Interrupted Aortic Arch

Overview

What is interrupted aortic arch (IAA)?

Interrupted aortic arch (IAA) is a rare condition in which your baby’s aorta — the large blood vessel coming from their heart that carries blood to the rest of their body — is incomplete or interrupted.

The human heart has four chambers: The left atrium and right atrium (plural: atria) on the top, and the left and right ventricles (pumping chambers) on the bottom. Blue blood, which does not have much oxygen, circulates from your body to the right atrium and then is pumped to the right ventricle, and then out to your lungs. The blood picks up oxygen from your lungs and then goes into the left atrium. From there, it goes into the left ventricle. The left ventricle pumps this oxygenated blood through the aorta to the rest of your body.

The normal shape of the aorta is an arch or candy cane. When your baby has an interrupted aortic arch, the arch portion of the aorta is incomplete. This means that your baby’s heart cannot send blood through the aorta. This is a life-threatening situation. If blood doesn’t get to any part of their body, your baby can go into shock and could die.

How common is interrupted aortic arch (IAA)?

IAA is an extremely rare condition. It occurs in 2 of every 100,000 births.

Symptoms and Causes

What is the cause of interrupted aortic arch (IAA)?

IAA is a genetic (inherited) disease. This means that this condition is passed down through your family. Specifically, a section of a chromosome that affects the heart’s anatomy is missing. This is responsible for changing the normal shape of the heart and its vessels.

Many babies who are born with IAA also have DiGeorge syndrome, which is also a genetic disorder caused by the missing part of the same chromosome. Children who are born with DiGeorge syndrome can suffer from repeated infections and facial defects such as cleft lip.

Although it’s rare, IAA is a life-threatening condition and must be treated right away. Babies who are born with IAA usually show symptoms right away.

What are the symptoms of interrupted aortic arch (IAA)?

Babies who have IAA may have the following symptoms:

  • Weakness and fatigue.
  • Rapid breathing.
  • Rapid heartbeat.
  • Poor feeding habits.

In addition, your baby’s skin may appear gray in certain areas because blood isn't reaching that part of their body.

Babies born with IAA may also have an atrial septal defect (ASD) or ventricular septal defect (VSD). A septal defect is a hole in the septum, the wall in the middle of the heart. In babies who have IAA, a ventricular septal defect allows some oxygenated blood to move from the left ventricle to the right ventricle, for circulation in the body.

Babies who have IAA may also have other heart defects, such as truncus arteriosus.

Diagnosis and Tests

How is interrupted aortic arch (IAA) diagnosed?

IAA is usually diagnosed with an echocardiogram. During an echocardiogram, ultrasound (high-frequency sound waves) from a handheld wand placed on their chest provides images of your baby’s heart valves and chambers. An IAA can also be seen with the use of ultrasound while your baby is still in the womb.

Management and Treatment

How is interrupted aortic arch (IAA) treated?

IAA is treated with surgery to repair the aorta and create an unobstructed flow of blood to the body and brain.

One of the signs of IAA is when a small blood vessel (the ductus arteriosus) that supplies blood to either the lungs or the body does not close after birth. This is called a patent ductus arteriosus (PDA). Because the PDA is supplying blood to the body, the first step in treating interrupted aortic arch is to keep the PDA open. This is done by giving your baby a drug known as prostaglandin E1.

During surgery, your baby’s surgeon will connect the two ends of the aorta so that they form a complete and correct arch. The PDA will be closed and the hole between the two pumping chambers (ventricular septal defect) will also be closed.

Outlook / Prognosis

What is the outlook if you have an interrupted aortic arch (IAA)?

After surgery for IAA, people with the condition usually have a good outlook. For example, 10 years after surgery, 81% of people are still alive.

A note from Cleveland Clinic

Interrupted aortic arch (IAA) is a rare and serious medical condition. The last thing you want to hear is that your baby has a life-threatening illness. The good news is that with prompt treatment, the outlook is good. If your child is diagnosed with this condition, they need immediate surgery or medical care in an ICU setting.

Last reviewed by a Cleveland Clinic medical professional on 09/14/2021.

References

  • American Heart Association. Your Aorta: The Pulse of Life. (https://www.heart.org/en/health-topics/aortic-aneurysm/your-aorta-the-pulse-of-life) Accessed 9/14/2021.
  • Holland M, Schulz A, Feins EN, Baird CW. Neonates with Right Aortic Arch Requiring Arch Reconstruction: A Single-Institution Experience. (https://pubmed.ncbi.nlm.nih.gov/33864758/) Ann Thorac Surg. 2021 Apr 14:S0003-4975(21):00696-2. Accessed 9/14/2021.
  • Mastropietro CW, Amula V, Sassalos P, et al. Characteristics and operative outcomes for children undergoing repair of truncus arteriosus: a contemporary multicenter analysis. (https://pubmed.ncbi.nlm.nih.gov/30954295/) J Thorac Cardiovasc Surg. 2019 Jun;157(6):2836-2398. Accessed 9/14/2021.
  • Ramirez Alcantara J, Mendez M. Interrupted Aortic Arch. (https://www.ncbi.nlm.nih.gov/books/NBK532902/) StatPearls. October 2020. Accessed 9/14/2021.

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