Aortic coarctation is a birth defect in which the major artery from the heart (the aorta) is narrowed somewhere along its length, most commonly just past the point where the aorta and the subclavian artery meet.
Causes, incidence, and risk factors
Coarctation means narrowing; aortic coarctaction is a birth defect (congenital disorder) in which a portion of the aorta is narrowed. The aorta is the source blood vessel for many arteries, which supply the body with blood and nutrients.
Aortic coarctation causes low blood pressure and low blood flow in the arteries that branch off below the narrow spot; high blood pressure occurs in the arteries that branch off closer to the heart. As a result, aortic coarctation often leads to high blood pressure in the upper body and arms (or one arm) and low blood pressure in the lower body and legs.
Aortic coarctation is more common in some genetic conditions, such as Turner’s syndrome, but it can also be associated with congenital abnormalities of the aortic valve, such as a bicuspid aortic valve.
Aortic coarctation occurs in approximately 1 out of 10,000 people. It is usually diagnosed in children or adults under 40.
Symptoms from coarctation depend on the severity of blood flow restriction. In severe cases, symptoms are present during infancy; in milder cases, symptoms may not develop until adolescence. Symptoms include decreased exercise performance, cold feet or legs, and shortness of breath. Other symptoms include:
- dizziness or fainting
- pounding headache
- chest pain
- cold extremities
- leg cramps with exercise
- hypertension (high blood pressure) with exercise
Note: There may be no symptoms.
Signs and tests
An examination reveals high blood pressure in the arms and low blood pressure in the legs, with a significant blood pressure difference between the arms and legs. The femoral (groin) pulse is weaker than the carotid (neck) pulse, or the femoral pulse may be totally absent.
Listening to the heart through a stethoscope reveals a murmur that is harsh and can be heard in the back. There may be signs of left-sided heart failure (especially in infants) or signs of aortic regurgitation.
Coarctation is often discovered during a newborn infant’s first examination or during a well-baby exam. The health care provider will detect that the femoral pulses are absent or very weak. Taking the pulses in an infant is an important part of the examination as there may not be any other symptoms or findings until the child is older.
Coarctation of the aorta can be confirmed by:
- X-ray of the chest (may also show abnormal ribs or “notching” of ribs caused by enlargement of the rib arteries)
- ECG that indicates left ventricle enlargement
- Doppler ultrasound of the aorta
- chest CT
- MRI of the chest
- cardiac catheterization and aortography
Both Doppler ultrasound and cardiac catheterization can detect an aortic pressure gradient, that is, a difference in pressure within the aorta, caused by the coarctation.
Surgery is usually advised. Occasionally, balloon angioplasty (using a technique similar to that used to open the coronary arteries, but performed on the aorta) may be an alternative to surgical repair.
With surgery, the narrowed segment of the aorta is removed, then repaired by anastomosis (placing the two free ends of the aorta back together) if the segment with the coarctation was short. If a longer segment must be removed, a Dacron graft (a synthetic material) is used to fill the gap.
Coarctation of the aorta is curable with surgery, and rapid improvement of symptoms often occurs after the repair. There is an increased risk for death caused by cardiovascular problems among patients who have undergone aortic repair; however, without treatment, most people with this condition die before they reach the age of 40.
Early surgical intervention (before 10 years old) is usually advised. Today, diagnosis of a coarctation and subsequent surgical repair typically occur during infancy.
- aortic aneurysm
- aortic dissection
- aortic rupture
- severe hypertension
- intracerebral hemorrhage
- heart failure
- premature development of coronary artery disease (CAD)
- paraplegia (rare complication of surgery to repair coarctation)
Calling your health care provider
Call your health care provider if symptoms indicate that coarctation of the aorta may be present.
Call your health care provider if fainting or chest pain develops; these may indicate an emergency situation.
There is no known way to prevent this disorder, however, awareness of risk may make early diagnosis and treatment possible.
by Martin A. Harms, M.D.
All ArmMed Media material is provided for information only and is neither advice nor a substitute for proper medical care. Consult a qualified healthcare professional who understands your particular history for individual concerns.