Lagan's Foundation

Understanding Pulmonary Valve Stenosis in Children

Pulmonary Valve Stenosis is a congenital heart defect that affects the structure of the pulmonary valve in the heart.

Pulmonary Valve Stenosis is a congenital heart defect that affects the structure of the pulmonary valve in the heart.

It is a relatively common heart defect and can range from mild to severe. 

 

 

Key Points:

  • Pulmonary Valve Stenosis is a narrowing of the pulmonary valve, which controls blood flow from the heart to the lungs
  • The outlook for those born with Pulmonary Valve Stenosis is very good, especially if surgery is successful 

What is Pulmonary Valve Stenosis in children?

 

Pulmonary Valve Stenosis (PVS) is a congenital heart defect that occurs when the pulmonary valve becomes narrow, making it difficult for blood to flow from the heart to the lungs. 

 

The narrowing of the pulmonary valve causes the right ventricle to work harder in order to send blood to the lungs. Over time, this can cause thickening of the right ventricle and put a strain on the heart.

What causes Pulmonary Valve Stenosis?

 

Although the exact cause of Pulmonary Valve Stenosis is unknown, the defect occurs when the baby’s heart doesn’t develop the way it should during pregnancy. 

Doctors don’t know why PVS develops, but it is generally understood that it isn’t caused by anything the mother does during pregnancy, meaning it isn’t something that can be prevented. 

The symptoms of Pulmonary Valve Stenosis in children

 

Many babies born with PVS will not show any signs or symptoms, whereas others may show mild symptoms that aren’t usually bothersome. 

 

In many cases of PVS, doctors will detect an abnormal heart murmur, which is a whooshing noise heard in the heart and is usually an indication that something may be wrong. 

 

Other symptoms of Pulmonary Valve Stenosis can include:

  • Becoming very tired 
  • Struggling to gain weight
  • Shortness of breath
  • Chest pains
  • Palpitations
  • Fainting
  • Swollen abdomen

How is Pulmonary Valve Stenosis diagnosed in children?

 

Doctors will often diagnose Pulmonary Valve Stenosis after the baby is born, but in some cases it may be detected before birth. 

After birth, PVS is usually diagnosed through an Echocardiagram. This is a test that creates moving images for the heart and valves, allowing doctors to get a full image of why the heart may not be working properly.

 

Other forms of diagnosis include:

  • Chest X-rays 
  • Electrocardiagram 
  • Cardiac catheterisation
  • Physical examinations 

 

Treatment of Pulmonary Valve Stenosis

 

If your child has mild Pulmonary Valve Stenosis, then they may only need occasional checkups. However, if your child has moderate to severe PVS, then they may need heart surgery soon after they are born. 

The types of surgery can include:

  • Balloon Valvuloplasty – This is a minimally invasive cardiac procedure that helps to widen the narrowed valve to help improve blood flow. A balloon is inserted through a catheter and is inflated to expand the valve. Once it has been inflated, it is deflated and the catheter and balloon is removed.
  • Pulmonary Valve Replacement – If Balloon Valvuloplasty does not work, open-heart surgery or a catheter procedure may be conducted to replace the pulmonary valve.

If there are other heart problems, the surgeon will also repair those in the same surgery. 

 

What is the life expectancy of a child with Pulmonary Valve Stenosis?

 

The life expectancy for children with Pulmonary Valve Stenosis who have been treated is generally good, with many children going on to live happy, healthy and long lives after surgery. 

In the long term, children are unlikely to have any symptoms at all and will continue to grow and develop just like other children their age. 

Some children with severe PVS may need to have repeat balloon procedures as they grow, but thanks to the advancement in medical science and technology, the outlook for these children is still very positive.  

 

Are there other conditions associated with Pulmonary Valve Stenosis in children?

 

Yes, Pulmonary Valve Stenosis is sometimes associated with other conditions, including other congenital heart defects such as Tetraology of Fallot, Atrioventricular Septal Defects and Ventricular Septal Defects. 

PVS is also more common in children who are born with rare genetic conditions such as Noonan Syndrome and Williams Syndrome. 

 

What happens as your child grows up after having Pulmonary Valve Stenosis?

 

After your child has had Pulmonary Valve Stenosis, they will likely need to have regular health checks with a paediatric cardiologist throughout their childhood. The outlook for children who have had PVS is very positive, with many children going on to live healthy, normal lives. 

In some cases, your child may need to take antibiotics to prevent infections from forming in the heart lining or valves, however, your child’s doctor will guide you and discuss this with you if this is the case.