Single Ventricle

What is the heart defect called Single Ventricle ?

While the normal heart has two ventricles, in some birth defects, one of these ventricles may be absent or poorly developed. This condition is called Single Ventricle or Univentricular heart.

The ventricular structure may resemble the normal left ventricle or the normal right ventricle. Sometimes, it resembles neither, and this is called Indeterminate ventricle morphology.

In a single ventricle heart, there are two normal atria – right and left. These open into the ventricle through an atrio-ventricular (AV) valve. There might be
two AV valves, both opening into the ventricle – a condition called Double Inlet Ventricle – or one AV valve only, with the other one being absent (Atresia)

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Tricuspid Atresia – TA

Tricuspid atresia and the Fontan principle are rather complex congenital heart defects. So if you can’t figure out the condition even after reading this article, please understand that this anomaly is so complex that even cardiologists have trouble understanding its repair.

So if you don’t, console yourself that you are in distinguished company!

What is tricuspid atresia ?

Triscupid Atresia is a condition where the Tricuspid Valve, which guards the junction between the right atrium and the right ventricle, is either absent or is imperforate – that is, it does not have an opening to allow blood flow across it. There are many ways the valve can be imperforate – the leaflets of the valve may be formed but tightly stuck to each other, or may not be formed at all, with muscle tissue of the heart forming a wall where the valve should have been.

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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

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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Double Outlet Right Ventricle – DORV

What is Double Outlet Right Ventricle (DORV) ?

Some of the defects I have described are “simple”, some are a little “complex” – but DORV is something else.

It is a common term that actually describes a wide spectrum of heart disease, ranging from something similar to a Ventricular Septal Defect (VSD), through Tetralogy of Fallot (ToF) to Transposition of the Great Arteries (TGA).

It is sometimes like one, at other times like another, and occasionally a mixture of some of them. So if at first you are baffled, don’t worry. I too was, and figured it out only after a long hard struggle.

What is Double Outlet Right Ventricle ?

Normally, a ventricle has just ONE outlet. For the left ventricle, this is the aorta. For the right ventricle it is the pulmonary artery. In DORV, both of these “outlet” blood vessels – aorta and pulmonary artery – arise from the RIGHT VENTRICLE, either totally or to a great extent.

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