We will use this page to try and explain the diagnosis arrived at by our fetal cardiac Doctor. We are not medical people so the information on this page and all other pages on the site are our understanding of what we have been told.

After our first scan at the Heath in Cardiff, the Doctor explained what he thought the problems were and the treatment that would be necessary to correct them. The main thing to point out here is that with current techniques (excluding heart transplant) it is not possible to make the heart function normally, the best we can hope for is to improve the circulation.

We will try to give an interpretation of how a normal heart functions before we explain how our baby's heart is working. 

Normal Heart Operation

Blood comes into the heart from the body via veins into the right atrium, this blood is passed to the right ventricle through the tricuspid valve. It is pumped from the right ventricle through the pulmonary artery to the lungs where it collects oxygen and turns red. The red blood then goes back to the heart via the pulmonary veins into the left atrium. It passes through the mitral valve into the left ventricle. From the left ventricle it pumped back to the body via the aorta. The body uses the oxygen in the blood turning it blue and passes it back to the heart where the whole cycle starts again.

For more realistic diagrams click here

Our Doctor feels that our baby has no (or very small and unusable (hypoplastic)) right ventricle and no tricuspid valve. There are two atriums as normal but there is no dividing wall between them. The Pulmonary artery and the aorta are both coming from the left ventricle which is much bigger than normal. There is also a narrowing of the pulmonary artery (Pulmonary Stenosis)

Some of the scans used by our Doctor

Implications and corrective procedures.

The main implication is that our baby will never have a strong blood supply to the lungs which until the third operation (detail later) will mean he will be a "Blue" baby as there isn't a lot of oxygen in the blood. This will mean that he tires more quickly than other children of the same age. It shouldn't have any adverse effects on growth or mental development. He will need to be on medication all of his life, to keep the blood thin to prevent clotting. This medication will also mean that if cut he will bleed for longer than is normal which in turn means that he won't be able to take part in contact sports like rugby or karate.

All of the corrective procedures are designed to increase the blood flow across the lungs thereby increasing the oxygen

The first procedure is called the Shunt operation and takes place within the first few weeks, sometimes days

 All babies when born have a small tube that goes from the aorta (body artery) to the pulmonary artery (lung artery) this tube increases blood supply to the lungs. However this tube dissolves after about a week, the shunt operation replaces the natural tube with an artificial one.

The second procedure is called the Glenn Operation and takes place between 9 and 18 months

Normally the body vein (vena cava) feeds into the right atrium from bottom and top. The Glenn operation splits the vein and feeds the top part (Superior Vena Cava) into the right lung artery while the bottom vein (inferior vena cava) still feeds into the right atrium.

The third procedure is called the Fontan operation and takes place between 2 and 6 years

This operation adds a tube to the bottom body vein (inferior vena cava) to meet the pulmonary artery in the same place as the Glenn operation. This leaves some of the body blood going to the right atrium an the rest going to the lung to collect oxygen.

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