Interrupted aortic arch

PDF download is not available for Arabic and Urdu languages at this time. Please use the browser print function instead

Learn about interrupted aortic arch in children. This is a defect of the aorta not developing properly, and causes congestive heart failure.

Key points

  • A child with an interrupted aortic arch will have symptoms as the ductus arteriosus begins to close.
  • An interrupted aortic arch will be treated with drugs and open heart surgery during the first few days of life.
  • Complete repair of the defect results in a very high survival rate.

Interrupted aortic arch is a defect that results when the aorta does not develop properly. With this condition, oxygen-rich blood from the left side of the heart cannot reach all areas of the body because of the defective aorta. The heart manages to get enough blood to the body by way of the ductus arteriosus, which has stayed open after birth. It usually occurs with a large atrial or ventricular septal defect.

Normal heartNormal heart showing placement of the atria, ventricles, aorta, superior vena cava, inferior vena cava, pulmonary artery and pulmonary veins
The heart has two upper (receiving) and two lower (pumping) chambers. Blood flows into the upper chambers (the right atrium and the left atrium). The lower chambers (the right and left ventricles) pump blood out of the heart. The heart valves open and close to keep blood flowing in the correct direction.
Interrupted aortic archHeart with divided aorta and patent ductus arteriosus that allows blood to get to the lower body
Interrupted aortic arch is a malformation of the aorta, resulting in the aortic arch being divided. This results in the body not receiving enough blood high in oxygen. A patent ductus arteriosus provides a bypass, allowing blood low in oxygen to reach the body.

What are the symptoms of interrupted aortic arch?

A child with this condition will develop symptoms as the ductus arteriosus begins to close, and will require surgery during the first few days of life. Symptoms include weakness, poor feeding, fatigue, shortness of breath and laboured breathing, fast heart rate, and low oxygen levels. Congestive heart failure may also develop as part of this heart condition.

An electrocardiogram and an echocardiogram will be done to diagnose this defect.

How is interrupted aortic arch treated?

At first, a drug like prostaglandin will be given to keep the ductus arteriosus open to improve blood flow. Open heart surgery within the first few days of life will be done to correct the defect. This will involve connecting the two separate pieces of the aorta, closing the PDA, and closing the ASD or VSD.

What is the long-term outlook for children with interrupted aortic arch?

Complete repair of the defect results in about a 90% survival rate for children with interrupted aortic arch.

Last updated: December 4th 2009