Coarctation of the Aorta in Infants and Children

What Is Coarctation of the Aorta?

Coarctation of the aorta occurs when the aorta is pinched or constricted. This constriction (or narrowing) is usually only in one location and occurs between the upper and lower half of the body. This obstructs blood flow to the lower part of the body and increases blood pressure in the upper half of the body (above the constriction).

Typical Heart

Heart with Coarctation of the Aorta

How Is Coarctation of the Aorta Diagnosed?

Diagnosis is confirmed with an echocardiogram. Sometimes other tests such as cardiac catheterization or an MRI are needed to help show the area of narrowing.

What Are the Symptoms of Coarctation of the Aorta?

In a less severe coarctation, sometimes there are no symptoms at birth, but the obstruction worsens over time, causing high blood pressure in the upper body and less blood flow to the lower body. The left heart has to pump harder and at higher pressures to overcome the obstruction. If the left ventricle cannot do so, it is called “heart failure.”

Additionally, there may be a difference in the pulses between the upper and lower half of the body. An abnormal murmur may also be noted on examination.

With very severe narrowing, symptoms usually develop soon after birth when the patent ductus arteriosus (PDA) closes. These symptoms include poor color, rapid breathing, mottled skin. All of these are symptoms of shock and may be confused with other illnesses that can occur in infants, such as severe infections.

There may not be abnormal murmurs on examination, and the blood pressure may not be elevated in the upper half of the body if the heart is not working well (heart failure). However, there may be other symptoms noted on the examination such as weak pulses and a difference between the pulses in the upper and lower half of the body. That alerts doctors to the possibility of coarctation of the aorta.

How Is Coarctation of the Aorta Treated?

In those cases with symptoms at birth, prostaglandin can be given to open up the ductus arteriosus and at least allow some blood to get to the lower half of the body. This is only a temporary solution because an intervention such as balloon dilation (angioplasty) or surgery is needed.

The surgical repair of aortic coarctation is done through an incision on the left chest below the armpit. The aorta is exposed near the heart. The aorta is clamped on both sides of the narrowing, and the narrow segment with the adjacent ductus arteriosus is cut out. The two ends of the aorta are then sewn together, clamps are removed and flow through the aorta is reestablished.

In an otherwise asymptomatic patient, intervention is performed on an elective basis. Patients with high blood pressure, thickened left ventricle (hypertrophy) or heart failure should have an intervention. This prevents future problems like developing high blood pressure as an adult.

What Are the Treatment Risks?

The risks of surgery or balloon angioplasty both include bleeding and infection. Special risks of balloon angioplasty include rupture of the aorta or the development of an aneurysm (ballooning of the aorta). Special risks of surgery include injury to one of the nerves that supplies the vocal cord, injury to arteries that go to the spine and injury to organs in the lower body that do not receive blood during the surgery while the aorta is clamped.

There may be very high blood pressure after the repair from both procedures. Additionally, there may be difficulty resuming eating and normal bowel movements, which is called post-coarctectomy syndrome.

What Are the Long-term Effects of Coarctation of the Aorta?

The long-term outlook for untreated coarctation of the aorta is a shortened lifespan due to hypertension (high blood pressure) and its effects on the heart (heart failure). The outlook after intervention is favorable, but long-term follow-up is required.

Lifestyle is nearly normal (depending on whether there is residual hypertension) with near normal lifespan. Rarely (5–10% of the time), coarctation of the aorta may recur. Some of these cases can be treated by initial or repeat balloon angioplasty. The long-term results of this procedure are still being studied. Also, blood pressure may stay high even after the coarctation has been repaired. Lifelong follow-up by a pediatric cardiologist is recommended.

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