomaly ◽  
The Right ◽  
Apical Displacement

Ebstein's anomaly (EA) is a rare congenital heart disease characterized by “atrialization” of the right ventricle, due to apical displacement of the tricuspid leaflets into the right ventricle. Patients with EA may develop all kinds of supraventricular arrhythmias requiring radiofrequency ablation. Atrial fibrillation (Afib) is a common arrhythmia in EA patients, and results in debilitating symptoms that often require surgical treatment. This is a follow-up report of 2 patients with EA undergoing radiofrequency ablation for Afib. The first patient underwent pulmonary vein isolation (PVI) and the ablation of a concomitant atrioventricular nodal reentrant tachycardia. The second patient was also treated with a PVI and a redo PVI 8 months later. Both patients remain in sinus rhythm 8 months on. Radiofrequency ablation is the therapy of choice for patients with pharmacological refractory Afib, but it is not common in patients with EA.

scholarly journals Prenatal Sonographic Detection of Ebstein’s Anomaly

2017 ◽  
Vol 33 (3) ◽  
pp. 225-230
Author(s):  
Mariah C. Root ◽  
Kelsy L. Fisher
Keyword(s):  
Tricuspid Valve ◽  
Congenital Heart Defect ◽  
Congenital Heart ◽  
Ebstein’S Anomaly ◽  
Rare Type ◽  
Sonographic Appearance ◽  
Ebstein's Anomaly ◽  
Treatment Characteristic ◽  
The Right

Ebstein’s anomaly is a rare type of congenital heart defect characterized by a malformation of the tricuspid valve and the right side of the heart. This case study presents a well-documented case of Ebstein’s anomaly that was diagnosed prenatally using sonography. The ability of prenatal sonography to detect and accurately diagnosis this case allowed for a change in the management of the pregnancy to properly evaluate the condition and prepare for treatment. In addition, information regarding Ebstein’s anomaly is reviewed and specifically addresses etiology, symptoms, diagnosis, treatment, characteristic sonographic appearance, and common differential diagnoses.

scholarly journals Tricuspid valve dysplasia and Ebstein's anomaly in dogs: case report

2006 ◽  
Vol 58 (5) ◽  
pp. 762-767 ◽  
Author(s):  
M.G. Sousa ◽  
D.G. Gerardi ◽  
R.O. Alves ◽  
A.A. Camacho
Keyword(s):  
Heart Failure ◽  
Tricuspid Valve ◽  
Medical Therapy ◽  
Diagnostic Methods ◽  
The Other ◽  
Ebstein’S Anomaly ◽  
Post Mortem ◽  
Ebstein's Anomaly ◽  
Tricuspid Insufficiency ◽  
The Right

Two cases of tricuspid valve dysplasia are reported. Dogs were presented for evaluation of weakness and ascites. In both cases, echocardiography disclosed tricuspid insufficiency and, in one of them, tricuspid leaflets also were displaced down into the right ventricle, substantiating Ebstein's anomaly. Medical therapy for congestive heart failure was initiated. One of the dogs suddenly died shortly after diagnosis was established. Although the other dog recovered much better initially, sudden death also occurred. Post-mortem examinations showed right atrioventricular enlargement, and thickened tricuspid leaflets. Clinical features, diagnostic methods and medical therapy are discussed in this paper.

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