Interrupted Aortic Arch

Interrupted aortic arch is a birth defect in which a small section of the aorta is missing.

The aorta carries oxygen-rich (red) blood from the left ventricle of the heart to all parts of the body. Normally, the aorta has a curved shape. Just above the heart, small arteries branch off the aorta and go to the head and arms. Then the aorta curves down toward the belly, lower body and legs. The part that curves down is called the descending aorta.

This is a normal heart. The aorta leaves the heart near the top and then curves down toward the lower body. In interrupted aortic arch, the first part of this curve, or arch, is not connected to the rest.

In interrupted aortic arch, the first part of the aorta (which supplies blood to the head and arms) is not connected to the rest (which supplies blood to the lower body and legs).

This means oxygen-rich blood from the heart cannot reach the lower body and legs in the normal way. Instead, the descending aorta connects to the pulmonary artery by a blood vessel called the ductus arteriosus.

Most babies with interrupted aortic arch also have a hole in the wall between the right and left ventricles called a ventricular septal defect (VSD).

• How does the ductus arteriosus affect blood flow?

  It is normal for babies to have a ductus arteriosus. Before a baby is born, this vessel shifts blood away from their lungs into their aorta. This works fine because babies do not breathe on their own before birth, so they do not need blood to get to their lungs to pick up oxygen.

  Normally, the ductus arteriosus closes soon after birth. In babies with a normal aorta, this closure does not cause problems. But in babies with interrupted aortic arch, the closure means blood cannot get to the descending aorta.

  Before the ductus arteriosus closes, babies born with this defect do get blood to their lower body. But it is not oxygen-rich blood coming from their left ventricle. For most babies, it is a mix of oxygen-rich and oxygen-poor blood.

  This happens because most babies with interrupted aortic arch also have a ventricular septal defect. This hole allows some oxygen-rich blood to flow from the left ventricle to the right ventricle and then to the ductus arteriosus.

• The experts you need are here
  • Our team of more than 40 pediatric cardiologists has diagnosed and treated this condition in many babies — and we provide any ongoing care patients need as they grow into young adults.
  • Our pediatric cardiac surgeons perform more than 500 procedures yearly. Each has experience correcting interrupted aortic arch and related heart conditions in newborns and babies.
  • We also have a pediatric cardiac anesthesia team and a Cardiac Intensive Care Unit ready to care for children who have heart surgery.
• Among the nation's top programs

  Our surgical outcomes are among the best in the nation year after year, including outcomes for VSDs.

• Care from before birth through young adulthood
  • If your developing baby is diagnosed with interrupted aortic arch before birth, our Fetal Care and Treatment Center team works closely with you and your family to plan and prepare for the care your baby will need.
  • Your child’s treatment plan is custom-made. We plan and carry out their surgery based on the specific details of their heart defect, like if they have VSD along with interrupted aortic arch. We closely check your child’s needs to make sure they get the care that is right for them at every age.
  • We have a special Adult Congenital Heart Disease Program to meet your child’s long-term healthcare needs. This program, shared with the University of Washington, transitions your child to adult care when they are ready.
• Our approach reduces the number of procedures your child needs
  • At Seattle Children’s, we set goals to reduce the number of procedures your child is likely to need for interrupted aortic arch.
  • After surgery for this condition, some patients develop a narrow spot where their aorta was repaired. This puts extra stress on their heart. It may mean they need a cardiac catheterization procedure or another surgery to open the aorta again.
  • By carefully studying the steps in our surgical approach and the results for our patients, we designed a standard way to repair the aorta with lower risk of getting a narrow spot in the future.
• Support for your whole family
  • We are committed to your child’s overall health and well-being and to helping your child live a full and active life.
  • Whatever types of care your child needs, we will help your family through this experience. We will discuss your child’s condition and treatment options in ways you understand and involve you in every decision.
  • Our child life specialists know how to help children understand their illnesses and treatments in ways that make sense for their age.
  • Seattle Children’s has many resources, from financial to spiritual, to support your child and your family and make the journey as smooth as possible.
  • Many children and families travel to Seattle Children’s for heart surgery or other care. We help you coordinate travel and housing so you can stay focused on your child.
  • Read more about the supportive care we offer.