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Coarctation of the Aorta and Hypoplastic 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 coarctation of the aorta, we’re here to ease your concerns, answer your questions, and give your child the best possible chance for a healthy future.
The aorta is the main artery that sends oxygen-rich blood from the heart to the body. “Coarctation” refers to a narrowing or constriction of the aorta — usually found in the descending aorta (the blood vessel that delivers oxygen-rich blood from the left ventricle to the body) — which hinders blood flow to the lower half of the body. When the narrowed area is longer and affects the entire arch of the aorta rather than only a segment, it may be called a “hypoplastic aortic arch.”
The narrowing forces the left ventricle (one of the heart’s two lower chambers that receive blood from the atria) to work harder to pump blood past the constriction. This leads to an increase in pressure in the left ventricle, causing enlargement (“hypertrophy”) of the ventricle. Because the kidneys (which control blood pressure in the long-term) are beyond the constriction and sense a lower blood pressure, they send signals to the brain, heart and blood vessels to increase the blood pressure. Over years, this can cause high blood pressure (also called “hypertension”) and a higher risk of strokes and heart attacks in adulthood.
When a child is a newborn, blood flow to the lower body may go around the narrowed area through a blood vessel called the “ductus arteriosus.” The ductus arteriosus forms a connection between the pulmonary artery and the aorta. The ductus arteriosus is necessary when babies are in utero (in the womb) and receiving oxygenated blood from the uterus rather than their own lungs, but it usually closes shortly after birth. If it stays open (called a “patent ductus arteriosus,” or “PDA”), and the coarctation is severe, this may cause oxygen-poor blood from the pulmonary artery to enter the aorta and travel to the lower body. Babies in this situation may look bluish (or “cyanotic”) in the lower extremities (their legs and feet).
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:
Depending on the severity of the narrowing, coarctation of the aorta may be diagnosed immediately after birth, or may not become apparent for months (even years). When diagnosed during the newborn period, it’s often diagnosed as the ductus arteriosus closes, which leads to poor blood flow to the lower body and high blood pressure in the upper body. If the ductus remains open, it may be diagnosed during the newborn period due to the lower oxygen levels in the blood of the lower body.
Our Nemours Cardiac Center pediatric heart experts can confirm a coarctation of the aorta diagnosis through an echocardiogram (“echo”) — a completely safe and painless test that uses ultrasound (sound waves) to build a series of pictures of the heart.
We often also perform a cardiac catheterization to evaluate the degree of narrowing and accurately measure the blood pressure difference between upper body and lower body. Catheterization involves inserting a thin plastic, flexible tube (called a “catheter”) into an artery and vein that lead to the heart. Cardiac catheterization is done under sedation with local anesthesia or under general anesthesia, depending on your child’s age and condition.
Children with coarctation of the aorta will need the coarctation area repaired. Depending on the exact anatomy of the coarctation, the child’s age, and whether a child has had previous repairs, there are at least two options for treatment:
- cardiac catheterization
Surgical treatment usually involves something called an “end-to-end anastomosis,” in which our Nemours Cardiac Center heart surgeons remove the narrowed portion of the aorta and then sew the two ends together. This leaves children with a wider aorta and one made entirely of their own tissue. In older children, or when there is a hypoplastic aortic arch, we may consider other techniques, including using a patch to widen the narrowed portion of the aorta. The patch material may be a synthetic material or a blood vessel from the arm (called the “subclavian artery”).
The catheter technique involves using a balloon inside the artery to open up the narrowed area. A pediatric cardiac catheterization physician inserts a balloon on a heart catheter, which is passed through an artery in the leg that leads up to the descending aorta. Sometimes (and especially in older children and adults) we use a stent (a wire cage inserted over top of the balloon and expanded on the inside of the artery) to keep the narrowed area open.
Most children won’t need another procedure after repair of the coarctation. But in some cases (approximately 10–20 percent) the narrowed area doesn’t grow normally after being repaired and a narrowing redevelops. In these cases, children need an additional procedure (most commonly a balloon catheter procedure) to reopen the narrow area. And, although it’s unclear why, in the years following a successful repair children may develop high blood pressure, which can be managed with medicine.
If your child has coarctation of the aorta, 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