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Trusted answers from The Hospital for Sick Children September 02, 2010
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Aortic Atresia

With this condition, there is no opening, or a very small opening, from the left ventricle into the aorta. “Atresia” refers to a missing heart structure. The valve cannot open properly, which means the blood can’t move from the left ventricle to the body. The only source of blood flow to the body will be through the ductus ateriosus. Aortic atresia usually occurs in combination with other heart defects, typically hypoplastic left heart syndrome (HLHS).

Aortic Atresia
With this condition, the aortic valve, between the left ventricle and aorta, either doesn't exist or is only partially formed. The result is that blood can't flow from the left ventricle to the body. Instead, blood reaches the body by way of a patent ductus arteriosus. This condition is often accompanied by a coarctation of the aorta.

What are the symptoms of aortic atresia?

Symptoms usually occur soon after birth. The main symptoms are a heart murmur, cyanosis, rapid breathing or trouble breathing, irritability, low energy, and clammy skin. How serious the symptoms are depends on the severity of the defect and the presence of other defects.

The doctor may order any of the following tests: chest X-ray, electrocardiogram, echocardiogram, cardiac catheterization, and cardiac magnetic resonance imaging.

How is aortic atresia treated?

Treatment usually involves giving your child drugs to help ease the heart’s work. One drug, called prostaglandin, which is given by IV, can help keep the ductus arteriosus open to permit blood flow to the body.

Multiple surgeries may be needed. As well as being able to help diagnose the condition, cardiac catheterization can sometimes be done to help correct a problem. The procedure may rarely involve a balloon atrial septostomy to improve the mix of oxygen-rich blood and oxygen-poor blood between the atria.

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Last ReviewedReviewed by
June 21, 2004Joel Kirsh, MD, FRCPC
 
 
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