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Coarctation of the Aorta

Conditions

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Overview 

Coarctation of the aorta is a congenital heart defect in which a portion of the aorta is narrower than it should be. The aorta is the major artery carrying oxygen-rich blood from the heart out to the body. 

When the aorta is narrowed, less blood can flow through (similar to a clogged pipe). The degree of narrowing can range from mild to severe. In cases of significant coarctation, surgery is required after birth to repair this defect. A child with greater narrowing of the aorta will have more symptoms. The symptoms will also be seen at an early age. In some cases, coarctation is seen in infancy. In others, it may not be seen until school-age or teenage years. Coarctation of the aorta may be diagnosed in infants, school-age children, or teens. 

Babies born with coarctation of the aorta frequently have other congenital heart defects, including abnormalities in the mitral valve and the aortic valve, and a ventricular septal defect (VSD), a hole in the wall between the heart’s lower chambers (ventricles). About half of children with coarctation of the aorta also have an abnormal mitral valve. This is the valve between the left upper and lower chambers of the hert.

The condition can also be associated with chromosomal abnormalities. 

What Causes Coarctation of the Aorta? 

Coarctation of the aorta is a congenital heart defect, meaning it is present at birth. The condition occurs early during fetal development when the baby’s aorta does not form properly.  

Most of the time, coarctation of the aorta has no known cause. Some heart defects present at birth have a genetic link. This means they happen more often in certain families. 

An estimated 1 in every 1,800 babies born in the United States each year are born with coarctation of the aorta, according to the Centers for Disease Control and Prevention (CDC). 

How does Coarctation of the Aorta Affect Babies? 

In a normal heart, the left ventricle pumps oxygen-rich blood out to the aorta. The aorta has several branches (called arteries) that arise from it, which in turn supply oxygen-rich blood to different areas of the body. 

In a baby with coarctation of the aorta, the narrowing in the aorta reduces blood flow (and thereby oxygen delivery) to the areas of the body beyond the narrowing. In most cases, this narrowing occurs just past the arteries that branch off and carry blood to the head and arms, but before the arteries that deliver blood to the abdomen, kidneys, legs and feet. As a result, the flow of oxygen-rich blood to the lower body is reduced. 

Complications may include: 

  • Damage to the organs in the lower body that don't get enough blood, such as the kidneys, intestines, and liver 
  • Weakened left ventricle (left-sided pumping chamber) as the heart has to work harder to push blood through the narrowed aorta 
  • Heart failure 

Coarctation of the Aorta Diagnosis 

The healthcare provider will ask about your child’s symptoms and health history. He or she will give your child a physical exam. The provider may refer you to a pediatric cardiologist is a doctor with special training to treat heart problems in children. The cardiologist will examine your child and listen to his or her heart and lungs. Your child may also have tests such as: 

  • Chest X-ray. A chest X-ray may show an enlarged heart or other changes caused by coarctation of the aorta. 
  • Electrocardiogram (ECG). An ECG records the electrical activity of the heart. It can find abnormal rhythms (arrhythmias) and heart muscle damage. 
  • Echocardiogram (echo). An echo uses sound waves to make a moving picture of the heart and heart valves. Most aortic coarctations are diagnosed by echocardiography. 
  • Cardiac catheterization (cath). A heart cath uses a thin, flexible tube (catheter) that is moved through the blood vessels into the heart. Dye is used to get a clear picture of the heart and aorta. Catheterization may also be used to correct the narrowing. 
  • MRI. MRI makes detailed pictures of the heart and aorta. It can clearly show where the narrowing is and how large it is. 

Coarctation of the Aorta Diagnosis During Pregnancy 

Coarctation of the aorta can be suspected during a routine prenatal ultrasound or after a fetal echocardiogram (focused ultrasound of the fetal heart performed by a fetal cardiologist). A diagnosis during pregnancy enables your family and your healthcare team to plan ahead for the specialized treatment and cardiac expertise your baby will need at birth, optimizing outcomes. 

Coarctation of the aorta is among the most challenging diagnoses to make because in fetal life the aorta can appear less narrow while the ductus arteriosus (a normal vessel connecting the pulmonary artery to the aorta) is open. Once this vessel closes after birth, the narrowing of the aorta becomes more evident. Therefore, in some cases, a coarctation may not be diagnosed until after birth. 

Coarctation of the Aorta Diagnosis After Birth 

The timing of diagnosis after birth will depend upon the severity of the narrowing and associated defects. 

