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Interrupted Aortic Arch
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Children’s heart conditions can’t be prevented, but a lot can be done to improve and often completely repair their hearts at any age. Thanks to advanced technology and the pediatric heart experts at the Nemours Cardiac Center (based at Nemours/Alfred I. duPont Hospital for Children), most children born with a heart problem — even newborns only hours or days old — can be quickly diagnosed and treated right when it matters the most. At the Cardiac Center, we specialize in early detection and repair of congenital heart defects (also often called, “congenital heart disease”).
If your child’s been diagnosed with interrupted aortic arch, we’re here to ease your concerns, answer your questions, and give your child the best possible chance for a healthy future.
Normally, oxygen rich “red” blood travels out of the left side of the heart into the aorta, first providing blood flow to the head and arms and continuing down into the abdomen and feet, providing blood flow to all parts of the body. The area where the aorta makes a U-turn and begins traveling down to the abdomen is called the “aortic arch.” An interrupted aortic arch happens when this area doesn’t develop fully and is separated into two, unconnected parts:
- one connected to the aortic valve and left ventricle supplies some of the vessels connected to the head and arms
- the other provides blood flow to the remaining head and arm vessels and lower body
There are different types of interrupted aortic arch, which are determined by exactly where the interruption occurs and which of the vessels to the head and upper extremity branch off of which part.
An interrupted aortic arch is typically accompanied by a ventricular septal defect (or “VSD,” a hole in the wall or “septum” between the lower chambers or ventricles of the heart). Oxygen-rich blood flows across the VSD to the right ventricle, where it mixes with oxygen-poor blood and is pumped through the pulmonary artery to the lungs.
Right after birth, some of this mixed blood travels to the lower body through a patent ductus arteriosus (or “PDA” — a connection between the pulmonary artery and descending aorta that’s necessary in the womb but usually closes within the first few hours or days after birth). This preserves the blood flow to the lower body, although that blood has less oxygen because it’s mixed with “blue” blood from the right side of the heart. When the PDA begins to close shortly after birth, the blood flow to the lower body dramatically decreases. This causes the lower half of the body to be bluish or “cyanotic.”
How Does the Heart Normally Work?
When your child has a congenital heart defect, there’s usually something wrong with the structure of the heart. In order to understand your child’s condition, it can help to know how the heart should work normally.
Learn More About Normal Cardiac Anatomy »
Nemours’ experts at KidsHealth.org also offer these helpful resources to help both you and your child understand how the heart works:
Interrupted aortic arch is usually diagnosed before birth with a fetal echocardiogram (“echo”) or immediately after birth. Caregivers may also hear a murmur due to blood flowing across the ventricular septal defect or the patent ductus arteriosus. As the PDA closes following birth, there may be a marked decrease in blood flow to the lower body, resulting in:
- infrequent urination
- decreased feeding
- pale and cool legs
An echocardiogram (“echo”) is usually all we need to make the diagnosis. An echo is a completely safe and painless test that uses ultrasound (sound waves) to build a series of pictures of the heart.
To repair the problem, children with an interrupted aortic arch will need surgery, which usually consists of closing the ventricular septal defect with a patch and connecting the two disconnected portions of the aortic arch.
Because interrupted aortic arch is a complicated abnormality, it’s common for children to need additional operations over the course of their lifetime. Close to half of children will require later operations to address problems that happen as babies grow into adolescents and adults.
If your child has interrupted aortic arch, know that at the Nemours Cardiac Center we’re here to give your child the very best, most comprehensive and compassionate care. Our goal is to guide your family, from start to finish, through your child’s heart defect journey — and to help your child live the healthiest, most fulfilling life possible.
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Information for Patients
Outpatient Services and Inpatient Units: (302) 651-6660
After 5 p.m. and Weekends:
Cardiac Intensive Care Unit: (302) 651-6644
General Inpatient Unit, 2B: (302) 651-6690