The Leeds Teaching Hospitals NHS Trust

Conditions and procedures

The Leeds Congenital Heart Unit works very closely with the Children's Heart Surgery Fund which has funded the development of a website with more detail about treatments and conditions. Below, as well as information, you will find links through to the relevant pages on the Leeds Congenital Hearts website.

This section contains information about specific congenital heart conditions. It is important to be aware that each person can have differing aspects of these conditions so not all this information may be applicable to one person.

Aortic Stenosis 

Aortic stenosis is a narrowing of the valve letting blood flow from the heart to the artery feeding the body with blood (the aorta). Almost all patients with aortic stenosis will need major heart surgery at some stage in their life, but the age at which surgery is necessary is very variable. If the valve is severely narrowed treatment may be necessary within the first few weeks of life, but if the narrowing is slight treatment may not be necessary until later in adult life. The timing of treatment depends on how badly narrowed the valve is, how it changes as time goes by (it almost always gets worse with increasing age), and how well the heart is coping with the extra work of pumping blood through the narrow valve.

Atrial Septal Defect (ASD) 

An atrial septal defect (ASD) is a hole between the two small chambers of the heart. It allows blood from the left-sided chamber to pass to the right-sided chamber. This results in the right side of the heart being overloaded with blood and also results in the lungs becoming congested because they receive an excessive amount of blood.

Such defects cause only mild symptoms in young children (such as breathlessness on exertion), but if an ASD is left untreated over a period of many years it eventually leads to permanent damage to the heart and sometimes to the lungs as well.

Coarctation of the aorta 

This is a serious heart condition in which there is a narrowing in the main artery (the aorta) which comes off the heart to feed the body with blood. The narrowing usually occurs just after the aorta has given off branches to supply the head and arms with blood, preventing normal circulation to the lower half of the body. In some patients, the coarctation is not apparent early in life but gradually develops over time (sometimes weeks, sometimes years).

Complete Atrioventricular Septal Defect 

This is a serious heart abnormality which consists of a complicated hole between the right and left sides of the heart. The hole occurs in the centre of the heart, effectively producing two holes – one between the two small pumping chambers (atriums) and the other between the two main pumping chambers (ventricles)

Heart murmur
 

A heart murmur is an unusual sound that can be heard between heartbeats. Heart murmurs can be harmless or abnormal and you or your child will have been referred for treatment if an abnormal heart murmur has been detected.  This can be a sign of a more serious problem.

Interrupted Aortic Arch
 

This is a very serious heart condition in which there is a complete blockage in the main artery (the aorta) which comes off the heart to feed the body with blood. The blockage usually occurs just after the aorta has given off branches to supply the head and arms with blood, preventing normal circulation to the lower half of the body.

Partial Atrio-Ventricular Septal Defect
 

This is a serious heart abnormality which consists of a hole between the two small pumping chambers (atriums). The hole also affects the inlet valves of the heart. Children with partial AVSD usually look perfectly well when they are young, but if the defect is not repaired it can eventually (after many years) cause permanent damage to the heart and the lungs. Surgery is usually recommended before school and sometimes before the age of 1 year.

Patent Ductus Arteriosus
 

The arterial duct (patent ductus arteriosus, or PDA) is a short blood vessel connecting the two main arteries which come off the heart to feed the lungs and the body with blood. It is a normal part of the circulation before birth and normally closes by itself within the first week or so after birth. Sometimes the duct fails to close by itself. In this case, we recommend treatment to close the duct.

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 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

Pulmonary Atresia with Ventricular Septal Defect
 

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

  • the artery which normally allows blood to be pumped from the heart to the lungs(the pulmonary artery) is completely blocked
  • the main pumping chamber on the right side of the heart is small.
Most babies with pulmonary atresia will need an operation within the first few weeks of life to make sure they can feed their lungs with blood. This operation involves connecting the artery leading to one of the arms to the artery which leads to the lungs and is called a “shunt” operation. This doesn’t make the baby’s heart normal, but it usually allows the baby to survive until he or she is old enough to consider more major surgery.

Pulmonary Stenosis
 

In this condition, the valve letting blood flow from the heart to the arteries in the lungs is narrowed. In most cases, it is not a serious problem.
Many patients have mild pulmonary stenosis and do not need any treatment at all, but the valve can become narrower as the patient grows, so checkups in the outpatient clinic will be necessary even if the patient appears perfectly well.

Small Ventricular Septal Defect (Muscular)
 

A muscular ventricular septal defect is a hole in the muscle wall between the two main pumping chambers of the heart (the ventricles). Most holes in the thick muscular part of the ventricular septum gradually get smaller or even close off completely on their own as the child grows. In some cases this happens within a few months, in others it may take many years and in some, the VSD doesn’t close at all. Even if a small VSD does not close by itself, it does not usually need any treatment and does not usually stop the patient leading a completely normal life.

Small Ventricular Septal Defect (Perimembraneous)
 

A ventricular septal defect is a hole in the wall between the two main pumping chambers of the heart (the ventricles). 

When there is a hole between the two ventricles (a VSD), blood flows from the left ventricle to the right ventricle through the hole. Some patients may have more than one VSD. When the hole occurs in the thinnest part of the septum it is called a “perimembraneous” VSD.

Most VSDs gradually get smaller or even close off completely on their own as the child grows. In some cases this happens within a few months, in others it may take many years and in some, the VSD doesn‘t close at all. Even if a small VSD does not close by itself it does not usually need any treatment and does not usually stop the patient leading a completely normal life. Occasionally, as children with this type of VSD grow new abnormalities such as narrowing beneath the outlet valves of the heart or leaky valves can arise. Although such problems only occur in a very small number of children, they can be serious enough to need surgical treatment, so regular check-ups in the outpatient clinic are recommended.

Tetralogy of Fallot
 

In the normal circulation, blood passes through the lungs to collect oxygen from the air. In babies with Tetralogy the narrowing in the heart causes blood flow to the lungs to be reduced, making the blood oxygen level low. This makes the baby (particularly the lips and hands) appear blue. How blue the baby looks depends on how severe the narrowing in the heart is; some babies appear pink or are just slightly blue when they cry but others may appear blue all the time. Some children with tetralogy may have attacks where they become suddenly very blue or very pale or floppy or faint. These are known as “hypercyanotic attacks” or are sometimes called “spells”. If your child has attacks like this you should tell us immediately as the attacks can be very serious and may even be life-threatening.

Transposition of the Great Arteries
 

This is a serious heart condition in which the main artery which should arise from the right side of the heart comes from the left side, and the artery which should arise from the left side of the heart comes from the right side. In a baby with transposition the blue blood gets pumped back to the body instead of to the lungs, the baby appears blue shortly after birth and immediate treatment is required.

Ventricular Septal Defect (Large)
 

A ventricular septal defect is a hole in the wall between the two main pumping chambers of the heart (the ventricles). Some patients have more than one VSD.
Babies with a VSD usually appear perfectly well in the first week or so of life, but many gradually become breathless over the first month or so after birth because the increased blood flow to the lungs makes the lungs congested. Babies who are very breathless often cannot feed normally and may not gain weight well because they put so much energy into breathing. Even quite big VSDs can gradually get smaller or even close off completely on their own as the child grows. However, if the VSD remains large enough to cause high blood pressure in the lungs for a long time (more than a year or so) there is a serious risk that the arteries in the lungs will become permanently damaged by the high blood pressure.