CASE 16850 Published on 03.07.2020

Ebstein's Anomaly

Section

Cardiovascular

Case Type

Clinical Cases

Authors

 Dr. Babatunde Ebenezer Osobu1, Prof. Dr. Christian Loewe2

1. Radiology Department, University College Hospital, PMB 5116, Queen Elizabeth Road, Mokola, Ibadan, Oyo State, Nigeria

2. Division of Cardiovascular Imaging and Interventional Radiology, Department of Biomedical Imaging and image-guided therapy, Medical University of Vienna, Vienna, Austria.

Patient

68 years, male

Categories
Area of Interest Cardiac ; Imaging Technique Conventional radiography, CT
Clinical History

A 68-year-old male patient who presented with palpitation and dyspnea of a week duration.

Imaging Findings

The patient had chest x-ray with frontal and lateral views done which showed moderate cardiomegaly with biventricular preponderance. Ring-like calcific opacity is noted in the region of the mitral valve (Fig 1 – 2). A suspicion of Ebstein’s Anomaly was made.

Contrast-enhanced CT scan of the heart with axial and coronal views were performed which showed cardiomegaly with enlarged right atrium and ventricle. There is right ventricular myocardial hypertrophy with displacement of the septal and posterior leaflet of the tricuspid valve (white arrow) in the direction of the apex. Calcified mitral valve is also seen (Fig 3 – 4). A diagnosis of Ebstein’s Anomaly was made.

 

Discussion

Ebstein’s anomaly is a rare congenital cardiac anomaly characterized by variable developmental anomaly of the tricuspid valve and right-sided cardiac chambers. It accounts for only 0.5% of congenital cardiac defects (1) and occurs in 1 in 210,000 live births (2).  Although, it is the most common cause of congenital tricuspid regurgitation. No gender predilection. Most cases are sporadic but familiar Ebstein’s anomaly has been reported (3). It may also be seen in patients with Noonan syndrome and long term Lithium therapy (3).

In Ebstein’s anomaly a portion of the right ventricle lies between the normally located tricuspid valve annulus and the displaced tricuspid valve leaflets. This portion is referred to as the “atrialized” right ventricle while the remaining right ventricle distal to the displaced leaflets is known as the functional right ventricle (4).

The systemic venous return during a cardiac cycle flows from the right atrium into the atrialized right ventricle during atrial systole. In ventricular systole, some blood flows to the pulmonary trunk from the functional right ventricle but the blood within the atrialized right ventricle flows retrogradely into the true right atrium. Therefore, there is functional obstruction at the level of the tricuspid annulus. Pressure and volume overload at the atrial level may be relieved by an Atrial septal defect or a persistent foramen ovale (4).

Clinical manifestations depend on severity of the abnormality. It often presents during antenatal period with the development of Hydrops fetalis and fetal tachyarrythmias. In less severe cases, it may present at birth. The physiological closure of foramen ovale worsens the symptoms. An infant may present acutely in the neonatal period with tachypnea, difficulty in feeding and cyanosis. Older patients with less severity may present with dyspnea or palpitation. In some individuals, Ebstein’s anomaly is not diagnosed until the eight decades of life (5).

It can be associated with Right ventricular outflow tract abnormalities, Atrial septal defect (especially ostium secundum type), Ventricular septal defect and Tetralogy of Fallot.

Chest radiograph depends on the severity of the abnormality. There is often cardiomegaly with right-sided preponderance giving the “box shape”. There may be pulmonary oligemia. In some cases, chest radiograph finding is relatively nonspecific.

Although the diagnosis of Ebstein’s anomaly is based on Echocardiographic findings, Magnetic Resonance Imaging and Computed Tomography allow direct visualization of anatomic details.

Management depends upon the degree of impairment of cardiac function in the severely ill neonate, it is imperative to keep the Foramen ovale and the Ductus arteriosus patent. Surgery when required consists of Tricuspid valve replacement, placation of the atrialized right ventricle and closure of the defect in the atrial septum (4).

Differential Diagnosis List
Ebstein’s anomaly
Pulmonary Stenosis
Tetralogy of Fallot
Large Pericardial effusion
Intracardiac tumours
Final Diagnosis
Ebstein’s anomaly
Case information
URL: https://www.eurorad.org/case/16850
DOI: 10.35100/eurorad/case.16850
ISSN: 1563-4086
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