Monthly Archives: Mar 2003

Hypoplastic Left Heart Syndrome – HLHS

Hypoplastic Left Heart Syndrome - HLHS

The “Hypoplastic Left Heart Syndrome” – or more conveniently, “HLHS” – is a unique defect of the heart in many ways. Until a decade ago, the diagnosis was a virtual death sentence. Today, the revolution in cardiac surgical thinking and technique has changed the situation radically.

Whereas survival beyond the first few months of life was previously unheard of, many centers are today reporting encouraging results. And it is bound to improve further as more knowledge is gained from the early experience.

What is the HLHS ?

The heart has two upper and two lower chambers – one of each is right sided and the other left sided. The left sided chambers, with their blood vessels and valves are sometimes referred to as the LEFT HEART. ( This does not mean that the person has TWO hearts! )

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Ebstein’s Anomaly

Ebstein's Anomaly

What is Ebstein’s anomaly ?

It was in 1866 that Ebstein first described the heart defect that bears his name. The defect includes the following components:

  • Displacement of the tricuspid valve (that lies between the right atrium and right ventricle) leaflets downwards from it’s normal position.
  • Abnormalities of the right ventricle
  • “Atrialization” of a portion of the right ventricle
  • An inter-atrial septal defect (ASD)

What happens in Ebstein’s anomaly ?

The primary problem is displacement of the tricuspid valve down from the normal position, which in turn results in the abnormal valve becoming leaky. This valve leak, combined with right ventricle muscle abnormalities, causes heart enlargement with heart failure, along with a “shunt” of blood from the right atrium, across the ASD, into the left side of the heart and circulation.

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