Testing & diagnosis for coarctation of the aorta in children

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At Boston Children's Hospital, we know that the first step to restoring your child’s health is to form an accurate and timely diagnosis.

Diagnosing the infant

Your child's doctor may have heard a heart murmur (a noise caused by the turbulence of blood flowing through a narrow region) during a physical exam and referred your child to a pediatric cardiologist* for a diagnosis. The murmur can—but doesn’t usually—result from the coarctation itself; more often, the murmur is the result of secondary defects, such as a bicuspid aortic valve or a VSD.

*A pediatric cardiologist specializes in the diagnosis and medical management of congenital heart defects—as well as any heart problems that may develop later in childhood.

The first line of diagnosis for COA is the clinical exam, beginning with obtaining the child’s vital signs. The pediatric cardiologist obtains a four-limb blood pressure—measuring the blood pressure in both arms and both legs.

The doctor may suspect a coarctation if the blood pressure in the child’s legs is lower than the pressure in his arms. The baby’s femoral (thigh) or foot pulses will be weak and therefore very difficult for the doctor to feel. (Part of the newborn discharge exam is to be able to feel four good pulses.)

Other tests that help with the diagnosis—or with treatment planning—may include:

  • echocardiogram (echo, cardiac ultrasound): evaluates the structure and function of the heart by using sound waves, recorded on an electronic sensor, that produce a moving picture of the heart and heart valves. At Boston Children’s, the echocardiogram is the primary diagnostic tool to confirm a diagnosis of COA, especially in young children.
  • electrocardiogra (ECG or EKG): records the electrical activity of the heart, shows abnormal rhythms (arrhythmias) and detects heart muscle damage.
  • cardiovascular MRI (magnetic resonance imaging): a diagnostic procedure that uses a combination of large magnets, radiofrequencies and a computer to produce detailed images of organs and structures within the body.
  • chest x-ray: a diagnostic test that uses invisible electromagnetic energy beams to produce images of internal tissues, bones and organs onto film. At Boston Children’s, x-ray is used to diagnose COA only occasionally.

A word about prenatal diagnosis

COA can sometimes be detected prenatally through cardiac ultrasound: The test can pick up the size discrepancy between the left heart and the right heart, as well as the narrowing of the aortic arch. The aortic arch may not be obstructed in the fetus because of the ductus arteriosus, which is open (patent) in the fetus. But the obstruction can show up in the newborn after his ductus arteriosus closes.

So, while knowing in advance that the newborn will have COA can help doctors and families to be prepared for treatment after birth, there’s really no need to treat the fetus in utero.

Diagnosing the older child, teen or adult

Mild or moderate cases of COA may not be diagnosed until school-age, adolescence or even adulthood—whenever symptoms appear (sometimes it’s detected because of elevation on routine blood pressure measurement). As a child with COA grows, his aorta will not grow at the same rate; so with a relative narrowing later in life, signs and symptoms can appear, such as:

  • high blood pressure
  • nosebleeds
  • a heart murmur
  • headaches (from high blood pressure above the COA)
  • cramps in the lower sections of the body (from low blood pressure below the COA)

With the onset of symptoms, cardiologists will diagnose COA through physical examination and the use of advanced diagnostic tools.

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Coarctation of the aorta (COA) Glossary

aorta  one of the heart’s two great arteries. In a normal heart, the aorta arises from the left ventricle and pumps oxygen-rich blood out to the body. In COA, the aorta is narrowed and/or obstructed, usually just beyond the arch, preventing proper red blood flow out to the body.

bicuspid aortic valve  a defect of the aortic valve in which the valve has two flaps (leaflets) instead of the usual three. The aortic valve opens when the left ventricle pumps blood into the aorta, then closes after the blood is ejected to prevent the ejected blood from going back into the heart.

cardiac/cardio  pertaining to the heart 

cardiac catheterization  provides detailed visual information and measurements about the structures inside the heart, cardiac catheterization is a procedure that can be performed on a fetus, a baby, a child or an adult. Catheterization can be diagnostic-only, diagnostic and therapeutic, or diagnostic and interventional.

