Ebstein’s anomaly

2020 ◽  
pp. 277-290
Author(s):  
Laura Vazquez-Garcia ◽  
Michael Rigby
Keyword(s):  
Exercise Tolerance ◽  
Ebstein’S Anomaly ◽  
Surgical Strategies ◽  
Detailed Review ◽  
Intensive Care Management ◽  
Ebstein's Anomaly ◽  
Disease Spectrum ◽  
Rare Malformation ◽  
The Right ◽  
Apical Displacement

Ebstein’s anomaly (EA) is a rare malformation of the tricuspid valve (TV), characterized by apical displacement of the annular attachments of the septal and inferior (mural) leaflets into the right ventricular (RV) cavity, with varying degrees of adherence to the underlying myocardium. The natural history is quite variable and depends on the severity and presence of associated arrhythmias, which plays a significant role with regard to the prognosis. Prenatal diagnosis is associated with higher perinatal mortality, as these usually represent the severe end of the disease spectrum. Different surgical strategies have been employed, although the cone operation, introduced in the past decade, has significantly changed the surgical approach and improved the outcomes of these patients. This chapter presents the case of a newborn with an antenatal diagnosis of EA who presented with severe cyanosis and major arrhythmias at birth requiring intensive care management for the first month of life. After some years of being relatively asymptomatic, he re-presented with recurrent arrhythmias and decreased exercise tolerance, requiring multiple interventions. His clinical course and management are presented, together with a detailed review of this disease from the anatomical aspects to its diagnosis and medico-surgical management.

scholarly journals Ebstein’s Anomaly, Left Ventricular Noncompaction and Gerbode-Like Defect Triad (Fetal Diagnosis and Neonatal Course)

2021 ◽  
Vol 2021 ◽  
pp. 1-5
Author(s):  
Mohammad Mehdi ◽  
Snigdha Bhatia ◽  
Mehul Patel ◽  
Ashraf Aly
Keyword(s):  
Heart Defects ◽  
Fontan Procedure ◽  
Left Ventricular ◽  
Ebstein’S Anomaly ◽  
Left Ventricular Noncompaction ◽  
Fetal Diagnosis ◽  
Ventricular Noncompaction ◽  
Ebstein's Anomaly ◽  
The Right ◽  
Apical Displacement

Ebstein’s anomaly is characterized by the apical displacement of the septal and posterior leaflets of the tricuspid valve with atrialization of the right ventricle (RV). It is commonly associated with other heart defects including left ventricular noncompaction. We describe a case of prenatally diagnosed Ebstein’s anomaly in association with left ventricular noncompaction and a septal defect between the left ventricle and the atrialized portion of the RV (Gerbode-like defect). The patient underwent a modified Blalock−Taussig shunt followed by Glenn procedure because of severe RV hypoplasia and RV outflow tract obstruction. The patient tolerated both procedures and is doing clinically well in anticipation of Fontan procedure for single ventricle palliation.

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 A Rare Manifestation of Asymptomatic Ebstein’s Anomaly with Tricuspid Valve Endocarditis

2017 ◽  
Vol 2017 ◽  
pp. 1-4
Author(s):  
Carmel Moazez ◽  
Vicken Zeitjian ◽  
Christian Breburda ◽  
Ranjini Roy
Keyword(s):  
Tricuspid Valve ◽  
Wide Spectrum ◽  
Left Ventricular ◽  
Ebstein’S Anomaly ◽  
Ebstein's Anomaly ◽  
Normal Left Ventricular ◽  
The Right ◽  
New Diagnosis ◽  
Apical Displacement ◽  
Tricuspid Valve Endocarditis

