Hello and welcome to the Zero to Finals podcast. My name is Tom and in this episode

I'm going to be talking to you about Ebstein's Anomily. And if you want to follow along with

written notes on this topic, you can follow along at zero tofinals.com slash

Ebsteins or in the cardiology section of the zero to finals pediatrics book. So let's get straight into it.

Epstein's Anomaly is a congenital heart condition where the tricuspid valve is set lower in the right side of the heart

towards the apex of the heart and this causes a bigger right atrium and a smaller right ventricle.

This leads to poor flow from the right atrium to the right ventricle,

and therefore poor flow to the pulmonary vessels.

It's often associated with a right to left shunt across the atria via an atrial septal defect.

When this happens, blood bypasses the lungs and leads to cyanosis.

Epstein's anomaly can also be associated with Wolf Parkinson-White syndrome.

So how does it present?

The typical presenting features include evidence of heart failure, for example, edema, a gallop rhythm heard on

auscultation, cyanosis if there's an associated atrial septal defect, shortness of breath, and

tachycneal, which is a raised respiratory rate, poor feeding, and potentially collapse or cardiac arrest.

Symptoms in patients who have an associated atrial septal defect often present a few days

after birth when the ductus arteriosis closes. Where there's a right to left shunt across

the atrial septal defect,

the ductus arteriosus allows blood to flow from the aorta into the pulmonary vessels to get oxygenated.

And this minimizes the cyanosis.

However, when the duct closes,

the patient then loses the benefit of having the ductus arteriosis

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