Understanding Tricuspid Atresia

I’m not sure about you, but until recently I knew very little about how one of the most important organs in our body actually functions!

If that description fits you too, here’s a diagram from The Royal Melbourne Children’s Hospital that shows how a normal heart works:

Normal heart
There are four chambers in the heart with the right side pumping non-oxygenated blood through the lungs which comes back to the left side of the heart oxygenated and ready to be pumped to the rest of the body.

Our baby has been diagnosed with a condition called Tricuspid Atresia. There’s a lot of information about it on google – and we’ve found some helpful, some fascinating, but most a bit overwhelming.

The following information comes from our discussions with the baby’s specialists, along with websites that they recommended to us. I’ll focus on tricuspid atresia, but for more information about Congenital Heart Disease (CHD) in general, have a look at the HeartKids NSW website.

Tricuspid Atresia

Tricuspid atresia is a congenital (i.e. present at birth) heart condition that affects approximately 1 in 10,000 babies born in Australia every year.

It is where the right side of the heart has not developed properly and there is no tricuspid valve, severely restricting blood flow to the lungs. Blood that returns from the body to the right atrium of the heart cannot directly enter the right ventricle, and must pass through a hole in the atrial septum into the left atrium and the left ventricle.

Here is a comparison of a normal heart and one with tricuspid atresia, also from The Royal Children’s Hospital Melbourne:

Normal heart

As for the causes? Recent research suggests 20% of CHD can be attributed to chromosomal abnormalities – with a common link to Down Syndrome (not likely in our case). However, for the vast majority of cases (80%) the cause is unknown, the condition has occurred during the baby’s early development.

We’re thankful for recent news that funding grants have been allocated to the Victor Chang Cardiac Research Institute for genome mapping to try and identify which gene is responsible for CHD.

Treatment

Have you ever wondered how a baby’s lungs and heart work while they are in utero and don’t need to breathe? I don’t think it had ever occurred to me before our CHD diagnosis.

A pretty cool feature of the human body is that while a baby is still in the womb, they have a vessel which recirculates blood around the heart and to the placenta which does the job of the lungs before birth. This vessel, called the ‘ductus arteriosus’ naturally closes in the first few days after the baby is born, because the baby begins to receive oxygen from the lungs.

However, for our baby the first step in treatment is to keep the duct open, which is first done by giving the baby antibiotics straight away after birth.

This gives the doctors time to work out what the heart actually looks like (done through a series of scans, including an echocardiogram), so that they can determine the next steps in treatment.

What follows is the generally expected plan for treating tricuspid atresia. But it’s also important to remember that every baby is unique and so this might not be how things pan out for our baby!

There is a likely three step plan for surgery:

  1. Blalock-Taussig Shunt Operation before 2 weeks old – this first operation involves inserting an artificial tube between the two sides of the heart, made from goretex material. After which the baby will spend 4-6 weeks in hospital recovering and being monitored.
  2. Bidirectional Cavo Pulmonary Connection (BCPC) at 4 to 6 months old – this operation is performed to connect a main vein from the upper part of the body to the lung circulation.The purpose of this operation is to improve the level of oxygen in the baby’s blood.
  3. Fontan Procedure at 3 to 5 years old – the final, and most permanent operation is the Fontan procedure. By connecting one of the veins directly to the pulmonary artery, all blood returning from the body passes through the lungs and picks up oxygen before being pumped to the body. This allows a higher level of oxygen saturation in the blood.

Surgeons have been successfully performing these operations for a number of decades. Long term quality of life is generally good, however further complications might require ongoing medication or a pacemaker.

I hope that gives you a general understanding of our baby’s condition, if you’d like to know more here are some of the websites that were recommended to us:

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5 thoughts on “Understanding Tricuspid Atresia

  1. Dear Sarah,
    It is amazing that little Baby Cameron is safely snuggled into Mummy, growing stronger by the day . Our previous neighbour has worked with Dr Richard Chard and once again confirmed,that you are in excellent hands.
    Give little Bubba a pat from me and tell him/ her to stay cosy for a while longer.
    Lots if love to you all . Aunty Jx

    Like

    1. Your positivity and reassurance is inspiring! Oh Sarah and Gus you’ve kept such a cool head during everything! I am truely in awe of you guys. 3 operations required for Hannah is much better than I thought. And you’re already one down! Or is it 4 operations? Another one two weeks after birth? So in another week and a bit?
      I’ll continue to read but what great info thanks for sharing!

      Like

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