Ebstein’s anomaly is a rare congenital heart disease that presents with apical displacement of the septal and posterior leaflets of the tricuspid valve. It has a wide spectrum of clinical presentations and has been shown to manifest itself any time from birth to adulthood. Our patient is a 43-year-old male with a history of intravenous heroin abuse who presented to the emergency department with worsening shortness of breath and lower extremity edema. He denied any prior cardiac history. A transthoracic echo showed normal left ventricular function, but a large 2.2 × 2.1 cm echodensity on the septal leaflet of the tricuspid valve consistent with vegetation with severe tricuspid regurgitation and probable leaflet perforation. It also demonstrated severe right heart enlargement with atrialization of the right ventricle and apical displacement of the tricuspid valve consistent with Ebstein’s anomaly. This is a rare case of an adult who presented with asymptomatic Ebstein’s anomaly. There have been few reports of tricuspid valve endocarditis with Ebstein’s anomaly in the literature. To our knowledge, this represents the fifth reported case of a new diagnosis of Ebstein’s anomaly in the setting of endocarditis and the second case of Ebstein’s anomaly and endocarditis in an intravenous drug abuser.

SUCCESSFUL CORRECTION OF EBSTEIN’S ANOMALY CRITICAL FORM COMPLICATED BY THROMBOSIS OF THE RIGHT VENTRICLE IN 10 MONTH-OLD CHILD

2017 ◽  
Vol 96 (1) ◽  
pp. 206-208
Author(s):  
R. R. Movsesyan ◽  
V. A. Bolsunovskiy ◽  
A. V. Bolsunovskiy ◽  
A. L. Tsytko ◽  
D. R. Yamgurov
Keyword(s):  
Right Ventricle ◽  
Ebstein’S Anomaly ◽  
Ebstein's Anomaly ◽  
Critical Form ◽  
The Right

Two decades of experience on ablation in children with Ebstein’s anomaly

2021 ◽  
pp. 1-7
Author(s):  
Tevfik Karagöz ◽  
İlker Ertuğrul ◽  
Ebru Aypar ◽  
Aydın Adıgüzel ◽  
Hayrettin Hakan Aykan ◽  
...  
Keyword(s):  
Atrial Flutter ◽  
Atrial Tachycardia ◽  
Ebstein’S Anomaly ◽  
Atrioventricular Nodal Reentrant Tachycardia ◽  
Success Rates ◽  
Recurrence Rates ◽  
Ebstein's Anomaly ◽  
Accessory Pathways ◽  
Rare Occasion ◽  
The Right

Abstract Introduction: Accessory pathways are commonly seen due to delamination of tricuspid valve leaflets. In addition to accessory pathways, an enlarged right atrium due to tricuspid regurgitation and incisional scars creates substrates for atrial re-entries and ectopic tachycardia. We sought to describe our experience with catheter ablation in children with Ebstein’s anomaly. Methods and results: During the study period, of 89 patients diagnosed with Ebstein’s anomaly, 26 (30.9%) of them who underwent 33 ablation procedures were included in the study. Accessory pathways were observed in the majority of procedures (n = 27), whereas atrial flutter was observed in five, atrioventricular nodal reentrant tachycardia in five, and atrial tachycardia in two procedures. Accessory pathways were commonly localised in the right posteroseptal (n = 10 patients), right posterolateral (n = 14 patients), septal (n = two patients), and left posteroseptal (n = one patient) areas. Multiple accessory pathways and coexistent arrhythmia were observed in six procedures. All ablation attempts related to the accessory pathways were successful, but recurrence was observed in five (19%) of the ablations. Ablation for atrial flutter was performed in five patients; two of them were ablated successfully. One of the atrial tachycardia cases was ablated successfully. Conclusions: Ablation in patients with Ebstein’s anomaly is challenging, and due to nature of the disease, it is not a rare occasion in this group of patients. Ablation of accessory pathways has high success, but also relatively high recurrence rates, whereas ablation of atrial arrhythmias has lower success rates, especially in operated patients.

scholarly journals A clinical case of repeated correction of Ebstein’s anomaly using the ‘cone reconstruction’ method

