Coarctation of the Aorta

An introduction to the Symptoms, Diagnosis and Treatment for Coarctation of the Aorta

Overview

Coarctation of the aorta — or aortic coarctation — is a narrowing of the aorta, the large blood vessel that branches off your heart and delivers oxygen-rich blood to your body. When this occurs, your heart must pump harder to force blood through the narrowed part of your aorta.

Coarctation of the aorta is generally present at birth (congenital). The condition can range from mild to severe, and might not be detected until adulthood, depending on how narrowed the aorta is.

Coarctation of the aorta often occurs along with other heart defects. While treatment is usually successful, the condition requires careful lifelong follow-up.

Coarctation of the Aorta

Symptoms

Symptoms that result from coarctation of the aorta depend on the severity of the condition. Most people don’t have symptoms. Children with serious aortic narrowing may show signs and symptoms earlier in life, but those with mild narrowings may not have symptoms and might not be diagnosed until adulthood. People may have signs or symptoms of other heart defects that they have along with coarctation of the aorta. A significant proportion of people with aortic coarctation will also have a bicuspid aortic valve (see section).

Babies with severe coarctation of the aorta may begin having signs and symptoms shortly after birth. These include:

  • Pale skin
  • Irritability
  • Heavy sweating
  • Difficulty breathing
  • Difficulty feeding

Left untreated, aortic coarctation in babies might lead to heart failure or death.

Older children and adults with coarctation of the aorta often don’t have symptoms because their narrowing may be less severe. However, people with milder coarctation may develop high blood pressure (hypertension) measured in your arms.

Signs and symptoms might include:

  • High blood pressure
  • Headache
  • Muscle weakness
  • Leg cramps or cold feet
  • Nosebleeds
  • Chest pain

 

Diagnosis

The age at which coarctation of the aorta is diagnosed depends on the severity of the condition. If the aortic coarctation is severe, it’s usually diagnosed during infancy. Testing for coarctation of the aorta before birth often isn’t possible.

Adults and older children who are diagnosed with coarctation of the aorta may have milder cases and not have symptoms. They may often appear healthy until a doctor detects:

  • High blood pressure in the arms
  • A blood pressure difference between the arms and legs, with higher blood pressure in the arms and lower blood pressure in the legs
  • A weak or delayed pulse in the legs
  • A heart murmur — an abnormal whooshing sound caused by faster blood flow through the narrowed area

Diagnostic tests

Tests to confirm a diagnosis of coarctation of the aorta may include:

  • Echocardiogram: Echocardiograms use high-pitched sound waves to produce an image of your heart. Sound waves bounce off your heart and produce moving images that can be viewed on a video screen. An echocardiogram can often detect the location and severity of the aortic coarctation and show other heart defects, such as a bicuspid aortic valve. Doctors often use echocardiograms to diagnose coarctation of the aorta and determine the most appropriate treatment options for you.
  • Electrocardiogram (ECG): An ECG records the electrical activity in your heart each time it contracts. During this procedure, you will have patches with wires (electrodes) placed on your chest, wrists and ankles. The electrodes measure electrical activity, which is recorded on paper or a computer monitor. If the coarctation of the aorta is severe, the ECG may show that the walls of the lower heart chambers are thickened (ventricular hypertrophy).
  • Chest X-ray: A chest X-ray creates images of your heart and lungs. A chest X-ray might show a narrowing in the aorta at the site of the coarctation or an enlarged section of the aorta or both.
  • Magnetic resonance imaging (MRI): An MRI uses a powerful magnetic field and radio waves to produce detailed images of your heart and blood vessels. It can reveal the location and severity of the coarctation of the aorta, determine whether it affects other blood vessels in your body, and detect whether you have other heart defects. Doctors may also use this test to determine your treatment options.
  • Computerized tomography (CT) scan: A CT scan uses a series of X-rays to create detailed cross-sectional images of your body. In a CT angiogram, your doctor injects a dye into a blood vessel to highlight blood flow in your arteries and veins. A CT angiogram allows your doctor to see the location and severity of the coarctation of the aorta, determine whether it affects other blood vessels in your body, and detect other heart defects. Your doctor may also use this test to determine your treatment options.
  • Cardiac catheterization: During this procedure, your doctor inserts a long, thin tube (catheter) into an artery or vein in your groin, arm or neck and threads it to your heart using X-ray imaging. Your doctor may inject a dye through the catheter to make your heart structures visible on X-ray pictures. The dye can also measure pressures and oxygen levels in the chambers of the heart and in the blood vessels. Cardiac catheterization can help determine the severity of the aortic coarctation. This test isn’t often used to diagnose coarctation of the aorta, but your doctor may use it to help plan surgery or other treatment, if you need it. Catheter procedures may be used to perform certain treatments for coarctation of the aorta.

