An interrupted aortic arch (IAA) is an abnormality of the major artery of the body, the aorta. When an aortic arch is interrupted, it means that at some point along the curve, the part going up (ascending aorta) ends and is not attached to the part going down (descending aorta). This means that the heart cannot send blood through the aorta to the lower body.

There are three types of IAAs:

• Type A: The interruption happens just past the left subclavian artery. Up to 30 to 40% of infants with IAA have type A.
• Type B: The interruption happens between the left carotid artery and the left subclavian artery. Type B is the most common type of IAA, accounting for about 53% of reported cases.
• Type C: The interruption happens between the innominate artery and the left carotid artery. Type C is the least common type, representing about 4% of reported cases.

The specialists at Norton Children’s Heart Institute, affiliated with the UofL School of Medicine — the leading provider of pediatric heart care in Louisville and Southern Indiana — can help your child with an interrupted aortic arch.

The board-certified and fellowship-trained specialists at Norton Children’s Heart Institute have the skills and experience to provide a pinpoint diagnosis and develop a customized treatment plan for you and your child — for life.

Norton Children’s has a network of outreach diagnostic and treatment services throughout Kentucky and Southern Indiana.

Interrupted Aortic Arch Symptoms

An IAA is a life-threatening condition. When a baby is born with IAA, the only way for the lower body to get blood is through a small blood vessel called the ductus arteriosus, and even then, that blood does not contain the oxygen level we need. The ductus arteriosus normally closes on its own a day or two after birth. When it closes, an infant with an IAA will get extremely sick.

Babies with IAA usually can seem healthy at birth but become very ill within the first days of life. Symptoms include:

• Bluish skin tone (cyanosis)
• Rapid breathing
• Rapid heartbeat
• Poor feeding

An IAA almost always is associated with a ventricular septal defect (VSD), which will cause a heart murmur — a specific “whooshing” sound — that may be heard by the hospital staff.

DiGeorge syndrome is a condition associated with IAA, type B. Children with DiGeorge syndrome may have low calcium and immune system abnormalities. DiGeorge syndrome can be diagnosed with a blood test if suspected.

Diagnosing Interrupted Aortic Arch

The specialists at Norton Children’s Heart Institute can diagnose IAA with a fetal imaging scan before the baby is born or after birth with an echocardiogram. Other tests performed may include:

• CT angiography: Uses a computed tomography (CT) scanner to produce detailed images of the blood vessels following a dye injection.
• Chest X-ray: This shows pictures of the heart and lungs, and can show heart and lung issues, including extra blood flow or fluid in the lungs.
• Echocardiogram (echo): This test uses sound waves (ultrasound) to produce images of the heart and blood vessels’ structures on a screen. It can show structure of the heart and ductus arteriosus and also the function of the heart. Norton Children’s Heart Institute has 28 tele-echo locations throughout Kentucky and Southern Indiana.
• Electrocardiogram (ECG or EKG): A test that checks the heart’s electrical activity to show damage or irregular rhythms.
• Heart catheterization: This invasive procedure studies the structure, function, and provides direct pressure measurements of the heart and large blood vessels.

Interrupted Aortic Arch Treatment

Survival is not possible without surgery. Babies with an IAA may get medicine to keep the ductus arteriosus open until surgery can be done. A baby needs surgery to fix the interruption and connect the ascending aorta to the descending aorta. Our pediatric cardiothoracic surgeons will connect the two separate portions of the aorta, close the ventricular septal defect when present, and tie off (ligate) the patent ductus arteriosus.

Long-term follow-up by our pediatric cardiologists is essential to assess growth of the aortic valve region and the reconstructed aortic arch. Ten percent to 20 percent of patients may need another operation or procedure to address further issues with these areas. When the child becomes an adult, a congenital heart defect specialist should continue to provide care.

Why Choose Norton Children’s Heart Institute

• Norton Children’s Hospital has been a pioneer in pediatric cardiothoracic surgery, performing Kentucky’s first pediatric heart transplant in 1986 and becoming the second site in the United States to perform an infant heart transplant.
• The American Board of Thoracic Surgery has certified our cardiothoracic surgeons in congenital heart surgery.
• The Adult Congenital Heart Association has accredited Norton Children’s Heart Adult Congenital Heart Disease Program as the only comprehensive care center in Kentucky and Indiana treating adults born with a heart defect.
• More than 17,000 children a year visit Norton Children’s Heart Institute for advanced heart care.
• Norton Children’s Heart Institute has offices across Kentucky and Southern Indiana to bring quality pediatric heart care closer to home.
• The Jennifer Lawrence Cardiac Intensive Care Unit (CICU) at Norton Children’s Hospital is the largest dedicated CICU in Kentucky, equipped with 17 private rooms and the newest technology available for heart care.