Coarctation of the Aorta
When a baby is born with a narrowed section of aorta (the large artery that delivers oxygen-rich blood from the heart to the rest of the body), it is a condition known as coarctation of the aorta. A congenital (or “present from birth”) heart anomaly, coarctation of the aorta is typically diagnosed in newborns, especially when the condition is severe enough to cause noticeable, life-threatening symptoms. If the narrowing is mild, however, the condition may not be diagnosed until later in childhood or even as a teen or an adult.
Most commonly, the narrowing occurs just below where the arteries branch off to bring oxygen-rich blood to the upper portion of the body, including the head and arms. For this reason, blood flow to the organs in the lower portion of the body and the legs is reduced. One sign of coarctation of the aorta is having high blood pressure readings in the arms and low blood pressure readings in the legs.
According to the Centers for Disease Control and Prevention (CDC), about 2,200 babies with coarctation of the aorta are born each year in the United States. It’s twice as common in males than in females, and many babies with this condition are also born with other heart-related birth defects. The most common associated condition is an alteration in the structure of the aortic valve, known as a bicuspid aortic valve.
Fortunately, surgery can restore blood flow throughout the body, effectively curing the condition.
What is coarctation of the aorta?
Coarctation of the aorta is a congenital anomaly that affects the heart’s structure. It occurs when the aorta, the body’s main artery, doesn’t form correctly during development in utero.
When the heart develops normally, there is no constriction, or narrowing, along the aorta, allowing oxygen-rich blood to flow freely throughout the body.
However, in a person with coarctation of the aorta, the aorta narrows near the ductus arteriosus, the blood vessel that connects the aorta to the left lung (pulmonary) artery. This structure exists as an open vessel during fetal development, allowing blood to bypass the lungs. In the first few days of a newborn’s life, that small vessel typically closes spontaneously. In a person with coarctation, the closure of the ductus arteriosus may worsen the narrowing of the aorta significantly.
Because the heart structure looks different when the ductus arteriosus is open in utero, doctors usually can’t confirm the diagnosis until after the baby is born. Diagnosis of a newborn with coarctation of the aorta may occur several days after delivery, once the ductus arteriosus closes and symptoms of coarctation of the aorta appear.
The narrowed section of aorta prevents sufficient oxygen-rich blood from reaching the lower half of the body, including the legs and critical organs such as the kidneys and liver. When this happens, a newborn’s heart begins to pump harder than it should under normal conditions. In severe cases, the heart muscle can become thickened and enlarged, leading to heart failure. Without treatment, it may lead to death.
If a person is born with mild or moderate coarctation of the aorta, however, doctors may not recognize the condition until years later, when routine tests uncover high blood pressure levels or when the person experiences symptoms, such as fainting, a heart murmur, or kidney problems, among others.
What causes coarctation of the aorta?
Doctors aren’t sure what causes coarctation of the aorta. It may be caused by a structural abnormality that only becomes evident once the ductus arteriosus closes.
What are the symptoms of coarctation of the aorta?
Newborns with coarctation of the aorta may have:
- Pale skin
- A heart murmur
- A quick heartbeat
- Difficulty breathing
- Increased sleepiness
- Poor feeding habits
Children, teens, or adults who have undiagnosed coarctation of the aorta may have:
- High blood pressure
- Chest pain
- Cold feet
- Shortness of breath
- Poor growth
- Failure to thrive
- Leg cramps or leg fatigue while exercising
- Kidney problems
What are the risk factors for coarctation of the aorta?
People are at risk of being born with coarctation of the aorta if they have one or both of the following:
- Other heart-related birth defects, such as a bicuspid aortic valve, congenital mitral valve stenosis, or certain types of ventricular septal defects
- Turner syndrome
How is coarctation of the aorta diagnosed?
A patient’s medical history, physical exam, and the results of diagnostic tests can be used to diagnose coarctation of the aorta.
If your newborn has developed symptoms of coarctation of the aorta, it’s important to tell the doctor which symptoms have arisen and how long they have been occurring. For older patients, tell your doctor if you have high blood pressure, a history of fainting, and kidney disease.
During the physical examination, doctors will look for symptoms, such as sweatiness or pale skin. They will also listen to your or your child’s heartbeat for signs of a possible heart murmur, as well as take blood pressure levels in your or your child’s arms and legs, looking for a difference between those two readings. (For those with the condition, blood pressure would be high in the arms and low in the legs.)
The following diagnostic tests may also be used to confirm the presence of coarctation of the aorta:
- Echocardiogram, an ultrasound of the heart, which will show structural abnormalities
- Cardiac catheterization, a thin, flexible tube threaded through a blood vessel in the groin until it reaches the heart; X-ray images are taken to check for structural problems.
- Electrocardiogram, which records and measures the heart’s electrical activity after electrodes are attached to the chest at different locations near the heart
- Computed tomography (CT) scan or magnetic resonance imaging (MRI), which shows images of the heart that can be used to identify structural abnormalities
How is coarctation of the aorta treated?
Surgery is needed to correct coarctation of the aorta and widen the narrowed section of artery. Patients may also receive medication to manage their symptoms until they undergo surgery.
The following medications may be prescribed to manage symptoms:
- Prostaglandin E1, which is typically given to newborns before surgery. It is used to maintain the opening of the ductus arteriosus and, potentially, re-open it if it has already closed, improving blood flow. This medication may also help the narrowed section of artery to relax, also improving blood flow.
- Beta-blockers to manage high blood pressure
- Diuretics to help patients with heart failure
- Milrinone, dopamine, or dobutamine, which also help to manage heart failure
The following procedures may be used to treat coarctation of the aorta:
- Surgery. During this procedure, doctors remove the narrowed segment of aorta and connect the wider ends that remain on either side. They may also use tissue from elsewhere in the body to create a patch that is attached to the aorta to widen the space. Newborns and young children are more likely to have this procedure.
- Balloon angioplasty with stenting. During this minimally invasive procedure, doctors thread a thin tube through a blood vessel in the groin until it reaches the heart. They insert a deflated balloon through the tubing, then inflate it at the site of the narrowed aorta to widen the space. A mesh stent is implanted to keep the area open. Adults, teens, and older children are more likely to undergo this procedure. This is also typically the preferred procedure for infants who have undergone surgical repair and experienced a re-narrowing of the aorta.
What is the outlook for people with coarctation of the aorta?
Once coarctation of the aorta is diagnosed and treated, a newborn, child, teen, or adult is expected to fully recover. Sometimes, the narrowed artery is repaired, but a person’s blood pressure levels remain high. In some cases, narrowing of the aorta may recur and need to be treated again. This is most commonly managed by an interventional procedure.
When coarctation of the aorta is repaired, an individual should be able to live their life fully, without restrictions on their activities after a brief recovery period. However, patients should see a cardiologist annually to ensure proper heart health.
What makes Yale unique in its treatment of coarctation of the aorta?
“The Yale Congenital Heart Center provides comprehensive care for patients diagnosed with coarctation of the aorta at any age, from before birth through adulthood,” says Yale Medicine pediatric cardiologist Dina Ferdman, MD. “Patients have access to a team-based approach at all stages. Our infants and children are cared for in collaboration with our Neonatology and Pediatric Cardiac Intensive Care colleagues. Adult patients come through our Adult Congenital Heart Program. Regardless of age, all patients have access to both advanced surgical and catheterization treatments and interventions. Our surgical and interventional teams work hand-in-hand to provide a patient-centered approach, so that each patient receives a targeted repair of their coarctation lesion. Follow up is lifelong, and with our Adult Congenital Heart Program, there is a seamless transition of care as patients grow.”