Treatment

Treatment options for coarctation of the aorta depend on your age at the time of diagnosis and the severity of your condition. Other heart defects might be repaired at the same time as aortic coarctation.

Treatment approaches usually consist of surgery or a procedure called stent placement. A doctor trained in congenital heart conditions will evaluate you and determine the most appropriate treatment for your condition.

Surgery

There are several surgical techniques to repair aortic coarctation. Your doctor can discuss which type is most likely to successfully repair you or your child’s condition. The options include:

  • Resection with end-to-end anastomosis: This method involves removing the narrowed segment of the aorta (resection) followed by connecting the two ends of the aorta together (anastomosis).
  • Subclavian flap aortoplasty: A portion of the blood vessel that delivers blood to your left arm (left subclavian artery), might be used to expand the narrowed area of the aorta.
  • Bypass graft repair: This technique involves bypassing the narrowed area by inserting a plastic tube called a graft between the portions of the aorta.

Balloon angioplasty and stenting

Balloon angioplasty, often with stenting, is an option for initially treating aortic coarctation or for treating re-narrowing (re-coarctation) that has occurred after surgery. During this procedure, your doctor inserts a thin, flexible tube (catheter) into an artery in your groin and threads it through your blood vessels to your heart using X-ray imaging.

Your doctor places an uninflated balloon through the opening of the narrowed aorta. When the balloon is inflated, the aorta widens and blood flows more easily. Your doctor then inserts a mesh-covered hollow tube (stent) in the aorta to keep the narrowed part of the aorta open.

Medication

Medication isn’t used to repair coarctation of the aorta, but it might be used to control blood pressure before and after stent or surgery. Although repairing aortic coarctation improves blood pressure, many people still need to take blood pressure medication, even after a successful surgery or stenting.

 

Follow-up Care

Even after your coarctation has been repaired- either with surgery or a stenting procedue- you should still have lifelong follow up with a congenital heart specialist.

The most common long-term complication of coarctation of the aorta is high blood pressure. Although your blood pressure usually falls after the aortic coarctation has been repaired, it might still remain higher than normal.

Occasionally, the segment of the aorta that has been repaired will become weak and enlarge (aortic aneurysm) and might eventually rupture.

In some cases, the coarctation can recur, possibly even years after treatment. It’s possible to have additional surgeries or procedures to correct the re-narrowing or treat other complications.

Conditions We Treat

Pulmonary Valve Stenosis

Pulmonary valve stenosis is a condition in which a deformity on or near your pulmonary valve narrows the pulmonary valve opening and slows the blood flow. The pulmonary valve is located between the lower right heart chamber (right ventricle) and the pulmonary arteries. Adults occasionally have pulmonary valve stenosis as a complication of another illness, but mostly, pulmonary valve stenosis develops before birth as a congenital heart defect. Pulmonary valve stenosis ranges from mild to severe. Mild pulmonary stenosis doesn't usually worsen over time, but moderate and severe cases may worsen and require surgery. Fortunately, treatment is generally highly successful, and most people with pulmonary valve stenosis can expect to lead normal lives. Find Out More >>

Patent Foramen Ovale

A patent foramen ovale (PFO) is a hole in the heart that didn't close the way it should have after birth. During fetal development, a small flap-like opening — the foramen ovale — is present in the wall between the right and left upper chambers of the heart (atria). It normally closes during infancy. When the foramen ovale doesn't close, it's called a patent foramen ovale. Patent foramen ovale occurs in about 25% of the normal population, but most people with the condition never know they have it. Learning that you have a patent foramen ovale is understandably concerning, but most people never need treatment for this condition. A patent foramen ovale is often discovered during tests for other problems. In certain conditions, such as unexplained strokes (cryptogenic stroke) in young people, closure might be recommended by your doctor. Find Out More >>

