What is Coarctation of the Aorta?

Coarctation of the aorta is a congenital heart defect that requires aggressive treatment in about 50% of cases. The aorta is the main artery responsible for carrying blood from the left side of the heart to the body. This oxygen rich blood provides energy and allows the body to develop normally.

When coarctation of the aorta is present, the aorta is too narrow right below the area that supplies the smaller arteries leading to the upper and lower halves of the body. This area is called the juxta-ductal position. During fetal life and shortly after birth, the ductus, positioned right above the area of narrowing, allows for the blood to pass easily to the body.

Once the ductus closes, usually in the first day after an infant is born, coarctation of the aorta begins to severely limit bloodflow to the body. Mild coarctation of the aorta may not be noticeable initially. A great deal of narrowing, however, can cause heart and systemic failure, as the body cannot get the oxygen it needs.

A child with severe coarctation of the aorta generally presents symptoms shortly after birth. The child may have blue or pale mucus membranes. Blueness may be particularly noticed at the nail beds and around the eyes and nose. The child will not be able to eat well, and will have labored breathing. Also, physicians will note a significant heart murmur.

Children with severe coarctation of the aorta are in need of immediate treatment in order to restore proper blood flow to the body. To treat this condition, there are several options. Choice of treatment depends upon the nature of coarctation and also on the surgeon’s comfort level with various surgical options.

Most often, emergency surgery for coarctation of the aorta involves cutting the aorta, removing the narrowed portion, and reattaching the aorta. In some cases, a portion of pig, donor, or bovine valve may be necessary to make the aorta long enough. However, this is rare, and not preferable, as that portion of the valve will not grow.

Less frequently, the surgeon may be able to correct coarctation of the aorta by cutting through the aorta, thus widening it, and then placing a patch over the widened area. Both surgical options require heart-lung bypass, and though the operation is serious, rate of recovery is very good. Children may need to be on anti-coagulants and blood pressure medication, as coarctation often causes hypertension in the arms.

If coarctation of the aorta is not severe, parents may not even be aware their children have it. When it causes no issues for the child in health or growth, it may not necessitate repair. However, coarctation can worsen, so cardiologists usually elect to place a small tube to widen the aorta, so that the condition does not become more severe. When no surgery is attempted, the cardiologist will closely follow the patient for signs of increased hypertension and hypertrophy of the left ventricle.

Coarctation of the aorta may be present with other left-sided defects, most often a bicuspid aortic valve, which may require replacement later on. Children with this condition require lifetime follow-up with cardiologists, but when the condition is uncomplicated by other defects, life expectancy is quite good.