Ebstein's anomaly: sixteen years' experience with valve replacement without plication of the right ventricle.

A 36-year-old male with mild Ebstein's anomaly developed severe right-sided heart failure, following a 5-year-long course of hypereosinophilic syndrome. No regular followups had been done, during the years of antineoplastic therapy. A year after being cured from the hypereosinophilic syndrome, the patient developed right-sided heart failure symptoms and was found to have excessive fibrosis of the right ventricular endocardium and free tricuspid regurgitation. The findings were compatible with substantial scarring of the endocardium caused by the hypereosinophilic syndrome. Over a few years, the patient deteriorated significantly and was finally offered a heart transplant. Examination of the explanted heart revealed severe fibrosis of the right ventricle and almost complete sparing of the left.

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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

Journal of Cardiac Critical Care TSS ◽

10.1055/s-0041-1723626 ◽

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.

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

Cardiology ◽

10.1159/000484039 ◽

2017 ◽

Vol 139 (1) ◽

pp. 33-36 ◽

Cited By ~ 1

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.

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P843 A rare cause of chest pain: paradoxical embolism in Ebstein"s anomaly

European Heart Journal - Cardiovascular Imaging ◽

10.1093/ehjci/jez319.492 ◽

2020 ◽

Vol 21 (Supplement_1) ◽

Author(s):

H S A Abdelgawad ◽

N Hisham ◽

M Shehata ◽

M A Abdelhay

Keyword(s):

Congenital Heart Disease ◽

Heart Disease ◽

Right Ventricle ◽

Patent Foramen Ovale ◽

Congenital Heart ◽

Paradoxical Embolism ◽

Ebstein’S Anomaly ◽

Foramen Ovale ◽

Ebstein's Anomaly ◽

The Right

Abstract Introduction Ebstein’s anomaly is characterized by displacement of the proximal attachments of the tricuspid valve from the atrioventricular ring into the right ventricle. This structural abnormality divides the right ventricle into an ‘atrialized’ portion and a distal ‘ventricularized’ portion. The severity is variable and accounts for the broad clinical spectrum, from severe disease causing fetal or neonatal death to mild disease compatible with natural survival as late as the eighth decade of life. Ebstein’s anomaly is an uncommon defect occurring in less than 1% of patients with congenital heart disease, but it is disproportionately represented in the adult congenital heart disease population because of its favourable natural history Case report A 55 year old man with no previous cardiac history .He presented to our medical facility complaining of acute retrosternal squeezing retrosternal chest pain few hours before admission. On clinical examination, he had a pansystolic murmur over the tricuspid area .ECG showed right bundle branch block. Laboratory results were unremarkable except elevated cardiac enzymes. 2D Transthoracic Echocardiography revealed a small well-functioning right ventricle, 15 mm/m2 apical displacement of the tricuspid septal leaflet with severe tricuspid regurgitation . 2D and 3D transesophageal echocardiography with intravenous agitated saline injection revealed patent foramen ovale with right to left shunt with immediate crossing of bubbles.Coronary angiography revealed normal coronaries. The patient refused intervention and he was maintained on warfarin treatment . Conclusion The clinical presentation of Ebstein’s anomaly is highly variable—depending on anatomic severity, haemodynamics ,and degree of interatrial shunting. The majority of patients have shunting through a secundum ASD or patent-foramen ovale. Paradoxical embolism maybe an indication for PFO/ ASD closure Abstract P843 Figure.

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Left heart matters in Ebstein’s anomaly: the importance of a closer follow up—a case report

European Heart Journal - Case Reports ◽

10.1093/ehjcr/ytaa077 ◽

2020 ◽

Vol 4 (4) ◽

pp. 1-5 ◽

Cited By ~ 1

Author(s):

Maria Victoria Ordoñez ◽

Radwa Bedair ◽

Stephanie L Curtis

Keyword(s):

Right Ventricle ◽

Dynamic Compression ◽

Symptomatic Patient ◽

Left Ventricular ◽

Ebstein’S Anomaly ◽

Ebstein's Anomaly ◽

Ventricular Outflow Obstruction ◽

Left Ventricular Outflow ◽

The Right

Abstract Background Ebstein’s anomaly (EA) is mainly thought of as a right heart condition, however, congenital left-sided lesions can co-exist. Therefore, it is paramount to include the left side of the heart as part of a routine investigation in these patients. We present a 57-year-old symptomatic patient with EA and progressive tricuspid regurgitation (TR) associated with acquired left ventricular outflow obstruction (LVOTO). Case summary A 57-year-old women, known to have severe EA presented with shortness of breath and chest pain on exertion secondary to progression of the tricuspid valve regurgitation and right ventricle dilatation leading to a dynamic compression of the left outflow tract requiring surgical intervention. Discussion Left ventricular obstruction secondary to severe TR and dilation of the right ventricle can present and remain silent at rest but becoming significant on exertion. Therefore, we recommend that all patients with EA and significant TR undergo exercise echocardiography at regular intervals to specifically look for acquired dynamic LVOTO.

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The shape and function of the right ventricle in Ebstein's anomaly

International Journal of Cardiology ◽

10.1016/j.ijcard.2012.03.062 ◽

2013 ◽

Vol 167 (3) ◽

pp. 704-710 ◽

Cited By ~ 20

Author(s):

Christopher M. Lee ◽

Florence H. Sheehan ◽

Beatriz Bouzas ◽

Sylvia S.M. Chen ◽

Michael A. Gatzoulis ◽

...

Keyword(s):

Right Ventricle ◽

Ebstein’S Anomaly ◽

Ebstein's Anomaly ◽

The Right ◽

And Function

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Ebstein's anomaly as a cause of paroxysmal atrial fibrillation

Vojnosanitetski pregled ◽

10.2298/vsp0811847d ◽

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.

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