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Heart conditions

This list contains the most common types of congenital heart disease we treat. We also treat rare and very rare conditions.

Please click on the links under each condition to see information and cartoons explaining the conditions, provided by the Children´s Heart Federation.

Conditions and treatments

Anomalous pulmonary venous connection (APVC)

APVC is when all veins from the lungs are connected to the right (instead of the left) collecting chamber (atrium) of the heart. 

Treatment

It's usually diagnosed by echocardiogram and occasionally MRI. The type of abnormal connection (partial or total), determines whether surgery needs to be carried out urgently or not. An interventional approach is only carried out as a rescue treatment.

Aortic stenosis

Aortic stenosis occurs when the valve connecting the left pumping chamber of the heart and the main artery that goes to the body is too narrow.  

Treatment

It's typically diagnosed by echocardiography and treated with interventional heart catheterisation. Depending on the severity and the presence of other heart conditions, surgery might be necessary.

Atrial septal defect (ASD)

ASD is a hole in the wall between the two upper chambers of the heart (atria). 

Treatment

Diagnosis is usually by transthoracic, and occasionally, transoesophageal echocardiography.

ASDs are commonly treated using heart catheterisation, but depending on the size and location of the defect, surgery may be necessary.  

Coarctation (CoA) of the aorta

Coarctation (CoA) of the aorta is when the main body artery narrows, impairing the blood supply to the lower half of the body.

Treatment

Diagnosis and treatment for babies is based on clinical findings (such as weak pulses in the legs) and echocardiography. Typically treatment is surgery, but occasionally heart catheterisation may be the treatment of choice. 

In older children, diagnosis is based on clinical findings (such as weak pulses in the legs and high blood pressure), echocardiography and MRI. Treatment depends on the type of lesion. 

Surgery may be recommended, but in teenagers and adults, interventional heart catheterisation is usually the preferred treatment. 

Complete atrio-ventricular septal defect (AVSD)

AVSD occurs when, instead of two valves connecting the two collecting chambers (atria) with the two pumping chambers (ventricles), there is only one valve connecting all four chambers of the heart. 

Treatment

Diagnosis is usually established by echocardiography and ECG (electrocardiogram). 

This heart condition is treated surgically. The timing of surgery is dependant on the size of the defect (hole) and how well the cardiac valves are working. 

Partial atrio-ventricular septal defect (AVSD)

With a partial AVSD, as with a complete AVSD (outlined above) there is only a single valve connecting all four chambers, but no connection between the pumping chambers.

Treatment

As with the complete AVSD, treatment is usually surgery

Hypoplastic left heart syndrome (HLHS)

HLHS occurs where the left pumping chamber is underdeveloped.

Treatment

This very complex heart condition has been treated at Evelina London since 1992. Treatment usually involves three operations

  1. carried out in the neonatal (newborn) period
  2. in the first year of life
  3. between the ages of two and five

Since 2005, some patients have been able to have a combined surgical and interventional approach, avoiding cardiopulmonary bypass (hybrid operation).

Persistent ductus arteriosus (PDA)

PDA occurs when the arterial duct does not close. The arterial duct is a shortcut between the lung arteries and the main body artery (the aorta), which is necessary during pregnancy. This usually closes after birth, but in some people it doesn’t.  

Treatment

Whilst preterm babies typically require surgery, later in life this condition can be treated with catheter techniques

Pulmonary atresia with an intact ventricular septum

The lungs are typically supplied by an arterial duct. Pulmonary atresia with an intact ventricular septum occurs when the valve connecting the right pumping chamber of the heart (right ventricle) and the main artery to the lungs (pulmonary artery) does not open. The right ventricle can be underdeveloped.

Treatment

Depending on the size of the right ventricle, it may be possible to open the blockage with a heart catheterisation to balloon the valve and in some cases insert a stent at the arterial duct. If the right ventricle grows well enough, surgery may not be necessary.

Pulmonary atresia with ventricular septal defect

Pulmonary atresia with ventricular septal defect occurs when the valve connecting the right pumping chamber of the heart (right ventricle) and the main artery to the lungs (pulmonary artery) does not open. In addition, there is a connection between the two pumping chambers of the heart. Often, the lungs are supplied with blood by an arterial duct and/or abnormal vessels, so-called collateral arteries.

Treatment

Treatment depends on the size of the ventricles and the vessels connected to the heart. In most cases this involves surgery.

Pulmonary stenosis (PS)

Pulmonary stenosis occurs when the valve connecting the right pumping chamber of the heart (right ventricle) and the main artery to the lungs (pulmonary artery) is narrow.

Treatment

In most cases, a heart murmur leads to further investigation. Diagnosis is by echocardiography. Usually, treatment involves heart catheterisation to open the narrow valve with a balloon.

Tetralogy of Fallot (ToF)

ToF occurs when the part of the wall between the pumping chambers close to the valves is too far towards the right, leading to a narrowed valve between the right chamber and the lung arteries, a hole between the pumping chambers (VSD) and the aorta overriding this defect. The muscle of the right chamber can be quite thick.

Treatment

Depending on the size of the child, surgery involves one or two steps. In some cases an artificial vessel (called a BT-shunt), is needed first to provide enough blood flow into the lungs to allow the arteries to grow. Later, corrective surgery is carried out. In most cases the shunt is not necessary.

Transposition of the great arteries (TGA)

TGA is when the main body artery (aorta) and the main lung artery (pulmonary artery) are both connected to the wrong pumping chamber (ventricle). 

 

Treatment

Surgery is usually carried out within the first few weeks of life. Occasionally, creation of a bigger hole between the collecting chambers by catheter techniques is required as a first step..

Complex transposition of the great arteries

As with TGA above, but with complications.

Treatment

In most cases echocardiography can show all the anatomical details, but occasionally an MRI is needed to investigate the heart in even more detail. Treatment depends on the exact defect.

Congenitally corrected transposition of the great arteries

Congenitally corrected TGA ccurs when the main body artery (aorta) and the main lung artery (pulmonary artery) are both connected to the wrong pumping chamber (ventricle). The collecting chambers are wrongly connected as well.

Treatment

Depending on the presence of additional congenital heart disease, treatment is timed.

Tricuspid atresia

Tricuspid atresia is when the valve connecting the right collecting chamber (atrium) and the right pumping chamber (ventricle) does not open.

Treatment

Treatment consists of staged surgery

Ebstein's anomaly

Ebstein's anomaly is when the valve connecting the collecting chamber (atrium) and the right pumping chamber (ventricle) (the tricuspid valve) is displaced towards the tip of the heart.

Treatment

Depending on the degree of valvar displacement, surgery may be necessary. The frequent rhythm disturbances need to be followed.

Truncus arteriosus (single arterial trunk)

Truncus arteriosus occurs when instead of the main body artery (aorta) and the main lung artery (pulmonary artery), there is only one vessel arising from both ventricles.

Treatment

Treatment is usually surgery.

Ventricular septal defect (VSD) large

VSD large occurs when there is a large hole between both pumping chambers (ventricles).

Treatment

The large defects have to be closed surgically

Ventricular septal defect (VSD) small

VSD small is a hole between both pumping chambers (ventricles).

Treatment

Depending on the location of the defect, heart catheterisation may be possible, avoiding open heart surgery.

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