Coarctation of the aorta

Coarctation of the aorta, or aortic coarctation, is a congenital condition whereby

the aorta narrows in the area where the ductus arteriosus (ligamentum arteriosum after
regression) inserts.

Types

There are three types:

1. Preductal coarctation: The narrowing is proximal to the ductus arteriosus.

Blood flow to the aorta that is distal to the narrowing is dependent on the ductus
arteriosus; therefor severe coarctation can be life-threatening. Preductal
coarctation results when an intracardiac anomaly during fetal life decreases
blood flow through the left side of the heart, leading to hypoplastic development
of the aorta. This is the type seen in approximately 5% of infants with Turner
Syndrome.
2. Ductal coarctation: The narrowing occurs at the insertion of the ductus
arteriosus. This kind usually appears when the ductus arteriosus closes.
3. Postductal coarctation: The narrowing is distal to the insertion of the ductus
arteriosus. Even with an open ductus arteriosus blood flow to the lower body can be
impaired. This type is most common in adults. It is associated with notching of the
ribs (because of collateral circulation), hypertension in the upper extremities, and weak
pulses in the lower extremities. Postductal coarctation is most likely the result of the
extension of a muscular artery (ductus arteriosus) into an elastic artery (aorta) during
fetal life, where the contraction and fibrosis of the ductus arteriosus upon birth
subsequently narrows the
Signs and symptoms

In mild cases, children may show no signs or symptoms at first and their condition may
not be diagnosed until later in life. Some children born with coarctation of the aorta have
other heart defects, too, such as aortic stenosis, ventricular septal defect, patent ductus
arteriosus or mitral valve abnormalities.

Coarctation is about twice as common in boys as it is in girls. It’s common in girls who
have Turner syndrome.

Symptoms may be absent with mild narrowings (coarctation). When present, they
include: difficulty breathing, poor appetite or trouble feeding, failure to thrive. Later on,
children may develop symptoms related to problems with blood flow and an enlarged
heart. They may experience dizziness or shortness of breath, faint or near-fainting
episodes, chest pain, abnormal tiredness or fatigue, headaches, or nosebleeds. They
may have cold legs and feet or have pain in their legs with exercise (intermittent
claudication).

In more severe cases, where severe coarctations, babies may develop serious problems
soon after birth because not enough blood can get through the aorta to the rest of their
body. Arterial hypertension in the arms with normal to low blood pressure in the lower
extremities is classic. Poor peripheral pulses in the legs and a weak femoral artery pulse
may be found in severe cases.

The coarctation typically occurs after the left subclavian artery. However, if situated
before it, blood flow to the left arm is compromised and asynchronous or radial pulses of
different "strength" may be detected (normal on the right arm, weak or delayed on the
left). In these cases, a difference between the normal radial pulse in the right arm and
the delayed femoral pulse in the legs (either side) may be apparent, whilst no such delay
would be appreciated with palpation of both delayed left arm and either femoral pulses.
On the other hand, a coarctation occurring after the left subclavian artery will produce
synchronous radial pulses, but radial-femoral delay will be present under palpation in
either arm (both arm pulses are normal compared to the delayed leg pulses).
Diagnosis

Coarctation of the aorta can be accurately diagnosed with magnetic resonance

angiography. In teenagers and adults echocardiograms may not be conclusive. In adults
with untreated coarctation blood often reaches the lower body through collaterals, e.g.
internal thoracic arteries via. the subclavian arteries. Those can be seen on MR, CT
or angiography. An untreated coarctation may also result in hypertrophy of the left
ventricle.

Treatment

Therapy/Treatment is conservative if asymptomatic, but may require surgical resection

of the narrow segment if there is arterial hypertension. The first operations to treat
coarctation were carried out byClarence Crafoord in Sweden in 1944. In some
cases angioplasty can be performed to dilate the narrowed artery. If the coarctation is
left untreated, arterial hypertension may become permanent due to irreversible changes
in some organs (such as the kidney)

For fetuses at high risk for developing coarctation, a novel experimental treatment
approach is being investigated, wherein the mother inhales 45% oxygen three times a
day (3 x 3–4 hours) beyond 34 weeks of gestation. The oxygen is transferred via the
placenta to the fetus and results in dilatation of the fetal lung vessels. As a
consequence, the flow of blood through the fetal circulatory system increases, including
that through the underdeveloped arch. In suitable fetuses, marked increases in aortic
arch dimensions have been observed over treatment periods of about two to three
weeks[