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Prevalence of one in 3000 live births.
Abnormal narrowing the aorta in the area surrounding the ductus arteriosus.
Refers to called aortic narrowing, that is a congenital condition whereby the aorta is narrow, usually in the area where the ductus arteriosus.
They are most common in the aortic arch.
It refers to a narrowing of the aortic arch between the origin of the left subclavian artery and the insertion of the ductus arteiosus ligamentum.
Other heart defects may also occur when coarctation is present.
Such defects, typically occur on the left side of the heart.
There are three types of aortic coarctations:
Preductal coarctation: The narrowing is proximal to the ductus arteriosus.
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.
Preductal coarctation is seen in approximately 5% of infants with Turner syndrome.
Ductal coarctation: The narrowing occurs at the insertion of the ductus arteriosus, and appears when the ductus arteriosus closes.
Postductal coarctation Indicates the narrowing is distal to the insertion of the ductus arteriosus.
Postdoctoral coarction is the most common type in adults, and is associated with notching of the ribs due to collateral circulation, hypertension of the upper extremities, and weak pulse pulses in the lower extremities.
Postductal coarctation is most likely the result from the extension of a muscular artery into the aorta during fetal life, where the contraction and fibrosis of the ductus arteriosus upon birth subsequently narrows the aortic lumen.
Preductal coarctation refers to a constriction of the aorta at the area proximal to the ductus arteriosus.
In the preductal coarctation the ductus arteriosus remains patent with a right to left shunt and sufficient blood to the lower extremities.
Postductal coarctation is related to an aortic constriction distal to the ductus arteriosus.
Alernative locations of a coarctation of the aorta, relative to the ductus arteriosus. A: Ductal coarctation, B: Preductal coarctation, C: Postductal coarctation. 1: Aorta ascendens, 2: Arteria pulmonalis, 3: Ductus arteriosus, 4: Aorta descendens, 5: Truncus brachiocephalicus, 6: Arteria carotis communis sinistra, 7: Arteria subclavia sinistra.
In mild cases, children may show no signs or symptoms initially, and diagnosis may not be made until later in life.
Coarctation is about twice as common in boys as it is in girls.
It is common in girls who have Turner syndrome.
With mild narrowings patients may be asymptomatic.
Some children born with coarctation have additional heart defects, such as aortic stenosis, ventricular septal defect, patent ductus arteriosus or mitral valve abnormalities.
Being that the aorta is narrowed, the left ventricle must generate a much higher pressure in order to force enough blood through the aorta to deliver blood to the lower part of the body.
If the left ventricle function is not adequate to push blood through the coarctation there is a lack of blood to the lower half of the body.
Associated with elevated blood pressure in the arms and decreased blood pressure in the legs and weak or absent femoral pulses.
Collateral blood vessels develop to perfuse the lower extremities.
Manifests either by neonatal critical coarctation and non-neonatal coarctation.
Critical coarctation manifests as shock when the ductus arteriosus closes in the neonatal period.
Less severe forms manifest with proximal aortic hypertension best evaluated with right arm blood pressures and delayed and diminished pulses in the femoral arteries.
In pediatric patients with hypertension aortic coarctation needs to be ruled out.
Clinical findings include normal heart sounds, unless there is an associated heart valve anomaly, a continuous murmur over the back, and chest x-ray findings of a “3 sign” manifested by indentation of the aorta at the point of coarctation and rib notching related to intercostal artery collateral flow.
Critical coarctation in the neonate is treated initially with prostaglandin E1 to maintain ductus arteriosus patency.
Surgical correction with resection of the coarctation site with aortic anastomosis is the definitive therapy.
Following surgical correction re-coarctation can be treated with angioplasty.
In older children and adults balloon angioplasty or stenting may be appropriate management.