Right Aortic Arch

Right Aortic Arch (RAA) is a type of aortic arch variant defined by the way in which the aortic arch travels to the right of the trachea. The aorta is the main artery that delivers oxygen rich blood to the body.

The aortic arches form from the aortic sac and develop into several pairs of arches.  As the heart is forming, they develop and regress at different times and ultimately the right branch regresses leaving a left sided aortic arch. This means as the aorta leaves the heart, it arches to the left side.

RAA occurs due to the persistence of the right branch with the regression of the left branch. This means that the aorta arches to the right side. 

It is most commonly a normal variant.

A RAA is thought to occur in around one in 1,000 people, but is likely to be higher as identification during prenatal screening is increasing.

There are normally 3 blood vessels that arise from the aortic arch and in babies with a right aortic arch there can sometimes be four vessels. The presence of the other vessel can create what is known a vascular ring around the trachea (the breathing tube) and the oesophagus (the feeding tube). This can compress the trachea and the oesophagus and cause breathing or swallowing difficulties.

RAA can be associated with other congenital heart defects like Tetralogy of Fallot’s and Truncus Arteriosus. RAA is also associated with chromosomal abnormalities such as DiGeorge syndrome (22q11 deletion).

How can right aortic arch be spotted?

RAA can be detected at the 20 week anatomy scan or after birth by echocardiogram.

What are the symptoms of right aortic arch?

Most babies with a right aortic arch will not have any symptoms after they are born. However, if there is presence of a vascular ring then the baby may display the following signs:

• Noisy breathing
• Poor feeding
• Poor weight gain
• Difficulty swallowing
• Frequent chest infections

These signs and symptoms may not be apparent immediately. They may only develop over months or even years.

How can right aortic arch be treated?

The majority of babies with isolated RAA will not experience problems and no treatment is required.

Surgery is offered for those babies who have a vascular ring. The aim of the surgery is to divide the vascular ring to stop the compression of the trachea and oesophagus. In most cases the operation is performed by the surgeon entering the left side of the chest between the ribs. This is not ‘open heart’ surgery and your baby may only stay in hospital a couple of days following the procedure. Your baby will have a scar on the left side of their chest.

General advice & information for the future

If baby is showing any symptoms of noisy breathing or difficulty swallowing then a first review will be offered and further investigations will be indicated which may include a referral to ENT (Ear, Nose and Throat assessment).

Your baby may have routine follow up within the first few months of age and also may be offered further follow up appointments during the first few years of their life.

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