COARCTATION OF AORTA

 

What is coarctation of aorta?

 

Coarctation of aorta means narrowing of the main body artery. Aorta is the main body artery that arises from the left pumping chamber the left ventricle. Any narrowing of this artery increases the burden of work on the heart. This can affect its function from causing weakening of heart muscle and heart failure or increased thickening and stiffening of heart muscle affecting its filling with blood. The most common site of coarctation is just beyond the aortic arch that gives branches to supply the head, neck and upper arms. Interruption of aorta is the most severe form and a different entity where there is a complete break in continuity of the aorta. This needs urgent attention. It accounts for approximately 6% of all congenital heart diseases.

 

 

What causes coarctation ?
 

The cause is not known in most cases. It can be associated with other heart conditions such as hole in the heart or more complex conditions. It is common with certain genetic conditions such as Turner syndrome. 

 

 

What are the symptoms?
 

Most infants and children with less severe forms are diagnosed with a heart murmur or weak pulses in legs, picked up during routine baby checks or assessment of non cardiac problems. Generally, they may have mild or no symptoms. Mild coarctation may go undetected in childhood and may be picked up in teenagers or young adults with heart murmur, headaches (related to high blood pressure), or leg pains on exercise, when it progresses in severity. In teenagers or young adults with high blood pressure (hypertension), it is important to rule out coarctation of aorta as a treatable cause.

 

Babies with severe coarctation could be completely well for a few hours or even days and then rapidly get worse when the patent ductus arteriosus closes, resulting in poor feeding, grey colour and mottling and increasing breathlessness. This needs to be treated urgently with a special medication called prostaglandin that keeps the patent ductus arteriosus open and will support the circulation. After stabilisation, babies need to be transferred to a specialist cardiac centre where definitive treatment by surgery can be offered. With introduction of fetal echocardiography, it can be detected in babies before birth which helps in planning their treatment soon after birth before any worsening.

 

 

How is it diagnosed?
 

In babies or infants, echocardiography is a comprehensive investigation that allows for all the information needed before surgery. It also detects associated defects that may need treatment or need different surgical plan. In older children and teenagers, echocardiography does provide a diagnosis but sometimes Dr Khambadkone will need to arrange additional imaging such as Computerized tomography (CT) or Magnetic Resonance Imaging (MRI). Cardiac catheterization and angiography is only used when a key hole strategy is planned for treatment. Dr Khambadkone can discuss all options with you.
 

 

How is coarctation of aorta treated?
 

In babies, prostaglandin is an emergency medication that helps stabilize their circulation allowing transfer to a specialist cardiac centre. Definitive treatment is with surgery and Dr Khambadkone works closely with renowned cardiac surgeons who will perform the surgery. When babies or infants are very sick or if the risk of surgery is high, balloon angioplasty by cardiac catheterisation is used to improve the circulation and the condition of the baby, and to offer surgery at a later stage if there is recurrence of persistence of narrowing. Dr Khambadkone has the expertise and experience to perform this.

 

In older children and adults, the first line of treatment has become stent angioplasty. A metal slotted tube called a stent, often with a covering on it (covered stent) is put in the narrow area by using a balloon on a slender tube called a catheter. It is delivered to the narrow area through one of the arteries in the leg. The stent can be enlarged to a diameter matching the size of aorta in the patient and can be increased by stretching it with larger balloons as children grow to adult size.

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Cardiac catheterization and balloon angioplasty works most effectively for most re- coarctations. Rarely, re-operation may be necessary..

 

What is the prognosis?
 

Despite treatment, patients are at a higher risk of high blood pressure (hypertension) in later life. Lifelong regular monitoring is hence mandatory for all patients with coarctation after treatment. In some instances, blood pressure lowering medications may be necessary. Once treated, with regular monitoring for high blood pressure, the prognosis is generally good. Children can be active and lead normal lives. Young adults and adults may need some lifestyle changes, such as avoidance of smoking, weight training and maintaining good cardiovascular fitness.