Pulmonary atresia with intact ventricular septum

PA/IVS explained

What is pulmonary atresia with intact ventricular septum?

Pulmonary atresia with intact ventricular septum is a condition that causes a blockage between your right ventricle and your lungs. This stops blood flowing from the right side of your heart to your lungs to pick up oxygen.

The blockage happens because:

• The artery from your heart to your lungs (pulmonary artery) does not develop properly.
• The right ventricle has not developed properly and is smaller than normal.
• The valve between the chambers on the right side of your heart (your tricuspid valve) does not develop properly.

Pulmonary atresia with intact ventricular septum is sometimes called PA/IVS or hypoplastic right heart syndrome.

Some people with PA/IVS may have other congenital heart problems like atrial or ventricular septal defects.

Signs and symptoms

What are the signs and symptoms of PA/IVS?

Symptoms of PA/IVS include:

• blue or grey skin on the lips, tongue, mouth or hands
• fast breathing or shortness of breath
• heart murmur.

Symptoms usually appear soon after birth. Changes to a baby’s skin may be harder to see depending on the baby's skin colour.

Diagnosis

How is PA/IVS diagnosed?

PA/IVS may be diagnosed before birth.

An ultrasound or a foetal echocardiogram can be used to diagnose PA/IVS before you’re born. These tests show doctors pictures of the heart. They’re safe for babies. In some cases, PA/IVS is not diagnosed until after the baby is born.

The doctor might perform a test called pulse oximetry. It uses a small painless sensor to check how much oxygen is in the blood.

They may also need to perform one or more of the following tests:

• echocardiogram
• electrocardiogram
• chest X-ray
• cardiac catheterisation.

Treatment

Can PA/IVS be cured?

There is currently no cure for PA/IVS because it’s not possible to repair or create a new right ventricle.

But you can get treatment to help the blood flow better in your heart, treat symptoms and help with other heart conditions.

You may hear the treatments being called “palliative procedures”. This is because they help to manage symptoms, rather than repairing the problem.

There are different treatments available depending on your heart and whether you have other heart conditions. You’ll usually have treatment when you’re a baby or child.

Which medicines are used to treat PA/IVS?

You may be given medicine, called prostaglandins, shortly after you’re born. This helps keep a blood vessel, called the ductus arteriosus, open.

Your ductus arteriosus sends blood to your lungs before you’re born.

Normally this blood vessel closes itself shortly after you’re born. By keeping it open, more blood can get to your body, and it can help while you wait for surgery.

What surgery options are there?

To help improve symptoms and blood flow through your heart you may be offered surgery.

Heart surgery can be scary to consider. It’s important to talk through your options with your specialist doctor.

Because PA/IVS is a complex condition, you may need multiple operations over time to help improve blood flow and help your heart to work.

Surgeries you may have include:

• Valvotomy – to open a blocked valve. It can be done as open heart surgery or minimally invasive surgery (catheter valvotomy).
• Shunt operation – a small tube is put between two main arteries (the aorta and the pulmonary artery). This helps blood flow from the heart to the lungs.
• Pulmonary artery banding (PAB) – a band is placed around the pulmonary artery to slow down blood flow. This helps reduce pressure on the pulmonary artery.
• Septostomy – a hole is created in the wall of the heart (the septum). It helps to increase the amount of oxygen in the blood.

You may also need more surgery to increase the amount of blood going to the body. You may have one or both surgeries:

• Glenn shunt – one of the main veins going back to your heart (your superior vena cava) is connected to the main artery going to your lungs (your pulmonary artery).
• Fontan procedure (or complete TCPC) – one of the main veins going back to your heart (your inferior vena cava) is connected to the main artery going to your lungs (your pulmonary artery).

What if PA/IVS is not treated?

In some cases, you may not wish your baby to have surgery, or treatment may not be possible. This is a difficult decision to come to terms with, and your medical team will be able to discuss options with you and support you.

They will also explain to you what will happen after your baby is born. Your baby would continue to get comfort and care to manage any symptoms. You will be given time together to care for your baby, and hospital staff will support you.

Life with PA/IVS

What will life look like with PA/IVS?

For example, you may have less energy than other people your age or become tired quicker than other people. This can affect things like school, exercise and holidays. Planning your days can help, by breaking up activities and resting regularly.

You’ll also need to speak to your doctor before becoming pregnant in the future. This is so your healthcare team can help support you and your baby.

Will I have a higher risk of endocarditis?

People living with PA/IVS are at a higher risk of endocarditis. Endocarditis is a rare infection that causes inflammation of the valves and inner lining of the heart.

To reduce the risk of developing endocarditis, your doctor might recommend antibiotics before or after surgery.

In some cases, you may need to take antibiotics before you have dental work to reduce your risk of endocarditis. Check with your doctor and dentist to see if this applies to you.

Get support

• If you have questions, speak to our cardiac nurses on our Heart Helpline by phone, callback, email or online chat (Monday to Friday, 9am to 5pm).
• You can also speak to others with heart conditions by joining a support group or online community.
• Little Hearts Matter is a charity for people with single ventricle conditions, like PA/IVS. They offer information, support and services for you and your family.
• Join Little Hearts Matter’s adults support service. For any adult (over the age of 18) living with a single ventricle heart condition in the UK.
• Tiny Tickers can offer you and your loved one's support from pregnancy through to birth and living with your condition.