Specialized care for children with the congenital heart defect coarctation of the aorta 

For children with coarctation of the aorta — a heart defect in which a narrowed aorta affects the way blood is delivered from the heart to the rest of the body — the team in the Heart Institute at Johns Hopkins All Children’s Hospital in St. Petersburg, Florida, provides expert pediatric cardiology and surgical care to repair the defect and improve blood flow.

What is coarctation of the aorta? 

The aorta is the main artery that carries blood away from the heart to the rest of the body. Blood vessels branch off the aorta to supply blood throughout the body. Coarctation of the aorta is the narrowing of the main blood vessel that supplies blood to the body. 

The coarctation acts similarly to a hose with a kink in it that prevents water from flowing freely through the hose. The “kink” or coarctation of the aorta blocks blood flow to the lower half of the body. This causes high blood pressure in the upper part of the body before the coarctation, and low to no pressure in the lower half. 

Coarctation of the aorta can be associated with other conditions such as Turner syndrome or the congenital heart defect bicuspid aortic valve.

Interrupted aortic arch 

The most severe and life-threatening form of coarctation of the aorta is called “interrupted aortic arch.” With interrupted aortic arch, rather than a narrowed vessel, the aorta is incomplete or interrupted at a certain point so that no blood flows to the lower part of the body. The top of the aorta delivers blood to the upper body, but the lower body needs to receive blood from another source (usually a patent ductus arteriosus, or PDA). Interrupted aortic arch can be associated with DiGeorge syndrome, as well as an atrial septal defect (ASD), ventricular septal defect (VSD) or truncus arteriosus.

Symptoms of coarctation of the aorta 

In newborns, symptoms of coarctation of the aorta may include: 

• Poor feeding (not enough blood is able to flow to the stomach so this inhibits digestion)
• Weak femoral artery (taken in the groin area) or dorsal pedis (taken in the foot) pulse
• Fast breathing or labored breathing 
• Skin of lower extremities (legs, feet) looks pale or grey 

In severe cases, including interrupted aortic arch, additional symptoms to those above may include: 

• Pale appearance with poor perfusion 
• Lethargic or sluggish/tired 
• Low oxygen levels especially in the legs and feet

How is coarctation of the aorta diagnosed?

Coarctation of the aorta may sometimes be diagnosed before birth during a prenatal ultrasound, although this is very difficult. However, most cases are often not definitely diagnosed until after birth. 

All babies are born with what’s called a patent ductus arteriosus (PDA). This is an opening between the pulmonary artery and the aorta that allows blood to flow directly from the heart to the lungs before the baby is born. In babies with coarctation of the aorta, the PDA helps to supply blood to the lower parts of the body while the baby is still in-utero. 

A baby’s PDA should close within the first few days after birth. At that point, if coarctation of the aorta is suspected, an echocardiogram is used to diagnose the condition. Babies may also need a CT scan which allows doctors to obtain a more detailed image of the entire aortic arch.

More mild cases may not be detected until the child is older. Your child’s pediatrician may note that your child has high blood pressure or a weaker pulse in their leg and thus may have a coarctation. 

Interrupted aortic arch can often be diagnosed prenatally with a fetal echocardiogram. At Johns Hopkins All Children’s Hospital, our Fetal Heart Program team provides expert diagnosis using fetal echocardiogram as well as management before birth of congenital heart defects.

How is coarctation of the aorta treated? 

Treatment may depend on your child’s age when they are diagnosed and the severity of their condition. Immediate treatment includes a prostaglandin infusion. Prostaglandin is a medicine that is given intravenously (IV). It keeps the ductus arteriosus open. This allows blood flow to the lower body until surgery is done to fix the coarctation or interruption in the aortic arch. Severe cases of coarctation of the aorta, including interrupted aortic arch, require surgery to repair the defect in the first few days of life.

Your child’s heart surgeon will remove the narrowed portion of the aorta and reconnect the two ends. In cases of interrupted aortic arch your child’s surgeon may have to reconstruct part of the aorta with a patch because there is not enough existing tissue. 

In older children, it may be treated with a procedure to insert a very small balloon to inflate and open the narrowed portion of the aorta to improve blood flow. In adolescents and young adults, a stent (small metal tube) may also be placed after the balloon inflates the affected area to keep the blood vessel open.

Follow-up care 

After your child’s surgery, they will be cared for in our cardiovascular intensive care unit. Your child’s blood pressure is monitored to make sure there is no longer a difference between the pressure in the upper and lower parts of the body, and we will perform an echocardiogram to check how your child’s repaired aorta is functioning.

After discharge, your child will continue to be monitored regularly by their pediatric cardiologist. Sometimes the aorta may start to narrow again as the child gets older and older children and young adults who received a surgical repair as babies may require a balloon and/or stent placement later in life.

Adults who have been treated for coarctation of the aorta have a higher risk of developing high blood pressure, early coronary disease, or a re-narrowing of the aorta or dilation at the repair site. Patients can continue to receive care from our experts as adults in the Adult Congenital Heart Disease Program. Adults with congenital heart defects benefit from seeing a provider who understands how congenital heart defects can impact health long term.