If there is prenatal suspicion, newborns will undergo observation and testing in the hospital after birth. This includes frequent physical exams (monitoring of pulses and blood pressures in lower body) and echocardiography. 

In cases where there was not a prenatal suspicion, coarctation may be discovered because of an abnormal physical exam or after an abnormal routine newborn screening test (known as a pulse oximetry). Sometimes it is not diagnosed until after a baby develops complications from it. 

Coarctation of the Aorta Symptoms or signs may include: 

  • Weak or absent pulses in the feet 
  • Cool and/or pale legs and feet 
  • High blood pressure in the arms with low blood pressure in the legs 
  • Difficulty breathing or rapid breathing 
  • Irritability 
  • Poor feeding 
  • Decreased number of wet diapers 
  • Irritability 
  • Pale skin 
  • Sweating 
  • Heavy or fast breathing 
  • Poor feeding or eating 
  • Poor weight gain 

The more significant the narrowing of the aorta, the earlier the baby will have symptoms. Most critical cases come to attention within the first couple of days or weeks of life. In mild cases, a baby born with coarctation of the aorta may not have symptoms until adolescence or adulthood. These patients may present with high blood pressure, a heart murmur, or leg pain with exercise. Mild narrowing may not cause symptoms at all. The healthcare provider may find high blood pressure. Or he or she may hear an abnormal sound (heart murmur) when listening to your child's heart with a stethoscope. Some older children may complain of headaches or cramps in their lower body. 

Testing to confirm the diagnosis typically includes an echocardiogram, a chest x-ray, an electrocardiogram and in some cases a CT scan, MRI or cardiac catheterization. 

Specialized Evaluation and Prenatal Care for Coarctation of the Aorta Diagnosis 

If coarctation of the aorta is diagnosed during pregnancy, prompt referral to a fetal cardiac center is important for further evaluation and proper treatment planning. 

At Texas Children’s Fetal Center, we arrange for you to visit as quickly as possible for a comprehensive assessment by a team of specialists experienced in diagnosing and treating congenital heart defects. Your healthcare team will include maternal-fetal medicine physicians (OB/GYNs specializing in high-risk pregnancies), fetal and pediatric cardiologists, fetal imaging experts, geneticists, congenital heart surgeons and neonatologists. 

Additional testing may include: 

Our specialists will then meet with you about the results, provide treatment recommendations, and answer any questions your family has, to help you make the most informed decisions regarding your baby’s care and treatment. 

Delivery of Baby with Coarctation of the Aorta 

For the best possible outcomes, delivery should take place at a center with the highest quality congenital heart program, ensuring the expertise and resources required to treat coarctation of the aorta at birth, including an advanced neonatal intensive care unit (NICU) and cardiac intensive care unit (CICU). 

Delivery and postnatal care should be carefully planned and coordinated. Our Fetal Center team works closely with Texas Children’s Heart Center, seamlessly transitioning your baby’s care at delivery to this national leader in pediatric cardiology and heart surgery. Here, the heart specialists treating your newborn have been an integral part of their care team since before birth. 

Following delivery at Texas Children’s Pavilion for Women, newborns are transferred to Texas Children’s level IV NICU or Texas Children’s specialized CICU to receive the highest level of care available for premature and critically ill newborns. 

Coarctation of the Aorta Treatment 

The type and timing of treatment will depend on the severity of the condition, the presence of additional heart defects, the child’s age and overall health. 

Severe or critical coarctation of the aorta requires a surgery in the first several days of life. 

Prior to surgery patients will receive a special medication called prostaglandins (PGE) to either keep open (or re-open) a fetal blood vessel called a patent ductus arteriosus (PDA). The PDA is an artery present in fetal life that connects the pulmonary artery to the aorta; it normally closes after birth. When kept open (or re-opened) it will allow for the flow of blood around the area of narrowing in the aorta (think of an alternate, bypass road when there is a major traffic jam). This does not permit normal delivery of oxygen, because the oxygen level in the pulmonary artery (which carries oxygen-poor blood to the lungs) is less than that in the aorta, but it can stabilize the circulation and help reduce the risk of major complications. Babies often remain on this medication until the time of surgery.   

