scholarly journals Ebstein Anomaly With QRS Fragmentation on Electrocardiogram

2017 ◽  
Vol 5 (1) ◽  
pp. 232470961668871 ◽  
Author(s):  
Prakash Acharya ◽  
Jonathan Ross Ang ◽  
Bernard Gitler

Ebstein anomaly is a rare congenital disorder that involves the tricuspid valve and the right ventricle. It is associated with interatrial communication, which allows for paradoxical embolization causing unilateral blindness. Abnormal conduction through the atrialized right ventricle leads to QRS fragmentation on electrocardiogram. Its presence suggests a more severe abnormality and a higher risk of arrhythmia. The QRS fragmentation disappears after corrective surgery with resection of the atrialized right ventricle.

2021 ◽  
Vol 76 (1) ◽  
pp. 67-74
Author(s):  
Elena V. Penyaeva

Ebstein anomaly is a congenital heart disease, which is characterized by the presence of atrialized portion of the right ventricle, formed as a result of displacement of the tricuspid valve leaflets into the right ventricle and their partial adherence to the underlying myocardium. Atrialized portion in the right ventricle occupies the space between the fibrous annulus of the right atrioventricular orifice and the functional annulus of tricuspid valve, which represents a zone of closure of free (non-adherent to the underlying myocardium) edges of its leaflets. Ebstein anomaly is very rarely isolated, and can be combined with a number of heart diseases and be an integral part of hereditary syndromes. Currently, the role of genetic research in the investigation of the etiology of human diseases as well as understanding of the relationship between different diseases is increasing. The review presents literature data on the combination of Ebstein anomaly with other heart diseases (congenital heart diseases, Wolf-Parkinson-White syndrome, cardiomyopathies, including left ventricular noncompaction), inter alia, within the scope of hereditary syndromes (Noonan syndrome, 8p deletion syndrome, 18q deletion syndrome, 1p36 deletion syndrome, Pierre Robin syndrome). Genetic factors (gene and chromosomal mutations) lying at the core of Ebstein anomaly, as well as heart diseases combined with it, are highlighted. The analysis of published data suggests that Ebstein anomaly is a monogenic disease, and is characterized by allelic and locus genetic heterogeneity. The combination of Ebstein anomaly with other heart diseases is based on their genetic linkage.


2014 ◽  
Vol 33 (5) ◽  
pp. 268-274 ◽  
Author(s):  
Joseph Galea ◽  
Sarah Ellul ◽  
Aaron Schembri ◽  
Pierre Schembri-Wismayer ◽  
Jean Calleja-Agius

Cardiac congenital abnormalities are a leading cause in neonatal mortality occurring in up to 1 in 200 of live births. Ebstein anomaly, also known as Kassamali anomaly, accounts for 1 percent of all congenital cardiac anomalies. This congenital abnormality involves malformation of the tricuspid valve and of the right ventricle. In this review, the causes of the anomaly are outlined and the pathophysiology is discussed, with a focus on the symptoms, management, and treatments available to date.


2016 ◽  
Vol 19 (2) ◽  
pp. 077
Author(s):  
Ireneusz Haponiuk ◽  
Maciej Chojnicki ◽  
Konrad Paczkowski ◽  
Wojciech Kosiak ◽  
Radosław Jaworski ◽  
...  

The presence of a pathologic mass in the right ventricle (RV) may lead to hemodynamic consequences and to a life-threatening incident of pulmonary embolism. The diagnosis of an unstable thrombus in the right heart chamber usually necessitates intensive treatment to dissolve or remove the pathology. We present a report of an unusual complication of severe ketoacidosis: thrombus in the right ventricle, removed from the tricuspid valve (TV) apparatus. A four-year-old boy was diagnosed with diabetes mellitus (DM) type I de novo. During hospitalization, a 13.9 × 8.4 mm tumor in the RV was found in a routine cardiac ultrasound. The patient was referred for surgical removal of the floating lesion from the RV. The procedure was performed via midline sternotomy with extracorporeal circulation (ECC) and mild hypothermia. Control echocardiography showed complete tumor excision with normal atrioventricular valves and heart function. Surgical removal of the thrombus from the tricuspid valve apparatus was effective, safe, and a definitive therapy for thromboembolic complication of pediatric severe ketoacidosis.<br /><br />


1996 ◽  
Vol 27 (2) ◽  
pp. 343-344
Author(s):  
P.E.F. Daubeney ◽  
Z. Slavìk ◽  
B.R. Keeton ◽  
R.H. Anderson ◽  
S.A. Webber

1994 ◽  
Vol 239 (2) ◽  
pp. 216-223 ◽  
Author(s):  
Arnold C. G. Wenink ◽  
Bert J. Wisse ◽  
Pieter M. Groenendijk

