Cor triatriatum dexter masquerading as Ebstein's anomaly

2011 ◽  
Vol 21 (3) ◽  
pp. 354-356 ◽  
Author(s):  
Souheir Salam ◽  
David Gallacher ◽  
Orhan Uzun

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.

2007 ◽  
Vol 26 (3) ◽  
pp. 197-208 ◽  
Author(s):  
Sarah Pashia

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.


2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
H S A Abdelgawad ◽  
M Abdelnabi ◽  
A Almaghrabi ◽  
M Shehata ◽  
M A Abdelhay

Abstract Introduction Cor triatriatum dexter, or partitioning of the right atrium (RA) to form a triatrial heart, is an extremely rare congenital anomaly that is caused by the persistence of the right valve of the sinus venosus. The incidence of cor triatriatum is approximately 0.1% of congenital heart malformation. Typically, the right atrial partition is due to exaggerated fetal eustachian and the besian valves, which together form an incomplete septum across the lower part of the atrium. This septum may range from a reticulum to a substantial sheet of tissue Case report: 45-years old female patient with history of surgical closure of an atrial septal defect at the age of 14 years .She presented to our medical facility complaining of exertional dyspnea and bilateral lower limb edema for 4 years. On clinical examination, she had bilateral congested neck veins, a pansystolic murmur over the tricuspid area and a tender hepatomegaly. 2D Transthoracic Echocardiography revealed an unusual membranous structure that stretched across the right atrium with attachments superiorly at the free wall and inferiorly at the inter-atrial septum with a severe tricuspid regurgitation (Panel A)Intravenous agitated saline injection revealed an incomplete membrane. (Panel B).2D Transesophageal echocardiography showed an unusually prominent eustachian valve arose normally from the ostium of the inferior vena cava (IVC) and was pointing towards the interatrial septum just below the level of the fossa ovalis and no residual ASD could be seen. (Panel C) . 3D transesophageal echocardiography with zoomed mode from right atrial perspective confirmed the presence of an incomplete membrane extending transversely from the ostium of IVC and interatrial septum immediately below the fossa ovalis but not reaching RA free wall (arrow), no obstruction to the flow of the IVC, superior vena cava (SVC) , coronary sinus (CS) and the tricuspid valve (TV) was seen .(Panels D,E,F). Conclusion Since many patients are asymptomatic, the diagnosis of cor triatriatum dexter often is determined at postmortem examination. Antemortem diagnosis can be determined by echocardiography. 3D transesophageal echocardiography was able to detect cor triatriatum dexter that can be easily missed by 2D echocardiography. Abstract P1464 Figure.


2021 ◽  

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.


Radiology ◽  
1967 ◽  
Vol 89 (4) ◽  
pp. 694-700 ◽  
Author(s):  
Larry P. Elliott ◽  
Alexis F. Hartmann

2021 ◽  
Vol 5 (02) ◽  
pp. 147-150
Author(s):  
Ujjwal K. Chowdhury ◽  
Sukhjeet Singh ◽  
Niwin George ◽  
Lakshmi Kumari Sankhyan ◽  
Sandeep Sharan ◽  
...  

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.


Heart ◽  
1976 ◽  
Vol 38 (1) ◽  
pp. 108-111 ◽  
Author(s):  
L M Gerlis ◽  
R H Anderson

2017 ◽  
Vol 33 (3) ◽  
pp. 225-230
Author(s):  
Mariah C. Root ◽  
Kelsy L. Fisher

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.


2006 ◽  
Vol 58 (5) ◽  
pp. 762-767 ◽  
Author(s):  
M.G. Sousa ◽  
D.G. Gerardi ◽  
R.O. Alves ◽  
A.A. Camacho

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.


1996 ◽  
Vol 6 (1) ◽  
pp. 80-83
Author(s):  
Eberhard G. Mühler ◽  
Andreas Franke ◽  
Bruno J. Messmer

SummaryWe describe the symptoms, the techniques for imaging and the surgical therapy in a patient with divided right atrium (‘cor triatriatum dexter’) combined with atresia of the superior caval vein. In addition to peripheral venous congestion and cyanosis, macrocephaly was the most prominent sign, the high pressure in the superior caval vein disturbing the circulation of cerebrospinal fluid. Transesophageal echocardiography was the optimal technique for imaging the dividing shelf in the right atrium. Cardiac catheterization after surgical excision of the shelf and reconstruction of the junction between the superior caval vein and the right atrium excluded any residual gradient or shunt.


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