Treatment strategies include: 

  • Surgical repair. This is the most common approach if coarctation is diagnosed in infancy. A congenital heart surgeon removes the narrowed section of the aorta and sews or patches the two ends together. In some cases where there is underdevelopment of a larger portion of the aorta, the surgeon may repair and enlarge a larger portion of the aorta. 
  • Cardiac catheterization – balloon dilation. This is more commonly performed when coarctation is diagnosed later in children or young adults. During this procedure, a thin, flexible tube (catheter) is inserted into an artery in the groin and moved through the blood vessel to the narrowed section of the aorta. There is a balloon present on the end of the catheter which is then inflated to enlarge the narrowed portion of the aorta. In some cases, a small metal tube (stent) may also be inserted to keep the narrowed portion of the aorta open. 

Overall outcomes for cardiac catheterization and surgical repair are favorable. However, following repair, the infant or child may still require medications to treat high blood pressure. 

Babies born with coarctation of the aorta require close, lifelong monitoring by a cardiologist experienced in congenital heart conditions. In some cases, over time, the aorta may narrow again. 

Postnatal Care Team 

Depending on the severity of the condition, your baby’s postnatal care team may include: 

At Texas Children’s Heart Center, our pediatric cardiologists follow your child through adolescence, seamlessly transitioning their care at adulthood to a cardiologist who specializes in the treatment of adults with congenital heart defects.  

What are the complications of coarctation of the aorta? 

If untreated, coarctation of the aorta causes several problems: 

  • The left ventricle, which pumps blood through the aorta and to the body, becomes weak. 
  • Blood pressure is too high in the upper body and too low in the lower body. 
  • Kidneys don't work as they should. 
  • The aorta or other arteries can tear. This can cause uncontrolled bleeding or stroke. 

Living with Coarctation of the Aorta 

All children with coarctation of the aorta will need to have the defect fixed. Some infants will be very sick and need care in the intensive care unit (NICU) before the procedure. In some cases, a child may need emergency repair. Children who have few symptoms will have scheduled repairs. Most children who have had a coarctation of the aorta repair will live healthy lives. Activity levels, appetite, and growth should return to normal in time. 

After the repair, your child's cardiologist may recommend your child take antibiotics before surgeries or procedures, such as dental work. This is done to prevent infection. 

As the child grows, the aorta may again become narrow. If this happens, another balloon procedure or surgery may be done. 

Controlling blood pressure is very important. Your child's blood pressure may be higher after aortic coarctation repair. He or she may need to take medicines to help lower blood pressure. 

Your child will need regular follow-up care at a center offering pediatric or adult congenital cardiac care throughout life. 

Key points about coarctation of the aorta 

  • Coarctation of the aorta is a congenital heart defect. It means the aorta is narrower than it should be. 
  • Symptoms can vary from mild to more severe. They may include trouble breathing, poor weight gain, poor feeding, and pale skin. 
  • Coarctation of the aorta can be fixed by cardiac catheterization or surgery. 
  • After surgery, most children live healthy lives. 

Why Texas Children’s Fetal Center? 

  • A single location for expert maternal, fetal and pediatric care. Texas Children’s Hospital offers mothers and babies the specialized care required for the diagnosis and treatment of congenital heart conditions all in one location, for highly coordinated care and treatment planning. 
  • A skilled, experienced team with proven outcomes. We have a dedicated team of maternal-fetal medicine specialists, fetal imaging experts, fetal and pediatric cardiologists, congenital heart surgeons, neonatologists and others who work in concert to care for you and your baby every step of the way, using proven protocols we’ve developed over the years. With their combined expertise and unified approach, this team offers the best possible care for pregnancies involving coarctation of the aorta. 
  • We care for your child’s cardiac needs at every stage of life, from fetus to adulthood. Our comprehensive approach starts with your first prenatal visit and continues through delivery, postnatal care, childhood and adulthood, thanks to one of the nation’s leading teams of fetal, pediatric and adult congenital heart specialists. 

Texas Children’s – #1 in the Nation for Pediatric Cardiology and Heart Surgery 

Our Fetal Cardiology Program is a collaboration between Texas Children’s Fetal Center and Texas Children’s Heart Center, ranked #1 in the nation for pediatric cardiology and heart surgery by U.S. News & World Report, with outcomes among the best in the nation. 

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Texas Children’s, together with our partner institution Baylor College of Medicine, constantly strives to seek new and better treatments for babies with congenital heart defects. 

Learn more about our fetal cardiology research. 

For additional information, please contact the Cardiovascular Clinical Research Core at 832-826-2064 or email sandrea@texaschildrens.org.