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.

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

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

Reconstruction of the Right Heart in an Elderly Woman With Ebstein’s Anomaly and Severe Heart Failure

2007 ◽  
Vol 84 (5) ◽  
pp. 1745-1746 ◽  
Author(s):  
Takuya Nakayama ◽  
Miki Asano ◽  
Kozo Matsumoto ◽  
Norikazu Nomura ◽  
Takayuki Saito ◽  
...  
Keyword(s):  
Heart Failure ◽  
Elderly Woman ◽  
Severe Heart Failure ◽  
Ebstein’S Anomaly ◽  
Right Heart ◽  
Ebstein's Anomaly ◽  
The Right

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.

Tricuspid valve relocation with endomyocardial fibrosis removal for Löeffler’s endocarditis

2019 ◽  
Vol 56 (3) ◽  
pp. 622-624
Author(s):  
Tohru Asai ◽  
Fumihiro Miyashita ◽  
Hiromitsu Nota ◽  
Piers N Vigers
Keyword(s):  
Heart Failure ◽  
Right Ventricle ◽  
Right Atrium ◽  
Hypereosinophilic Syndrome ◽  
Right Heart Failure ◽  
Right Atrial ◽  
Endomyocardial Fibrosis ◽  
Right Heart ◽  
Atrial Enlargement ◽  
The Right

Abstract Löffler endocarditis with hypereosinophilic syndrome is rare but can cause critical ventricular obliteration by endomyocardial fibrosis. A 52-year-old woman experienced severe right heart failure with extreme shrinkage of her right ventricle, severe tricuspid regurgitation and marked right atrial enlargement. Preoperative tests showed identical pressures in the right atrium and pulmonary artery. Endocardial stripping was done, and to enlarge the right ventricle, we relocated the anterior and posterior tricuspid leaflets cephalad, up the right atrium wall, to ‘ventricularize’ a portion of the right atrium, with autologous pericardial augmentation of the tricuspid leaflets. An annuloplasty ring was added to reinforce the relocated tricuspid attachment. Right heart pressures normalized postoperatively. The patient recovered uneventfully. She has received corticosteroid therapy continuously and has shown no recurrence of heart failure in the 5 years since surgery.

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.

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

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.

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

2020 ◽  
Vol 4 (4) ◽  
pp. 1-5 ◽  
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.

Sign in / Sign up

Export Citation Format

Share Document