Coarctation of the Aorta

 

Condition Description

Coarctation of the aorta is a congenital defect of the heart where the major vessel that leaves the heart to go to the body, the aorta, has a narrowed segment. This segment is called a “coarctation”, and it causes high blood pressure in the vessel that is in front of the narrowed area. The narrowed segment is typically found just beyond the part of the aorta where the vessels go to the left arm, heart and head. The result is that there is higher pressure in the blood vessels of left arm, heart and brain and lower pressure in the rest of the body. There is no known cause for coarctation.

Coarctation of the aorta is a condition that may not be detected for many years if it involves a small segment of the aorta. Sometimes it is detected by finding an elevated blood pressure in the arms with lower blood pressure in the legs. There may be decreased or absent pulses in the legs as well. In about half of cases, this coarctation is severe enough that it is detected in the newborn period. In the rest of the cases it is milder, and blood flows through the narrowed area in sufficient quantities to sustain growth and activity without noticeable symptoms.

Prevalence

Coarctation accounts for 15-20% of congenital cardiac defects and is more common in males than in females. It occurs in 1 of 10,000 live births.

Common Associated Conditions

Children with coarctation of the aorta may have heart defects as well. They may have a defect with their aortic valve as well as ventricular septal defects. A patent ductus arteriosus is common in patients with some chromosomal abnormalities, such as Turner syndrome.

Short-term Treatment and Outcomes

There is considerable variation in the severity of coarctation, and that will determine the treatment plan. The segment may be very short and not too narrow, or extensive and very constricted. Thus the symptoms and treatment plan will vary.

If it is a small segment of narrowing, the surgery might be delayed and the child will be closely followed by a cardiologist to be sure there are no undue symptoms. Coarctation of the aorta is typically treated by surgery to remove the narrowed segment of the aorta. Typically the surgery is best done when the child is between 18 and 24 months of age. If it is a long segment a graft is sometimes inserted in its place. Sometimes coarctation can be corrected by a balloon angioplasty procedure done during cardiac catheterization. A plastic tube will be inserted in the narrowed segment and a balloon in the tube will be inflated to stretch the narrowed segment. Sometimes a mess tube is then inserted (called a stent) to hold the vessel open.

Long-term Treatment and Outcomes

Individuals who have had a coarctation of the aorta repair must be followed by a cardiologist for the rest of their lives because of the possibility of complications. Sometimes a surgical procedure has to be repeated if there is scarring that causes further narrowing of a segment of the aorta later in life. It will be important to have frequent blood pressure checks to be able to detect and promptly treat this condition. Antibiotics will be recommended with dental work so that the bacteria from the mouth that are released into the blood do not cause infection of the heart muscle (endocarditis).

Common Complications

Heart failure can occur, as the heart has to work extra-hard to pump blood through the narrowed section of the aorta. The heart will enlarge because of this effort. High blood pressure is a complication in uncorrected coarctation because the kidneys receive the blood that is under lower pressure and they will secret chemicals to raise the blood pressure to keep the kidneys perfused. High blood pressure can persist even after the coarctation is corrected.

High blood pressure can cause damage to various parts of the body (such as the eyes and kidneys), and it also can lead to serious conditions such as stroke. Thus high blood pressure should be monitored and treated throughout life. Problems with the aortic valve can also be found in about 30% of individuals with coarctation of the aorta, and this condition will also require careful assessment frequently.

Other complications of uncorrected coarctation could include pain in the legs and decreased exercise tolerance as well as chest pain.

Implications for Children's Development

Coarctation of the aorta is a condition for which surgical outcomes are good and children will lead normal and healthy lives without restriction of their activity. They will typically catch up in growth and have normal development following the recovery period of the surgery.

For more information, including resources for parents and general information about congenital cardiac conditions, visit the following websites:

Lasting Imprint of MN: Lasting Imprint exists to create a supportive community that will inspire people to join in the fight against congenital heart defects through education, research, and assistance to heart families.