Interrupted Aortic Arch

What is 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. The aorta normally has a candy cane shape with small arteries (blood vessels carrying oxygen-rich blood) branching off, first to the head and arms and then curving down toward the belly to the lower body and legs.

In interrupted aortic arch, the first part of the aorta (which supplies blood to the head and arms) is not connected either to the second or to the third parts of the aorta (which supply blood to the lower body and legs).

This means that oxygen-rich blood pumped out of the heart cannot reach the lower body and legs normally. Instead, the descending aorta is connected to the pulmonary artery by a blood vessel called the ductus arteriosus.

  • It’s normal for babies to have a ductus arteriosus. Before they’re born, this vessel shifts blood away from their lungs into their aorta. This works fine because babies don’t breathe on their own until after they’re born, so they don’t need blood going to their lungs to get oxygen.

    Normally, the ductus arteriosus closes soon after birth. In babies with a normal aorta, this closure does not cause problems. In babies with interrupted aortic arch, however, the closure means there’s no way for blood to get to the descending aorta.

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

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

    About 2 in every 100,000 babies have interrupted aortic arch.

Interrupted Aortic Arch at Seattle Children’s

  • U.S. News & World Report consistently ranks Seattle Children’s cardiology and heart surgery program as one of the best in the country. With more than 40 pediatric cardiologists, we have experience diagnosing and treating every kind of heart problem.

    Our heart team has treated many children with interrupted aortic arch. This condition always requires surgical repair. We have a dedicated pediatric cardiovascular surgical team, including 4 surgeons, a pediatric cardiac anesthesia team and a Cardiac Intensive Care Unit, ready to care for newborns or infants who need heart surgery early in life.

    See our statistics and outcomes for heart surgeries, including ventricular septal defect repair.

    Seattle Children’s has been treating children since 1907. Our team members are trained in their fields and in meeting the unique needs of children. For example, the doctors who give your child anesthesia (sedation) are board certified in pediatric anesthesiology. This means they have extra years of training in how to take care of kids. Our child life specialists know how to help children understand their illnesses and treatments in ways that make sense for their age.

    The Adult Congenital Heart Disease Program shared by Seattle Children’s and the University of Washington can help with care throughout your child’s life.

  • When you come to Seattle Children’s, a team of people will take care of your child. Along with your child’s heart doctor (cardiologist), you are connected with newborn specialists (neonatologists), lung doctors (pulmonologists), nurses, child life specialists, social workers and others, if their expertise is needed.

    We’re committed to your child’s overall health and well-being. We’ll help your family through this experience and discuss treatments in ways you can understand and involve you in every decision.

    Read more about the supportive care we offer.

Symptoms of Interrupted Aortic Arch

Your baby will probably seem fine at birth. When their ductus arteriosus starts to close, within the first week of life, they will start to have symptoms because their blood is not flowing the way it should.

They may have symptoms like these: 

  • Weakness
  • Trouble feeding
  • Getting tired easily
  • Working hard to breathe
  • Fast heartbeat
  • Bluish skin, lips or nail beds (cyanosis)

Diagnosing Interrupted Aortic Arch

To diagnose this condition, your doctor will examine your child and ask for details about any symptoms your child has, their health history and your family health history.

Your child will need tests that provide more information about how their heart and nearby blood vessels look and work. These include: 

They may also need cardiac catheterization.

Treating Interrupted Aortic Arch

Babies with interrupted aortic arch need surgery during the first week of life.

Before surgery takes place, your baby will need medicine (prostaglandin) that keeps the ductus arteriosus open so blood can get to the rest of the body.

Surgery will connect the 2 separate parts of the aorta, close the ventricular septal defect (if necessary) and close the patent (open) ductus arteriosus. Often, these steps can all be done during the same surgery. Your doctor may need to do more than 1 surgery depending on your baby’s condition, such as the size and position of their ventricular septal defect and the size of the outflow from the left ventricle.

To meet your child’s long-term healthcare needs, we have a special Adult Congenital Heart Disease Program to transition your child to adult care when they’re ready.

Contact Us 

Contact the Heart Center at 206-987-2515 to request an appointment, a second opinion or more information.

Providers, see how to refer a patient.