Pulmonary Atresia
What is Pulmonary Atresia?
Pulmonary atresia is a defect of the pulmonary valve.
Your pulmonary valve usually controls the blood flow from the lower right chamber of the heart to the main pulmonary artery (the blood vessel that carries blood from the heart to the lungs).
Pulmonary atresia occurs when this valve doesn’t form, and no blood can go from the right ventricle of the heart out to the lungs.
Most babies with pulmonary atresia will need medication to keep the ductus arteriosus open after birth.
Keeping this blood vessel open will help with blood flow to the lungs until the pulmonary valve can be repaired.
Pulmonary atresia is a life-threatening condition that needs emergency treatment.
What are the types of pulmonary atresia?
There are typically two types of pulmonary atresia, determined by whether or not a baby also has a ventricular septal defect (VSD). A VSD is a hole in the wall that separates the two lower chambers, or ventricles, of the heart.
Pulmonary atresia with an intact ventricular septum
In this type of pulmonary atresia, the wall – or septum – between the ventricles remains complete and intact. During pregnancy when the heart is developing, the intact septum restricts blood flow into or out of the right ventricle, meaning the right ventricle doesn’t fully develop and remains very small. When the right ventricle is under-developed, the heart can have trouble pumping blood to the lungs and the body.
Pulmonary atresia with ventricular septal defect
In contrast to pulmonary atresia with an intact ventricular septum, in this type of pulmonary atresia, there is a hole in the septum between the ventricles of the heart.
Under normal circumstances, blood passes through the pulmonary artery to the lungs, to collect oxygen. In babies who have pulmonary atresia with a VSD, blood cannot pass to the lungs in this way. Instead, the blood passes from the aorta to the pulmonary artery through a connection called the ductus arteriosus – often called ‘the duct’.
What are the symptoms of pulmonary atresia?
Symptoms of pulmonary atresia may present very soon after birth. These symptoms can include:
- Blue or gray skin, lips or fingernails due to low oxygen levels
- Fast breathing or shortness of breath
- Tiring easily
- Not feeding well
What treatment options are there?
Treatment can include:
Cather-based procedures:
Balloon atrial septostomy: A balloon is used to enlarge the natural hole in the wall between the upper chambers of the heart. This hole, called the foramen ovale, most often closes soon after birth. Making the hole larger lets blood move easily from the right side of the heart to the left side.
Stent placement: A doctor may put a rigid tube called a stent in the ductus arteriosus to prevent it from closing. This keeps blood flowing to the lungs.
Babies with pulmonary atresia often need many heart surgeries over time. The type of heart surgery depends on the size of the child’s lower right heart chamber and pulmonary artery.
Types of surgery for pulmonary atresia:
Shunting: This involves making a new route for blood to flow, called a bypass shunt. The shunt goes from the main blood vessel leading out of the heart, called the aorta, to the pulmonary arteries. This lets enough blood flow to the lungs. Most babies outgrow this shunt within a few months.
Glenn procedure: In this surgery, one of the large veins that returns blood to the heart is joined to the pulmonary artery. Another large vein keeps blood flowing to the right side of the heart. The heart then pumps it through the pulmonary valve that’s been repaired. This can help the right ventricle grow.
Fontan procedure: If the right lower heart chamber stays too small to do its work, surgeons may use this procedure to make a pathway. The pathway lets most, if not all, of the blood coming to the heart to flow into the pulmonary artery.
Heart transplant: In some cases, the heart is too damaged to fix, and a heart transplant may be needed.
If the baby also has a ventricular septal defect (VSD), surgery is done to patch the hole. Then the surgeon makes a connection from the right pumping chamber to the pulmonary artery. This repair may use an artificial valve.