Tetralogy of Fallot

Tetralogy of Fallot is a rare condition caused by a combination of four heart defects that are present at birth (congenital). These defects, which affect the structure of the heart, cause oxygen-poor blood to flow out of the heart and to the rest of the body. Tetralogy of Fallot is usually diagnosed during infancy or soon after. However, it might not be detected until later in life in some adults, depending on the severity of the defects and symptoms. With early diagnosis followed by appropriate surgical treatment, most people who have Tetralogy of Fallot live relatively normal lives. Many adults with repaired Tetralogy of Fallot will require another procedure or intervention during their lifetimes. It is important to have regular follow-up with a cardiologist trained in congenital heart disease who can evaluate you and determine the appropriate timing of another intervention or procedure. At the Irish Congenital Heart Centre, our specialists have trained in congenital heart disease and are expert at caring for people with Tetralogy of Fallot. Find Out More >>

Partial Anomalous Pulmonary Venous Return

Partial anomalous pulmonary venous return, sometimes called partial anomalous pulmonary venous connection, is a heart defect present at birth (congenital) in which some of the pulmonary veins carrying blood from the lungs to the heart flow into other blood vessels or into the heart's upper right chamber (right atrium), instead of correctly entering the heart's upper left chamber (left atrium). This causes some oxygen-rich blood from the lungs to mix with oxygen-poor blood before entering the right atrium. Some people with this defect also have a hole between the upper heart chambers (atrial septal defect), which allows blood to flow between the upper heart chambers (atria). People with this condition may also have other congenital heart defects. Find Out More >>

Ebstein Anomaly

Ebstein anomaly is a rare heart defect that's present at birth (congenital). In Ebstein anomaly, your tricuspid valve — the valve between the two right heart chambers (right atrium and right ventricle) — doesn't work properly. The tricuspid valve sits lower than normal in the right ventricle, and the tricuspid valve's leaflets are abnormally formed. Blood may leak back through the valve, making your heart work less efficiently. Ebstein anomaly may also lead to enlargement of the heart or heart failure. A significant proportion of people with Ebstein anomaly will also have a communication between the top two chambers of the heart - an atrial septal defect (ASD). Abnormal electrical activity of the heart, leading to abnormal heartbeats (arrhythmia) is also a common problem for people with Ebstein anomaly. If you have no signs or symptoms associated with Ebstein anomaly, careful monitoring of your heart may be all that's necessary. If signs and symptoms bother you, or if the heart is enlarging or becoming weaker, treatment for Ebstein anomaly may be necessary. Treatment options include medications and surgery. Find Out More >>

Atrial Septal Defect

An atrial septal defect (ASD) is a hole in the wall between the two upper chambers of your heart (atria). The condition is present at birth (congenital). Small defects may never cause a problem and may be found incidentally. It's also possible that small atrial septal defects may close on their own during infancy or early childhood. Large and long-standing atrial septal defects can damage your heart and lungs. An adult who has had an undetected atrial septal defect for decades may have a shortened life span from heart failure or high blood pressure that affects the arteries in the lungs (pulmonary hypertension). Surgery may be necessary to repair atrial septal defects to prevent complications. Find Out More >>

Atrioventricular Canal Defect

Atrioventricular canal defect is a combination of heart problems resulting in a defect in the centre of the heart. The condition occurs when there's a hole between the heart's chambers and problems with the valves that regulate blood flow in the heart. Sometimes called endocardial cushion defect or atrioventricular septal defect, atrioventricular canal defect is present at birth (congenital). The condition is often associated with Down Syndrome. Atrioventricular canal defect allows extra blood to flow to the lungs. The extra blood forces the heart to overwork, causing the heart muscle to enlarge. Untreated, atrioventricular canal defect can cause heart failure and high blood pressure in the lungs. Doctors generally recommend surgery during the first year of life to close the hole in the heart and to reconstruct the valves. Find Out More >>

