Heart and Blood Conditions

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.

Interrupted Aortic Arch in Children

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. 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

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 four 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.

When you come to Children's, a team of people will take care of your child. Along with your child's heart doctor (cardiologist), you are connected with surgeons, newborn specialists (neonatologists), lung doctors (pulmonologists), nurses, child life specialists, social workers and others, if their expertise is needed. We work together to meet all of your child's health needs and help your family through this experience.

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 Children's and the University of Washington can help with care throughout your child's life.

Contact Us

Contact the Heart Center at 206-987-2015 for a cardiac referral, a second opinion or more information.