A birth defect in which the major artery from the heart (the aorta) is narrowed somewhere along its length; most commonly the narrowing is just past the point where the aorta and the subclavian artery come together.

 

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Definition

A birth defect in which the major artery from the heart (the aorta) is narrowed somewhere along its length; most commonly the narrowing is just past the point where the aorta and the subclavian artery come together.

Causes, Incidence and Risk Factors

Coarctation is a birth defect (congenital disorder) in which a portion of the aorta is narrowed, resulting in low blood pressure and low blood flow past the defect and high blood pressure on the side that is closer to the heart (proximal to the defect). Most commonly, coarctation is located so that there is high blood pressure in the upper body and arms and low blood pressure in the lower body and legs.
There is an increased risk for aortic coarctation in some genetic conditions such as Turner's syndrome. It also occurs more often in boys than in girls. Symptoms from coarctation may not develop until adolescence, but can be present in infants depending on the severity of blood flow restriction. The symptoms include localized hypertension (high blood pressure in only certain parts of the body), cold feet or legs, decreased exercise performance, and heart failure. The pulse, normally felt in the groin (femoral pulse), is typically absent when a coarctation is present.
The disorder occurs in approximately 1 out of 10,000 people. It is usually diagnosed in children or adults less than 40 years old.
 

Symptoms

• dizziness
• pounding headache
• fainting
• nosebleed - symptom
• leg cramps with exercise
• absent or diminished pulses in the arteries of the groin (femoral pulses)
• cold legs or feet
• hypertension

Note: There may be no symptoms.

Signs And Tests

An examination reveals high blood pressure in the arms, with significant blood pressure difference between arms and legs. The femoral (groin) pulse is weaker than the carotid (neck) pulse, or the femoral pulse may be absent. Listening to the heart through a stethoscope reveals a murmur that is harsh and heard in the back. There may be signs of left-sided heart failure (especially in infants) or signs of aortic regurgitation.
Coarctation os 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. This 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 may be confirmed by:

• coronary angiography, looking at the aorta
• chest CT
• MRI of the chest
• echocardiography
• Doppler ultrasound of the aorta
• 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
• cardiac catheterization

Treatment

Surgery is usually advised. Occasionally, balloon angioplasty (using a similar technique 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 gap is small, or the gap may be bridged with a Dacron graft (a synthetic material used to fill larger gaps).

Expectations (Prognosis)

Coarctation of the aorta is curable with surgery, and rapid improvement of symptoms can be expected after the repair. There is an earlier incidence of cardiovascular death among people with aortic repair than among the general public; however, repair leads to a marked increase in longevity over those do not have the repair made. Early surgical intervention (before 10 years old) may improve these statistics. Today, diagnosis of a coarctation and the subsequent repair typically occur during infancy. Uncorrected, coarctation usually causes death before the person is 40 years old.

Complications

• aortic aneurysm
• aortic dissection
• aortic rupture
• severe hypertension
• endocarditis
• intracerebral hemorrhage
• stroke
• heart failure
• premature development of coronary artery disease (CAD)

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!
 

Prevention

There is no known way to prevent this disorder. Awareness of risk may make early diagnosis and treatment possible.