Tests for heart and circulatory conditions
Tests are used to diagnose a heart condition or to see how healthy your heart is. Find out what to expect from some of the most common tests.
Hypoplastic left heart syndrome explained
Hypoplastic left heart syndrome is when the left side of your heart does not develop properly. This includes problems such as:
- The left-hand lower chamber (left ventricle) is smaller than normal.
- The valves on the left side of your heart may be narrow, blocked or not formed at all.
- The main blood vessel (your aorta) can be smaller.
Sometimes, you may also have a hole between the top chambers of your heart (atria) called an atrial septal defect.
These problems mean that blood does not flow through your heart properly. Because the left side of your heart is not properly formed, it cannot pump blood high in oxygen to your body.
Blood high in oxygen must find another way to get to your body so it will enter the ductus arteriosus. This is a tube between the artery to your lungs and the main artery in your heart (aorta). The ductus arteriosus usually closes shortly after you’re born so doctors will give you medicine to keep it open.
The hole in your heart can cause blood low in oxygen and blood high in oxygen to mix. This means the body gets both types of blood. Your heart will have to work harder to supply blood high in oxygen to your body.
How a normal heart works
The way blood flows through your heart is important.
Right side of your heart
- Blood low in oxygen comes from your body to your heart. It enters the top chamber (your atrium).
- The blood goes through a valve into the bottom chamber (your ventricle).
- Your ventricle pumps the blood to your lungs to get oxygen.
Left side of your heart
- Blood high in oxygen comes from your lungs back to your heart. It enters the top chamber (your atrium).
- The blood goes through a valve into the bottom chamber (your ventricle).
- Your ventricle pumps the blood to your body.
Your heart valves act like doors, opening so blood can flow through the heart and closing to stop blood from going backwards.
Symptoms of hypoplastic left heart syndrome
Babies or young children can show symptoms of hypoplastic left heart syndrome including:
- a blue colour to your skin (cyanosis)
- feeling breathless
- not eating or feeding well
- feeling very tired (fatigue).
Speak to your doctor if you notice any new symptoms or symptoms that are getting worse.
Diagnosing hypoplastic left heart syndrome
Hypoplastic left heart syndrome will be diagnosed before you’re born or very soon after.
An ultrasound or a foetal echocardiogram can be used to diagnose hypoplastic left heart syndrome before you’re born. These tests show doctors pictures of the heart. They’re safe for babies.
Sometimes hypoplastic left heart syndrome cannot be seen on an ultrasound due to the size or position of the baby.
Babies born with hypoplastic left heart syndrome will show symptoms. They’ll usually have an echocardiogram and urgent treatment at a specialist unit or hospital.
Causes of hypoplastic left heart syndrome
We do not know why hypoplastic left heart syndrome happens.
Not knowing what has caused your condition can be worrying and frustrating. It can help to find out more about the causes of congenital heart disease.
Treatment for hypoplastic left heart syndrome
There is currently no cure for hypoplastic left heart syndrome because it’s not possible to repair or create a new left 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.
Here are some common treatments:
Medicine when you’re born
You may be given medicine 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.
Surgery
To help improve symptoms and blood flow through your heart you may be offered surgery. The surgery can re-route blood through the right side of your heart.
You’ll usually be offered surgery in 3 main stages:
- as a baby shortly after you’re born
- as a baby or young child
- as a young child, either before you start school or when you’re a bit older.
Heart surgery can be scary to consider. It’s important to talk through your options with your specialist doctor.
Because hypoplastic left heart syndrome is a complex condition, you may need multiple operations over time to help improve blood flow.
Types of surgery
The first operation you’ll usually have is called a Norwood procedure.
It involves connecting your lung artery (pulmonary) to your main blood vessel (aorta) to increase the amount of blood to your body. Surgeons will also create a permanent hole or pathway between the top chambers of your heart to help blood high in oxygen reach the aorta better.
You may be offered other types of surgery, such as:
- Modified Norwood procedure – your lung artery (pulmonary) is connected to your main blood vessel (aorta) to increase the amount of blood to your body. Your right ventricle also is connected to your lung artery using a tube to help blood reach your lungs.
- Hybrid procedure – a blood vessel called the ductus arteriosus that normally closes after birth is surgically kept open to help more blood get to your lungs. The surgeon may also create a hole, or make a hole bigger, between the top chambers of your heart.
- Damus procedure. – the base of your lung artery (pulmonary) is connected to the base of your main blood vessel (aorta) to increase the amount of blood to your body. These blood vessels will also be connected further up to help blood flow.
Find out more about these surgeries on the Little Heart’s Matter website.
You will also need more surgery to increase the amount of blood going to the body. You may have one or both of these 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).
Both procedures mean blood low in oxygen goes straight to your lungs instead of through your heart. More blood can get to your lungs to pick up oxygen and your heart does not have to work as hard.
As you get older you may need more surgery, including a heart transplant. Your doctor can tell you more about this.
Support with symptoms
Your doctor and specialist team will give you support with any symptoms you have. This may include medicines to help your heart work better.
Speak to them if you have any new symptoms or symptoms that are getting worse.
Deciding not to have treatment
In some cases, you may not wish your baby to have surgery. This is a difficult decision to make, and your medical team will be able to discuss options with you and support you.
They will also explain to you what would 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.
Managing hypoplastic left heart syndrome
People with hypoplastic left heart syndrome who have treatment are living longer due to advances in treatment and research people are living longer with this condition. However, it’s important to understand your condition and any adjustments you may need to make to your daily life to help manage it.
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.
It’s also important to go to your appointments, even if you feel well. Your doctors will check how your heart is working and can help answer any questions you have.
Endocarditis
Endocarditis is a rare infection that causes inflammation of the valves and inner lining of the heart.
People with hypoplastic left heart syndrome may have an increased risk of endocarditis.
To reduce the risk of developing endocarditis, your doctor or dentist might recommend antibiotics before or after any surgery or procedures.
Planning ahead
You may hear hypoplastic left heart syndrome being called a life-limiting condition. This means that there is no cure, but you can have treatment to help manage symptoms.
Because of this you may want to speak to your treatment team about what happens as you get older. This includes palliative care.
Thinking about the future and your care can feel scary and overwhelming. But it can help make sure you get the right support when you need it.
If you need support having difficult conversations Marie Curie can help you. You can also find support from Little Heart’s Matter and by calling our nurses on the Heart Helpline.
More information and support
There are lots of places to get information support if you or someone you love has hypoplastic left heart syndrome.
- 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 hypoplastic left heart syndrome. 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 ones support from pregnancy through to birth and living with your condition.
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