Bicuspid Aortic Valve

Bicuspid aortic valve is the commonest form of congenital heart disease. Approximately 1% of the population are born with this condition. A bicuspid aortic valve is an aortic valve that has only two leafelts (bicuspid) instead of the normal three leaflets (tricuspid) . Some people may also be born with one (unicuspid) or four (quadricuspid) leaflets, but these are rare. A bicuspid aortic valve may cause the heart's aortic valve to narrow (aortic valve stenosis). This narrowing prevents the valve from opening fully, which reduces or blocks blood flow from the heart to the body. In some cases, the bicuspid aortic valve doesn't close efficiently, causing blood to leak backward into the left ventricle (aortic valve regurgitation). Some people with a bicuspid aortic valve may develop an enlarged aorta — the main blood vessel leading from the heart (see also  our Aortopathy section) Most people with a bicuspid aortic valve aren't affected by valve problems until they are adults, and some may not be affected until they are older adults. However if you are known to have a biscupid aortic valve, you should be followed up by a cardiologist at regular intervals. Find Out More >>

Coarctation of the Aorta

Coarctation of the aorta — or aortic coarctation — is a narrowing of the aorta, the large blood vessel that branches off your heart and delivers oxygen-rich blood to your body. When this occurs, your heart must pump harder to force blood through the narrowed part of your aorta. Coarctation of the aorta is generally present at birth (congenital). The condition can range from mild to severe, and might not be detected until adulthood, depending on how narrowed the aorta is. Coarctation of the aorta often occurs along with other heart defects. While treatment is usually successful, the condition requires careful lifelong follow-up. Find Out More >>

Ventricular Septal Defect

A ventricular septal defect (VSD) is a type of hole in the heart. It is present at birth (congenital). The hole (defect) occurs in the wall (septum) that separates the heart's lower chambers (ventricles) and allows blood to pass from the left to the right side of the heart. This means that oxygen-rich blood then gets pumped back to the lungs instead of out to the body, causing the heart to work harder. A small ventricular septal defect may cause no problems, and many small VSDs close on their own. Medium or larger VSDs may need surgical repair early in life to prevent complications. Find Out More >>

Transposition of the Great Arteries

Transposition of the great arteries is a serious but rare heart defect present at birth (congenital), in which the two main arteries leaving the heart are reversed (transposed). The condition is also called dextro-transposition of the great arteries. A rarer type of this condition is called levo-transposition of the great arteries. Transposition of the great arteries changes the way blood circulates through the body, leaving a shortage of oxygen in blood flowing from the heart to the rest of the body. Without an adequate supply of oxygen-rich blood, the body can't function properly and your child faces serious complications or death without treatment. Transposition of the great arteries is usually detected either prenatally or within the first hours to weeks of life. Corrective surgery soon after birth is the usual treatment for transposition of the great arteries, and with proper treatment, the outlook is promising. Even when this condition is diagnosed and treated early in childhood, there is a potential to develop complications later in life. All people with Transposition of the Great Arteries should have lifelong follow up with a congenital heart specialist. Our experts at the Irish Congenital Heart Centre are trained in congenital cardiology and have vast experience in treating people with Transposition of the Great Arteries. Find Out More >>

Hypoplastic Left Heart Syndrome

Hypoplastic left heart syndrome is a complex and rare heart defect present at birth (congenital). The left side of the heart is critically underdeveloped in hypoplastic left heart syndrome. If your baby is born with hypoplastic left heart syndrome, the left side of the heart can't effectively pump blood to the body. Instead, the right side of the heart must pump blood to the lungs and to the rest of the body. Medication to prevent closure of the connection (ductus arteriosus) between the right and left sides, followed by either surgery or a heart transplant, is necessary to treat hypoplastic left heart syndrome. With advances in care, the outlook for babies born with hypoplastic left heart syndrome is better now than in the past. All people who are born with hypoplastic left heart syndrome will have undergone multiple complex cardiac surgeries and require lifelong follow up by a specialist congenital heart expert. Find Out More >>

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