Ebstein’s anomaly of the tricuspid valve Ebstein’s anomaly Physical examination with Clinical features

• Ebsetin’s anomaly of the tricuspid valve is a congeni­tal heart disease in which the tricuspid valve is placed at a lower level so that there is atrialization of the rigFit ventricle.
• A congenital heart condition resulting from downward displacement of the tricuspid valve from the anulus fibrosus. It causes fatigue, palpitations, and dyspnea.
• Abnormal downward placement of tricuspid valve within the RV; tricuspid regurgitation, hypoplasia of RV, and a right-to-left shunt are common
• There is a downward displacement of the tricuspid valve into the right ventricle due to anomalous at­. achment of tricuspid leaflets.

Clinical features  of Ebstein’s anomaly of the tricuspid valve

• Ebsetin’s anomaly of the tricuspid valve cyanosis ay or may not be present.
• ulmonary vascularity is normal or reduced. left ventricle is dominant.
• here may be transient neonatal cyanosis with recur­-ence years later.
• cyanosis is present in the neonatal age infant is achypneic and prognosis is bad.
• there is earl systolic decrescendo murmur of TR best eard in the tricuspid area.
• 3rd and 4th heart sounds are present resulting in a triple or quadruple rhythm.
• left ventricular impulse is seen
• VP is normal
• jrst heart sound is widely split and TI is loud
• there is short mid-diastolic, presystolic murmur.

Ebstein’s anomaly Physical examination —

• The last finding represents passive hepatic congestion resulting from tricuspid regurgitation and elevated right atrial pressure.
• The physical findings vary with the severity of pathology and the magnitude of right-to-left interatrial shunting.
• A systolic murmur from tricuspid regurgitation is a common finding
• When tricuspid regurgitation is severe, jugular venous distension and a prominent “v” wave may be seen

ECG

• shows tall, broad, right atrial P waves in V1, increased PR interval, right bundle block branch dee
• aves in L2, 3, aVF and Vl V2.

 X-ray

• decreased pulmonary vascularity, small aortic t and pulmonary trunk and large right atrium.
• In severe cases, the chest radiograph reveals massive cardiomegaly (often termed a “wall to wall” heart) with diminished pulmonary vascularity
• In a neonate the cardiac silhouette occupies the en­tire chest.
• The chest radiograph may be normal in patients with less severe disease.

2D echo

• Echocardiography shows apical displacement of tricuspid septal leaflet, abnormal RV size, and quantitates degree of tricuspid regurgitation.
• Shows leaflet abnormalities, atrialized right ventricle, paradoxical septal motion, tricuspid regurgitation, enlarged left ventricle and right-to- left shunt via atrial septal defect.

Treatment of Ebstein’s anomaly

• Prosthetic tricuspid valve may be surgically implanted.