Pulmonary Atresia (PA)

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What is Pulmonary Atresia (PA)

Pulmonary means ‘of the lungs’.
Atresia means absent.

Pulmonary atresia is an extremely rare form of congenital heart disease inwhich the pulmonary valve does not form properly. The pulmonary valve isa flap-like opening on the right side of the heart that allows blood to moveto the lungs.

In pulmonary atresia, a solid sheet of tissue forms where the valve opening should be. Because of this defect, blood from the right side of the heart cannot go to the lungs to pick up oxygen.Before birth, while the fetus is developing, this actually is not a threat to life, because the placenta provides oxygen for the baby and the lungs are not functional. Blood entering the right side of the fetal heart passes through an opening called the foramen ovale that allows oxygen-rich (red) blood to pass through to the left side of the heart and proceed to the body.

Causes

As with most congenital heart diseases, there is no known cause ofpulmonary atresia. The condition is associated with another type ofcongenital heart defect called a patent ductus arteriosus (PDA).

What are the symptoms of pulmonary atresia?

Symptoms will be noted shortly after birth. The obvious indication of PA is a newborn who becomes cyanotic (blue) in the transitional first day of life when the maternal source of oxygen (from the placenta) is removed. The degree of cyanosis is related to the presence of other defects that allow blood to mix, including a patent (open) ductus arteriosus.

The following are the most common symptoms of pulmonary atresia. However, each child may experience symptoms differently. Symptoms may include:

  • rapid breathing
  • difficulty breathing
  • irritability
  • lethargy
  • pale, cool, or clammy skin

The symptoms of pulmonary atresia may resemble other medical conditions or heart problems. Always consult your child’s physician for a diagnosis.

Diagnosis

If pulmonary atresia is diagnosed before birth, you may have other tests, as this condition is commonly seen in children with a 22q11.2 deletion (Di George Syndrome).

After birth, your baby can survive while the foetal circulation system (ductus arteriosus) is still open. While the ductus arteriosus is still open, blue blood will flow through it into the pulmonary artery, and thus to the lungs.

As the foetal circulation closes down, however, your baby will become increasingly blue (cyanosed) and have difficulty breathing and feeding. Most babies will need treatment within the first days or months of life.

When a heart condition is suspected the tests used can be:

Treatment

The treatment for pulmonary atresia is palliative. This means that your child’s heart cannot be corrected, that is, made to work like a normal heart, but it can be improved (palliated).

Medicine

A baby may be given medicine to keep the foetal circulation open a little longer. This allows some blood to go on being pumped from the right side of the heart to the left, and then from the left ventricle to the lungs through the duct.

BT Shunt

Your child may need a shunt (connection) to increase the amount of blood getting to the lungs. This is done by connecting a tube from an artery in the arm to the pulmonary arteries. This operation may be carried out through the side of the chest, while the heart is beating, or through the front using a heart lung bypass machine (open heart surgery). The increased blood flow should help the ulmonary arteries to grow larger. The shunt will be made of a synthetic material called Gore-Tex, will be inserted between the pulmonary artery and the aorta or their branches to ensure continued blood flow to the lungs. This is known as a modified Blalock-Taussig Shunt, (BT shunt).

Balloon septostomy

Another way of increasing blood to the lungs is to make the ASD, which was part of the foetal circulation system, bigger. This involves threading a fine tube – a catheter – through a vein into the heart and through the hole between the atria. A balloon on the end is then inflated and pulled back to make the hole bigger. Blue (deoxygenated) blood will then pass from the right atria to the left, into the left ventricle, from where it will be pumped into the aorta, the ductus arteriosus, and so to the lungs.

Radiofrequency perforation of the pulmonary valve

In some cases it may be possible to burn a way through the blocked pulmonary artery. A fine tube is inserted into a vein and passed through the heart. A hot wire is used to burn into the pulmonary artery. A balloon is then inserted and used to stretch the pulmonary valve and artery. This does not leave any scar.

Surgery

A series of operations may be necessary to bypass the blocked pulmonary artery. These are open heart surgeries – the heart will need to be stopped and opened to repair it. This means that a machine will have to take over the job that the heart normally does – the heart bypass machine.

The Fontan procedure is often used in cases on PA.

Also the Glenn shunt may be used. Although not exactly a Fontan operation, a bidirectionalGlenn shunt is similar to it and is called a type of “Partial Fontan”or “Hemi Fontan” operation. The bidirectional shunt is performed by connecting the superior vena cava (SVC) to the right branch of the pulmonary artery using fine sutures, and dividing or tying up thepulmonary artery. Now, venous blood from the head and upper limbs will pass directly to the lungs, bypassing the right ventricle. The venous blood from the lower body however will continue to enter the heart.
Rastelli operation and unifocalisation of the pulmonary arteries

Some children with pulmonary atresia and ventricular septal defect may undergo a number of staged operations to connect up the collateral arteries (MAPCA’s) – which supply blood to the lungs – back to the heart. Eventually it may be possible to connect the right ventricle using a homograft (human valve) to the lung arteries, and to close the ventricular septal defect. This will allow all the blue blood to be passed to the lungs and the red oxygenated blood to pass to the body.

Risk of procedures

There is a risk to your child in all the procedures, but how great that risk is depends on how severe the heart defect is, and how well your child is otherwise. The doctors will discuss risks with you in detail before asking you to consent to any of the operations.

How the child is affected

Treatment of pulmonary atresia can be very complicated, so it is difficult to judge how well your child will do during and after surgery.
Most children will be much better after treatment. Scars on the chest fade very rapidly in most children, but they will not go altogether. Smaller scars on the hands and neck usually fade away to nothing. Many children with this condition will continue to have a bluish tinge to their skin (cyanosis).
You may find that your child has other health problems associated with the heart condition or with a syndrome. Seek advice about getting Disability Living Allowance (DLA) if your child needs much more care than a child of his or her age, or is having problems walking.

Some of the following problems can occur after surgery or later in life:

  • Fluid on the lungs, chest infections or infected wounds are common problems after surgery.
  • Your child’s valves and shunts may need further surgery as they get older and bigger.
  • If the pulmonary valve has been unblocked, it is common for it to need to be replaced. This is usually with a homograft valve (human tissue). This can sometimes be done using a catheter rather than open heart surgery.
  • The electrical system of the heart is sometimes damaged after surgery, causing a very fast heart beat (called tachycardia), which may need medication to keep it stable.

If your child had an operation some years ago, they may have outgrown it and thus need further surgery.

Related Links

http://www.fontanoperation.com/fontan.htm
http://www.pted.org/?id=pulmonaryatresia1
http://www.nlm.nih.gov/medlineplus/ency/article/001091.htm
http://www.americanheart.org/presenter.jhtml?identifier=1303
Amanda, obtained her information from the various links mentioned above.

Disclaimer: The facts and opinions shown in this article are as accurate and up to date as possible, but are provided as general “information resources”, which may not be relevant to individual persons. This article is not a substitute for individual assessment and always take advice from a doctor who is familiar with the particular person.

Consult you or your child’s physician regard the specific outlook for you or your child.

Wrote by Amanda Prenctice
November 2009