Tricuspid Atresia

What is tricuspid atresia ?

Tricuspid atresia is a type of congenital heart defect, meaning it is there before birth. It involves the tricuspid valve, which is the valve that allows blood to flow from the right atrium into the right ventricle. Atresia means the absence or abnormal narrowing of a natural opening in the body.

Tricuspid atresia is a single ventricle condition.

Blood normally returns to the heart from the body into the right atrium where it then crosses the tricuspid valve into the right ventricle and is pumped to the lungs to collect oxygen. Those with tricuspid atresia have a tricuspid valve that has not developed properly. This means that blood cannot flow from the right atrium into the right ventricle. 

Growth and development of the ventricles is helped by blood flowing through them and because blood cannot flow through the tricuspid valve, the right ventricle remains small. 

Babies with tricuspid atresia are often born with a hole between the ventricles (VSD). This means that some blood can flow through the hole between the left and right ventricle for the blood to be pumped to the lungs through the pulmonary artery.

They can also receive blood to the lungs through a small blood vessel called the ductus arteriosus. During pregnancy, this structure connects the pulmonary artery to the aorta. Shortly after birth, this vessel usually closes on its own.

1. Tricuspid atresia
2. Atrial septal defect
3. Hypoplastic right ventricle
4. Ventricular septal defect

How can tricuspid atresia be spotted?

Tricuspid atresia can be detected at the 20 week anatomy scan or after birth by echocardiogram.

If it is not picked up in the antenatal period, once the baby is born it will display the following signs:

• Bluish skin colour
• Fast breathing
• Difficulty feeding
• Lethargy (sleepy or unresponsive)

If tricuspid atresia is diagnosed during the pregnancy, plans can be made to give the baby a medicine at birth called prostaglandin which keeps the ductus arteriosus open until the baby can have surgery. 

In most cases, children with tricuspid atresia require intensive medical intervention soon after birth. 

How can tricuspid atresia be treated?

Immediate treatment will be gaining intravenous access after birth and the medicine prostaglandin started to keep the duct open. This will enable blood to reach the body until surgery can take place.

Normally several stages of surgery are required to balance and optimise blood flow to the lungs and the body.

The cardiologist will assess whether:

• There is too much blood going to the lungs
• Not enough blood going to the lungs

If there is too much blood flow to the lungs pulmonary banding will be needed to restrict this excessive flow.

If there is not enough blood flow to the lungs then a Modified BT shunt or a stent will be needed to increase the blood going to the lungs.

An atrial septostomy may be needed to create or enlarge the natural opening between the atria to allow more blood to flow from the right atrium to the left atrium.

Glenn procedure: This surgery is usually done when the baby is around 6 months of age and the surgeons direct the blood flow from the upper body to the pulmonary artery so that the blood can collect oxygen from the lungs without having to go through the heart.

Fontan procedure: This is the third surgery that is usually done when the child is 2 – 6 years old. The surgeons will now be separating the circulation so that no mixing of blood occurs. This also decreases the workload on the single ventricle. This procedure does not create a normal blood circulation in the body but it does improve the circulation.

General advice/info for the future

Babies and children with tricuspid atresia will need lifelong follow-up. Most will be on medicines for the rest of their lives.

They will need repeat tests like echocardiogram, ECG and sometimes cardiac Magnetic Resonance Imaging (MRI) scans. The aim of these tests is to monitor your child’s heart function so that any future heart problems are diagnosed and treated quickly. 

Your cardiologist will advise you about any restrictions on activity that you should be aware of. As complicated as these conditions are, children are encouraged to be as active as they possibly can be. They are encouraged to live as normal a life as they possibly can. As your baby will have had open heart surgery, they will have a scar down the middle of the chest, and there will be small scars where drain tubes were used, but these will fade over time.

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