2020 ◽  
Vol 24 (2) ◽  
pp. 102
Author(s):  
N. M. Troshkinev ◽  
E. A. Svyazov ◽  
O. V. Mochula ◽  
N. A. Shmakova ◽  
I. V. Ivanova ◽  
...  
Keyword(s):  
Informed Consent ◽  
Conflict Of Interest ◽  
Ebstein’S Anomaly ◽  
Reconstruction Method ◽  
Hemodynamic Parameters ◽  
Resonance Imaging ◽  
Ebstein's Anomaly ◽  
Mean Pressure ◽  
Critical Revision ◽  
The Right

<p>We report the case of a 16-year-old child with Ebstein’s anomaly who successfully underwent recorrection via the ‘cone reconstruction’ method using a tricuspid valve-in-ring implant. Because the patient had high-grade atrioventricular block, a pacemaker was implanted. Ultrasound and magnetic resonance imaging were conducted to determine the anatomy of the right ventricle and its hemodynamic parameters. Echocardiography performed at the time of discharge revealed an improvement in the hemodynamic parameters of the patient after the correction: triuspid valve regurgitation is mild and peak/mean pressure gradient 10/5 mmHg.</p><p>Received 9 January 2020. Revised 16 March 2020. Accepted 17 March 2020.</p><p><strong>Informed consent:</strong> The patient’s informed consent to use the records for medical purposes is obtained.</p><p><strong>Funding:</strong> The study did not have sponsorship.</p><p><strong>Conflict of interest:</strong> Authors declare no conflict of interest.</p><p><strong>Author contributions</strong><br />Drafting the article: N.M. Troshkinev, O.V. Mochula, O.A. Egunov<br />Literature review: N.A. Shmakova, I.V. Ivanova<br />Illustrations: N.M. Troshkinev, O.V. Mochula<br />Critical revision of the article: E.V. Krivoshchekov, E.A. Svyazov<br />Surgical treatment: E.V. Krivoshchekov, O.A. Egunov, N.M. Troshkinev<br />Final approval of the version to be published: N.M. Troshkinev, E.A. Svyazov, O.V. Mochula, N.A. Shmakova, I.V. Ivanova, O.A. Egunov, E.V. Krivoshchekov</p>

scholarly journals SURGICAL PALLIATION IN PATIENTS WITH EBSTEIN'S ANOMALY AND CONGENITAL HYPOPLASIA OF THE RIGHT VENTRICLE

1960 ◽  
Vol 40 (3) ◽  
pp. 310-320 ◽  
Author(s):  
Milton Weinberg ◽  
Juan P. Bicoff ◽  
Magnus H. Agustsson ◽  
Zwi Steiger ◽  
Benjamin M. Gasul ◽  
...  
Keyword(s):  
Right Ventricle ◽  
Ebstein’S Anomaly ◽  
Ebstein's Anomaly ◽  
Surgical Palliation ◽  
The Right

scholarly journals Hypereosinophilic Syndrome Leading to Severe Right-Sided Heart Failure in a Patient with Ebstein's Anomaly

2013 ◽  
Vol 2013 ◽  
pp. 1-3 ◽  
Author(s):  
Joas John Kyabonaki ◽  
Bjarne Linde Nørgaard ◽  
Søren Høyer ◽  
Niels Holmark Andersen
Keyword(s):  
Heart Failure ◽  
Right Ventricle ◽  
Tricuspid Regurgitation ◽  
Hypereosinophilic Syndrome ◽  
Ebstein’S Anomaly ◽  
Ebstein's Anomaly ◽  
Long Course ◽  
The Right ◽  
Explanted Heart ◽  
Severe Fibrosis

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.

scholarly journals Exercise Tolerance in Patients With Ebstein’s Anomaly

1997 ◽  
Vol 29 (7) ◽  
pp. 1615-1622 ◽  
Author(s):  
Susan G MacLellan-Tobert ◽  
David J Driscoll ◽  
Carl D Mottram ◽  
Douglas W Mahoney ◽  
Peter C Wollan ◽  
...  
Keyword(s):  
Exercise Tolerance ◽  
Ebstein’S Anomaly ◽  
Ebstein's Anomaly
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