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Ebstein Anomaly

Ebstein Anomaly is a congenital heart defect characterized by displacement of the tricuspid valve. Since the tricuspid valve is not in its usual location, and is further displaced downward in the right ventricle towards the muscle.

In this condition some of the right ventricle muscle is lost and is replaced by a fiborus thin wall. Therefore the contraction of the right ventricle is affected to the degree of that displacement - in other words the more the wall of the tricuspid valve is displaced towards the apex of the right ventricle the more that ventricle will be weak.

The tricuspid valve will be very incompetant and will leak backwards. Therefore the right atrium will be very large and the right ventricle will be very small. Since the right ventricle is small not enough blood will be going through the pulmonary artery to the lungs so the baby will be blue (cyanosis).

The operation for this condition is very complex and involves reconstruction of the doors of the tricuspid valve to make it functional and prevent the leak.

Bundle of Kent

Ebstein Anomaly is associated with the presence of an electric connection between the right atrium and right ventricle via a bundle called Bundle on Kent. This makes the electric impulse bypass its usual course. This predisposes the child to have a condition called Wolf Parkinson White Syndrome (WPW) and Supraventricular Tachycardia.

Wolf-Parkinson-White Syndrome (WPW Syndrome)

One very important condition which is associated with Ebstein Anomaly is a heart arrhythmia which is called Wolf-Parkinson-White Syndrome (WPW Syndrome) which disposes the heart to beat irregularly very fast and produce a condition called Supra Vetricular Tachycardia (SVT).

This arrhythmia may be very difficult to control especially in this condition and can sometimes require multiple, sometimes lots, of medications.

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Children's Heart Institute

P. O. Box 10066 McLean, VA 22102

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phone - 703-481-5801

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