2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
J Tong ◽  
P Joseph Francis ◽  
E Lee

Abstract Background The presence of an intra-cardiac mass is always a cause for concern, with regards to not only aetiology, but also treatment of complications. We describe a case series of 2 right sided cardiac myxomas, where the first case described an unusual location for tumour occurrence, while the second case provided insights into complications of a cardiac myxoma. Methods The first case involved a 70 year old asymptomatic lady who was referred for an additional heart sound. A transthoracic echocardiogram (TTE) showed a large, mobile 1.5 X 1.2 cm mass, attached to the atrial surface of septal tricuspid valve leaflet, prolapsing in and out of the right sided chambers. This was confirmed on transoesophageal echocardiogram (TEE). Cardiac MRI (magnetic resonance imaging) showed a similar mass attached to the septal tricuspid leaflet with features consistent with a myxoma. She was referred to cardiothoracic surgery, and 2 lobulated tumours arising the septal tricuspid valve and adjacent posterior leaflet were seen. The tumours were resected and a bio-prosthetic tricuspid valve replacement implanted. Histology of the tumours showed myxomatous degeneration of tricuspid valve, consistent with cardiac myxoma. The second case was a 56 year old lady who had dyspnoea, pedal oedema and an elevated jugular venous pulse on examination. A TTE done showed a large 7 X 4 cm mass extending from the right atrium (RA) into the right ventricle (RV). The left ventricular ejection fraction was 35%. Cardiac MRI confirmed the presence of a large mass in the right ventricle that exerted pressure effects on the ventricular septum and RV anterior free wall. Intra-operatively, a large RA mass attached by a stalk to the fossa ovalis was seen. The mass was excised and histology was consistent with cardiac myxoma. A repeat transthoracic echocardiogram done 2 weeks later showed normalisation of the LVEF. See images below for more information. Conclusion While myxomas are the most common benign cardiac tumours, they occur less commonly in the right atrium, and much less so on the tricuspid valve. Clinical manifestations range from being completely asymptomatic, as in the 1st case, to non-specific constitutional symptoms such as fever or general malaise, and to life-threatening complications. These include embolism to the pulmonary circulation, causing sudden death, or to the systemic circulation through an intra-cardiac shunt, causing strokes. This risk is increased if the tumour is large, polypoidal and friable. Large tumours can also cause obstructive symptoms and heart failure. Thus timely diagnosis with multi-modality imaging tools, and definitive treatment with complete resection of the tumour are essential. Continued monitoring for recurrences of the tumour, which can occur in 1-5% of all cases, should be performed as well. Abstract 479 Figure. Right sided cardiac masses


1992 ◽  
Vol 2 (1) ◽  
pp. 42-52 ◽  
Author(s):  
William N. O'Connor ◽  
Carol M. Cottrill ◽  
Michael T. Marion ◽  
Jacqueline A. Noonan

SummaryFive previously reported cases have established the combination of a small right ventricle (sometimes with Uhl's anomaly), imperforate tricuspid valve with fibrotic tensor apparatus, congenital absence of the pulmonary valve, and an intact interventricular septum with muscular subaortic stenosis as a rare variant of tricuspid atresia. In this study of three new autopsy cases, we additionally identified Ebstein's malformation of the imperforate tricuspid valve, partial Uhl's anomaly, regional dysplasia of right ventricular myocardium and thinning of the interventricular septum by intramyocardial sinusoids from the right ventricle. All three new cases had epicardial anomalies of the right coronary artery—a fistula to right ventricle, ostial stenosis and proximal arterial hypoplasia. Sinusoidal connections from the right ventricle to the right coronary artery and to the sclerotic left anterior descending artery were identified in serial sections of the right ventricle and septum. Myofiber disarray, with thick walled intramyocardial arteries and sinusoids from left ventricle, involved the bulging subaortic interventricular septum. These studies are consistent with the hypothesis that defective development of the the right ventricle, along with its blood supply and associated atrioventricular and arterial valves, may underlie this unusual form of congenital heart disease.


Author(s):  
Akira Shiose ◽  
Parag Desai ◽  
Gerard J. Criner ◽  
Sheela Pai ◽  
Robert M. Steiner ◽  
...  

A 77-year-old woman presented with shortness of breath 1 year after a right upper lobectomy for lung cancer. She showed a possible intracardiac metastasis on positron emission tomography scan. There was no other evidence of recurrence. The large right ventricular mass was associated with the right ventricle free wall, the apex, the papillary muscle, and the chordae to the tricuspid valve. After mass resection of the right ventricle, a one-and-a-half ventricular repair was performed with tricuspid valve replacement and defect closure. The patient was discharged on postoperative day 14 without complications and has been well for the first 3 months after the surgery.


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