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Heart Defects and TAR Syndrome

By Dr Mike Bamford
Consultant Paediatrician
and Medical Advisor to the
TAR Syndrome Support Group

Atrial Septal Defect (ASD)

There are two types of heart defect associated with TAR syndrome. The most common of these is an Atrial Septal Defect (ASD). This is a defect in the wall between the two collecting chambers (atria) of the heart. It rarely causes symptoms in childhood.

It may be detected by the presence of a soft heart murmur and may be confirmed by echocardiology (ultrasound examination of the heart).


 With an ASD a patient is likely to have no symptoms in childhood but over many years it can cause strain on the right ventricle of the heart which produces problems in adult life. It is usual, therefore, if this problem is detected to plan closure of the heart defect. This is not an urgent procedure and is often considered around the age of 4 or 5.

This can often be performed using keyhole surgery (which is much safer for children with TAR who may be prone to bleed during surgery). The operation to close ASD’s is very safe in the hands of a good surgeon with low risk for the child.

Tetralogy of Fallot

The more severe but less common heart defect associated with TAR syndrome is Tetralogy of Fallot. This is a condition where there is a defect in the wall between the two pumping chambers (ventricles) of the heart – ventricular septal defect; together with a narrowing of the outflow from the heart to the lung – pulmonary stenosis.

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 Fortunately this is rare. It would usually be evident at birth with the presence of a loud murmur which could be heard by an examining doctor and may be confirmed on an ultrasound scan of the baby’s heart before birth.

This condition rarely causes problems in the first few weeks of life but with time tends to cause increasing blueness of the child (cyanosis). This condition will certainly need surgical intervention. This can take the form of an operation to help the baby’s lung circulation by bypassing the pulmonary obstruction or, in due course, a full surgical correction of the condition. If this condition is diagnosed it would require full explanation from a paediatric cardiologist and a heart surgeon. All children with TAR syndrome should have an examination for the possibility of a heart problem by an experienced paediatrician and an echocardiogram performed.

The majority of children with TAR syndrome have no heart problems. Children will not develop them in later life if they were not present at birth.

© 1998 Mike Bamford and TAR Syndrome Support Group

[Last modified: 9 September 2001]