cardiac intervention
a minimally invasive procedure, such as cardiac catheterization or angioplasty, to diagnose and/or treat heart disease

cardiac surgery
surgical procedure performed on the heart

cardiac surgeon
doctor who performs surgery on the heart. A pediatric cardiac surgeon performs surgery on the hearts of infants and children.

cardiologist  doctor who diagnoses and treats heart problems non-surgically. A pediatric cardiologist treats infants and children with heart problems. 

cardiovascular MRI (magnetic resonance imaging)
a diagnostic procedure that uses a combination of large magnets, radiofrequencies and a computer to produce detailed images of organs and structures within the body. 

The Center for Families at Children’s  dedicated to helping families find the information, services and resources they need to understand their child’s medical condition and take part in their care 

coarctation of the aorta (COA)  a narrowing of the aorta, the main artery that delivers oxygen-rich (red) blood to the body. Although this narrowing can occur anywhere in the aorta, it’s usually found in the segment just beyond the aortic arch 

congenital heart defect  heart defect present at birth. The heart usually forms in the first eight weeks of fetal development. It is thought that most congenital heart defects develop during this period.

medical determination of illness or disease based on physical examinations and advanced technology diagnostic testing tools 

ductus arteriosus  the prenatal connection that ensures blood flow between the aorta and the pulmonary artery, which usually closes shortly after birth 

echocardiogram (echo, cardiac ultrasound)  a diagnostic tool that evaluates the structure and function of the heart using electronically recorded sound waves that produce a moving picture of your child’s heart and heart valves. 

electrocardiogram (ECG, EKG)  a diagnostic tool that evaluates the electrical activity of your child’s heart. 

(bacterial) endocarditis
inflammation of the heart lining

fetal echocardiogram (fetal ultrasound)  a diagnostic tool that uses sound waves to evaluate the structure and function of the baby’s heart while still in utero. The ultrasound can detect a COA defect in the fetus. 

fetal magnetic imaging (MRI)  a non-invasive diagnostic tool that uses 3-D imaging technology produced by magnets to accurately determine the blood flow and functioning of your child’s heart in utero. Drawing on our expertise in pediatric imaging, Children’s is setting a new standard in fetal MRI. 

interventional cardiologist  doctor who specializes in performing minimally invasive procedures, such as cardiac catheterization or angioplasty, to diagnose and treat heart disease 

native tissue  the body’s own tissue (as opposed to artificial tissue)

the presenting reasons why a child needs medical attention; something a patient feels, rather than something discovered by exam or testing. A symptom’s characteristics—including but not limited to onset, quality, triggers and severity—help diagnosticians to determine an illness or disease, or to determine what further testing is needed to determine the illness or disease. 

patent (open) ductus arteriosus (PDA)
a blood vessel (the ductus arteriosus) that connects the two great arteries (the aorta and the pulmonary artery) and usually closes soon after birth. As long as the PDA remains open (patent), blood can pass from the right ventricle and pulmonary artery to the aorta and body, allowing some oxygen-rich blood to circulate. 

perfusion  the passage of blood through the blood vessels of the circulatory system 

secondary defect  a defect that accompanies (“is secondary to”) a main defect 

stent  a rigid metal tube inserted during a catheterization procedure to widen or keep open  a blood vessel 

sporadic  occurring by chance, occasionally, not inherited 

VSD  ventricular septal defect; an opening in the wall (septum) between the left and right ventricles of the heart; often a secondary defect to a COA

x-ray  a diagnostic test that uses invisible electromagnetic energy beams to produce images of internal tissues, bones and organs onto film. 

We are grateful to have been ranked #1 on U.S. News & World Report's list of the best children's hospitals in the nation for the third year in a row, an honor we could not have achieved without the patients and families who inspire us to do our very best for them. Thanks to you, Boston Children's is a place where we can write the greatest children's stories ever told.”
- Sandra L. Fenwick, President and CEO

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