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:
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.
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:
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.
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:
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.