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 can not 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. 30 to 40 percent 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 percent 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 percent of reported cases.

The specialists at Norton Children’s Heart Institute — 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, affiliated with the University of Louisville, have the skill and experience to provide a pinpoint diagnosis and develop a customized treatment plan for you and your child — for life.

The Society of Thoracic Surgeons has ranked Norton Children’s Heart Institute’s pediatric heart care among the best in the region. With our network of remote diagnostic and treatment services in Kentucky and Southern Indiana, your child can stay close to home for quality care.

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 while the baby is still in the womb or after birth with an echocardiogram. Other tests performed may include:

• CT angiography: uses a 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 aorta and also the function of the heart. Norton Children’s Heart Institute has 28 tele-echo locations in Kentucky and Southern Indiana so many patients don’t have to travel far for an echocardiogram.
• 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.

Why Norton Children’s Heart Institute?

Norton Children’s Heart Institute, affiliated with the University of Louisville, is a comprehensive pediatric heart surgery, heart failure and heart transplant program serving Kentucky, Southern Indiana and beyond.

The goal of the full-service Norton Children’s Heart Institute is to provide care for the child and the whole family. Our specialists are prepared to repair even the most complex congenital and acquired heart conditions.

Our heart team includes:

• Pediatric cardiothoracic surgeons
• Pediatric transplant surgeons
• Pediatric cardiologists
  • Fetal cardiologists
  • Adult congenital heart cardiologists
  • Heart failure/heart transplant cardiologists
  • Pediatric electrophysiologists
  • Pediatric cardiac catheterization cardiologists
• Pediatric cardiovascular anesthesiologists
• Pediatric intensive care physicians
• Cardiac critical care nurses
• Critical care pharmacists
• Family support team
• Child life specialists
• Rehabilitation specialists
• Social workers