Ebsteins anomaly of the tricuspid valve overview
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor-In-Chief: Cafer Zorkun, M.D., Ph.D. [2]}; Claudia P. Hochberg, M.D.; Priyamvada Singh, MBBS [3]
Overview
Ebstein's anomaly is a congenital heart defect in which the opening of the tricuspid valve is displaced towards the apex of the right ventricle of the heart (congenital apical displacement of the tricuspid valve that typically causes significant tricuspid regurgitation).
Anatomy
The picture below shows displacement of tricuspid valve towards the apex of right ventricle with subsequent atrialization of a portion of the morphologic right ventricle:
Pathophysiology
The annulus of the valve is in normal position. The valve leaflets however, are to a varying degree attached to the walls and septum of the right ventricle. There is subsequent atrialization of a portion of the morphologic right ventricle (which is then contiguous with the right atrium). This causes the right atrium to be large and the anatomic right ventricle to be small in size. 50% of cases involve an atrial shunt (either a PFO or an ASD).
Epidemiology and Demographics
Ebstein's anomaly is a rare congenital heart disease (observed in 5/100,000 newborns in the United States) with no gender predilection, but a higher incidence in Caucasians.
Risk Factors
Administration of Lithium Carbonate or benzodiazepines during pregnancy is associated with a higher risk of Ebstein's anomaly.
Natural History, Complications, Prognosis
The symptoms of Ebstein's anomaly vary in severity, with some patients experiencing either no symptoms or very mild symptoms and others experiencing symptoms that may worsen over time such as (cyanosis), heart failure, heart block, or other tachyarrhythmias or bradyarrhythmias. Paradoxical embolization, brain abscesses and pulmonary embolism may also occur.
Diagnosis
Symptoms
The symptoms of Ebstein's anomaly depend upon the degree of apical displacement of the tricuspid valve leaflet as well as the degree of dysfunction of the tricuspid valve. If the tricuspid valve is severely deformed, fetal hydrops may occur. If the valve is functioning, patients may remain symptom free for many years.
- Exertional dyspnea
- Failure to grow
- Fatigue and cyanosis
- Heart failure
- Murmur
- Palpitations may occur secondary to SVTs (supraventricular tachycardia) and WPW (Wolff-Parkinson-White syndrome).
- Paradoxical embolization may cause brain abscesses (right to left shunting due to interatrial communication)
Physical Examination
Ebstein's anomaly is characterized by tricuspid regurgitation and variable degrees a cyanosis depending upon the magnitude of right to left shunting. And elevation of the jugular venous pressure is often present.
Electrocardiography
The EKG is abnormal in 50 to 60% of patients, and will often show signs of right atrial enlargement, including "Himalayan" P waves which are P waves greater than 2.5 mm in height in leads 2, 3, and aVF. First-degree AV block, low QRS voltage, an atypical right bundle branch block, T wave inversions, and Wolff-Parkinson-White syndrome may also be present.
Chest X-Ray
The chest X Ray in Ebstein’s anomaly of the tricuspid valve may demonstrate cardiomegaly, a dilated right atrium and a pruned pulmonary vasculature.
Magnetic Resonance Imaging
Magnetic resonance imaging can be helpful as a diagnostic tool in conditions where the echocardiographic findings are inconclusive.[1]
Electrophysiologic Testing
Electrophysiologic testing may be done in Ebstein's anomaly patients who have rhythm disturbances such as tachyarrhythmias, or Wolff-Parkinson-White syndrome. An electrophysiology study helps in identifying accessory pathways prior to an ablation procedure.