Coarctation of the Aorta in Children

  • Boston Children's Hospital is here with expert help if your infant or child has been diagnosed with coarctation of the aorta (COA). While this condition is very serious, Boston Children’s has vast experience treating infants and children who have COA—with excellent short- and long-term outcomes. These pages will give you a basic understanding of the condition to help you plan for, and cope with, this rare congenital heart defect. Normal HeartCOARC

    In the infant or child with a normal heart, oxygen-poor (blue) blood returns to the right atrium from the body, enters the right ventricle and is pumped through the pulmonary artery into the lungs to receive oxygen. The oxygen-rich (red) blood returns to the left atrium from the lungs, passes into the left ventricle and is pumped out to the body through the aorta.

    COARC

    In COA, there’s a narrowing of the aorta, the main artery that delivers oxygen-rich (red) blood to the body. The narrowing restricts the amount of oxygen-rich blood that can travel to the lower part of the body.

    • Narrowing can occur anywhere in the aorta, but usually occurs in the segment just beyond the aortic arch—beyond the vessels that bring blood to the upper body, and before the vessels that bring blood to the lower body. This is why in COA, blood pressure in the arms will be high, and blood pressure in the legs and ankles will be low.
    • 75 percent of children with COA also have a bicuspid aortic valve—in which the aortic valve has two flaps (leaflets) instead of the usual three—as an accompanying (secondary) defect. (The aortic valve pulses blood from the left ventricle into the aorta.)
    • COA occurs in about 6 to 8 percent of all children with congenital heart disease, and occurs twice as often in boys as girls (as is the case with many “left heart” diseases). It also occurs in about 10 percent of girls who have Turner syndrome (a chromosomal abnormality).
    • The defect can be corrected with surgery or cardiac catheterization.
    • After correction, most children lead healthy lives but may need follow-up care.

    Please note: COA can occur as a defect by itself (in isolation), or in association with other defects, such as transposition of the great arteries, double outlet right ventricle or certain single ventricle defects. These pages will discuss COA as an isolated defect.

    How Boston Children’s Hospital approaches COA

    Our team in Boston Children’s Cardiac Surgery Department understands how distressing a diagnosis of coarctation of the aorta can be for parents. You can have peace of mind knowing that our surgeons treat some of the most complex pediatric heart conditions in the world, with overall success rates approaching 98 percent—among the highest in the nation for large pediatric cardiac centers. We use the following elements to provide the best possible outcomes:

    • accurate diagnosis and assessment: Subtle variations in the heart’s anatomy—such as the arrangement of the arteries that feed the heart—can negatively impact surgical outcomes if not identified ahead of time. We utilize the most advanced techniques available for precisely determining your child’s heart anatomy, with interpretation by highly experienced cardiologists.
    • sophisticated and effective therapies: Boston Children’s Congenital Heart Valve Program cares for children with congenital heart defects that involve absent or malfunctioning heart valves. Our surgeons have a strong record of excellence in heart valve repair and replacement, including minimally invasive techniques.
    • Babies with the most severe heart defects need emergency therapy. Our cardiac intensive care unit (CICU), cardiac catheterization labs and operating rooms are deal with the urgent needs of our smallest patients 24/7. Boston Children’s CICU was one of the first such units developed anywhere; professionals from many countries visit our CICU to learn advanced techniques of post-operative care.
    • experienced team of skilled professionals to perform surgery and other procedures:Children’s cardiac surgeons have vast experience in the surgical procedures used to repair this defect, and they work with nurses and doctors who are focused on providing expert care after surgery.  
    • close, expert medical follow-up: If you live in the Boston area, a Boston Children’s cardiologist will follow your child after surgical repair. If you live in another part of the United States or the world, a Boston Children’s cardiologist will work closely with your local cardiologist. Teen and adult patients with COA are followed by Boston Children’s cardiologists who have special training for adults with congenital heart problems.

    For more visual and audio information on COA, visit the COA section of our cardiovascular Multimedia Library.

    Coarctation of the aorta: Reviewed by Michael Freed, MD
    © Boston Children's Hospital, 2011

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