Pulmonary Atresia with Intact Ventricular Septum

This is a serious heart condition in which there are two main abnormalities:

  1. the artery which normally allows blood to be pumped from the heart to the lungs(the pulmonary artery) is completely blocked
  2. the main pumping chamber on the right side of the heart is small.

In the normal circulation, blood passes through the lungs to collect oxygen from the air. In babies with pulmonary atresia not enough blood passes through the lungs. This makes the blood passing round the body short of oxygen. The baby (particularly the lips and hands) appears blue, and if the body becomes very short of oxygen the baby will die.

Before babies are born the two main arteries arising from the heart (one called the the aorta, which feeds the body with blood and one called the pulmonary artery, which feeds the lungs with blood) are joined together by a short blood vessel called the arterial duct (often just called the duct). Most babies with pulmonary atresia appear well for a while because the duct allows the lungs to be supplied with blood from the aorta.

In normal babies the duct closes off by itself shortly after birth because it is no longer required, but if the duct closes in a baby with pulmonary atresia the baby will become very blue and will die because no blood can get to the lungs. The duct can usually be prevented from closing by giving the baby a drug called prostin. Most babies with pulmonary atresia will need an operation within the first few weeks of life to improve the blood supply to the lungs

Usually only a simple test such as an ultrasound scan of the heart (an “echocardiogram”) is required, although some babies will need more complicated Xray tests (called a cardiac catheterisation or CT scan).

The type of operation needed will depend upon the exact abnormality present. In some babies the pulmonary valve is present but there is no way through it. In this case it is sometimes possible to open the valve using “keyhole” treatment.

This is done by burning a small hole through the valve with a thin wire with a heated tip. The wire is passed through a small tube which is put in through the vein at the top of the leg and positioned underneath the blocked valve. Once a small hole is burned through the valve a balloon is blown up in the hole to make it larger, stretching the valve open. If this does not prove possible it may be possible to surgically cut the valve open (called a “valvotomy”).

In some babies it may not be possible to open up the blocked valve, or opening it up may not allow enough blood to get to the lungs. In these cases the blood supply to the lungs can be helped by a “shunt” operation. This involves connecting the artery leading to one of the arms to the artery which leads to the lungs. This doesn’t put
the baby’s heart back to normal, but it usually allows the baby to survive until he or she is old enough to consider more major surgery.

What are the risks of the procedure?
All heart operations in babies are serious and will carry some risk of the baby dying or having major complications such as brain damage, kidney damage or lung complications such as pneumonia or fluid collecting around the lungs. For shunt operations the risk of a baby dying is small (about 2 in 100). These risks are higher if the baby has other abnormalities or if the baby has been born prematurely.

Further surgery when the baby has grown
All children with pulmonary atresia who have had a shunt operation as a baby will need further treatment when they are older. Exactly what this treatment will be depends very much on how the baby grows. Some children who have only had the valve opened up may also need further operations later, but some may not. Even many years after apparently successful surgery new problems may arise which require treatment, so it is important to attend the outpatient clinic.

General advice for the future
Some children with heart disease cannot manage as much physical exercise as a normal child. We do not usually stop children with heart disease from doing exercise. You will be told if there are specific forms of exercise your child should avoid.

All patients with pulmonary atresia will be at risk of infection in the heart (called endocarditis), both before and after surgery. Such infections may be caused by infections of the teeth or gums. It is important to look after your child’s teeth and visit the dentist regularly. Ear or body piercing and tattooing are best avoided as they also carry a small risk of infection which may spread to the heart.

Written in July 2008, updated October 2013 by the Paediatric Cardiology Team.