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Congenital Heart Defects: Ebstein's 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-Wide Syndrome (WPW Syndrome)
One very important condition which is associated with Ebstein Anomaly is a heart arythmia which is called Wolf-Parkinson-Wide Syndrome (WPW Syndrome) which disposes the heart to beat irregularly very fast and produce a condition called Supra Vetricular Tachycardia (SVT).

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


Babies with this condition usually appear blue at birth, have difficulty breathing, and difficulty with feedings, and may require immediate surgical attention.

The chest x-ray of a child with Ebstein Anomaly shows a very large heart and also shows that the lungs are lacking blood.

Difficulty with feedings, labored breathing, enlarged heart in chest x-ray is what constitutes congestive heart failure in children with this condition.


Problems with the Walls:
Atrial Septal Defect (ASD) : Fix | Ventricular Septal Defect (VSD) : Fix

Problems with the Doors:
Tricuspid Valve Atresia : Fix 1 | Fix 2 | Fix 3 | Ebstein's Anomaly
Hypoplastic Left Heart Syndrome : Fix 1 | Fix 2 | Fix 3
Pulmonary Valve Stenosis
| Aortic Valve Stenosis

Problems with the Chambers:
Hypoplastic Right Ventricle

Problems with the Hallways:
Coarctation of the Aorta : Fix | Branch Pulmonary Artery Stenosis
Transposition of the Great Arteries : Fixed
Total Anomalous Pulmonary Venous Connection

Problems with the Plumbing:
Anomalous Origin of the Coronary Arteries from the Pulmonary Artery

Problems Involving More Than One Part of the Heart:
Tetralogy of Fallot : Fixed
Truncus Arteriosus : Fixed | Common Atrioventricular Canal : Fix 1 | Fix 2

Ductus Arteriosus : Fix 1 | Fix 2

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The next defect on the Problems with the Doors tour is Hypoplastic Left Heart Syndrome.

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