Interrupted Aortic Arch: Causes, Symptoms, and Treatment
Medically reviewed by Achuta Uppu, MD, Internal Medicine. Last reviewed: October 23, 2026.
What kind of doctor treats Interrupted Aortic Arch?
A pediatric cardiologist (a children's heart doctor) usually leads care for interrupted aortic arch, and a pediatric heart surgeon does the repair. Nearly all babies show signs in the first 2 weeks of life, and most show them on the first day. A direct primary care or concierge doctor can be a first stop for an evaluation and can refer you to a specialist when needed.
What is interrupted aortic arch?
Interrupted aortic arch, or IAA, is a rare heart defect that a baby is born with. In IAA, there is a gap in the aorta, the large blood vessel that carries blood from the heart to the body. The gap is between the part that leaves the heart and the part that runs down the body.
Before birth, a small blood vessel called the ductus arteriosus gives blood to the lower body. After birth, this vessel closes. In IAA, it is the only way blood can reach the parts below the gap. When it closes, the baby can become very sick very fast.
There are three types, called A, B, and C, based on where the gap is. Type B is the most common. A hole between the lower pumping chambers of the heart, called a ventricular septal defect, is often present too.
IAA is rare, at about 1 in 15,000 births.
Symptoms
A baby may look well until the ductus arteriosus closes. Then signs can include:
- Fast breathing and hard breathing.
- Trouble feeding.
- A bluish color of the skin, called cyanosis.
- Not passing urine.
Without quick treatment, these problems can lead to shock and death. A doctor may find missing pulses and a different blood pressure in the right arm than in the legs.
Causes and who gets it
Almost half of babies with IAA have a missing piece of chromosome 22, called a 22q11.2 deletion. This causes DiGeorge syndrome, which is the most common syndrome linked to IAA. CHARGE syndrome can also occur with IAA.
A mother's diabetes that started before pregnancy is a risk factor. Use of the medicine isotretinoin is a risk factor for type B. If you are pregnant or plan to be, talk with your health care provider about all the medicines you take.
How doctors diagnose it
IAA is easily missed on a scan done during pregnancy. If a scan before birth suggests it, tests after birth must confirm it. A newborn screening test called pulse oximetry can lead to an earlier diagnosis. A pulse oximeter is a clip placed on a baby's hand or foot that measures blood oxygen.
When IAA is suspected, doctors do a blood gas test, a chest X-ray, an electrocardiogram, and an echocardiogram. An echocardiogram is an ultrasound of the heart. It shows where the gap is. Other scans can show more detail before surgery. Doctors also do a lab test called FISH that looks for the chromosome 22 deletion.
Treatment
A medicine called prostaglandin E1 is started early to keep the ductus arteriosus open. This keeps blood flowing to the lower body until surgery. A baby in shock gets medicines to support the heart.
Once IAA is found, the treatment is immediate surgery. The surgeon joins the two parts of the aorta so blood can flow freely. The surgeon also repairs other heart defects, most often a hole in the wall between heart chambers.
How a baby does depends on birth weight, other heart defects, and the timing of surgery. Many children need more than one heart surgery. Even after a repair, people with a heart defect from birth need regular follow-up with a cardiologist for life.
When to see a doctor
Call 911 if a baby has blue or grey skin, trouble or abnormal breathing, trouble feeding, or is very sleepy or hard to wake.
If you are expecting or have a child with a heart defect, ask the care team about regular heart checkups.
How a direct primary care or concierge doctor fits in
A direct primary care doctor charges a flat monthly fee instead of billing insurance, while a concierge doctor usually charges more and often works alongside insurance. Either kind of doctor can help with checkups after surgery and can help set up visits with the heart team.
Common questions
Is interrupted aortic arch life threatening?
Yes, it can be. When the ductus arteriosus closes, the baby can go into shock. Starting prostaglandin E1 early and having surgery right away help protect the baby.
Does interrupted aortic arch come with other conditions?
Often it does. A hole between the heart chambers is common. About half of babies with IAA also have DiGeorge syndrome.
How is interrupted aortic arch repaired?
Surgery repairs interrupted aortic arch. The surgeon joins the two parts of the aorta so blood flows freely. Other heart defects, most often a hole between the heart chambers, are repaired as well.
Will my child need more surgeries?
Many children need more than one heart surgery. A cardiologist should follow your child for life, even after a repair.
Sources
- StatPearls (NCBI Bookshelf) - Interrupted Aortic Arch
- CDC - Interrupted Aortic Arch (Birth Defects Surveillance Manual)
- MedlinePlus - Congenital Heart Disease
Medical Disclaimer
This content is for general educational and informational purposes only and is not medical advice. It is not a substitute for professional medical advice, diagnosis, or treatment, and reading it does not create a doctor-patient relationship. Always seek the advice of your physician or another qualified health provider with any questions about a medical condition, and never disregard or delay seeking professional advice because of something you read here. If you think you may have a medical emergency, call 911 (or your local emergency number) immediately.
