By: Prof. Dr. Seyed Saeid Zamanieh Shahri, MD  and  Prof. Dr. Sonia Sayyedalhosseini, MD

What is Coarctation of the aorta?

Aortic Coarctation refers to narrowing of the aortic artery. The aorta is a large blood vessel that branches from the heart and carries oxygen-rich blood to the body. When aortic Coarctation occurs, the heart pump must work harder to pump blood out of the narrowed part of the aorta. Aortic Coarctation in infants is generally present from birth and is congenital. Aortic Coarctation can be mild to severe and may not even be detected until puberty. The severity of aortic Coarctation depends on the degree of aortic artery stenosis.

It is often associated with other heart defects. While treatment of this condition is often successful, it requires lifelong care and follow-up.

Symptoms of aortic Coarctation:

Symptoms of aortic Coarctation disease depend on the severity of the condition. Most people with aortic Coarctation have no symptoms. In children, it may show symptoms from childhood, but in cases where the degree of narrowing is mild, aortic Coarctation may not be diagnosed until puberty. People may show signs of a heart defect that may be associated with aortic Coarctation. Babies with severe aortic Coarctation may show symptoms shortly after birth. These symptoms include:

• • Pale skin
• • Irritability
• • Severe sweating
• • Difficulty breathing
• • Difficulty eating

Failure to treat aortic Coarctation in children can lead to heart failure and death. Older children and adults with aortic Coarctation often do not show clinical symptoms; because the amount of aortic artery stenosis in them may be less severe. If you show signs of aortic Coarctation after birth, you will have high blood pressure mainly in the arms, but you will have low blood pressure in the lower limbs and legs. Symptoms of aortic Coarctation include:

• • High blood pressure
• • Headache
• • Muscle weakness
• • Cramps in the legs or cold feet
• • Nose bleeding
• • Chest pain

See a doctor right away if you or your child shows any of the following symptoms:

• • Severe chest pain and fatigue
• • Sudden shortness of breath
• • High blood pressure that has no specific justification Experiencing these symptoms does not

necessarily mean that you or your child will have a serious problem. But it is better to investigate the cause of these symptoms immediately. Early diagnosis and treatment can save your life.

Cause of aortic Coarctation

The cause of aortic Coarctation is not clear. For unknown reasons, mild to severe narrowing may occur in part of the aorta. Although aortic Coarctation disease can occur anywhere in the aorta, aortic Coarctation often occurs near a part called the ductus arteriosus. These conditions in aortic Coarctation usually occur before birth. Congenital heart defects are among the most common defects. Rarely, aortic Coarctation may be acquired in a person’s life. In rare cases, damage can lead to aortic Coarctation. For example, Takayasu arthritis is due to severe hardening of the arteries or conditions that cause inflammation of the arteries. It can lead to narrowing and aortic Coarctation.

Aortic Coarctation usually occurs farther away from the blood vessels that branch off to the upper body and also before the blood vessels that supply blood to the lower body. This is why blood pressure is high in the hands but low in the lower limbs such as the knees or feet. In left ventricular aortic Coarctation, the heart works harder to pump blood out of the narrowed part of the aorta, and blood pressure in the left ventricle rises. This is why the left ventricular wall thickens and becomes hypertrophic.

Risk factors in aortic Coarctation

Aortic Coarctation often occurs in conjunction with other congenital heart defects. However, doctors do not know what causes multiple heart defects. This condition is more common in boys than girls. Some of the heart conditions listed below makes people more prone to aortic Coarctation:

Double aortic valve: The aortic valve separates the lower part of the heart (left ventricle) from the aorta. Double-leaf aortic valves have two legs instead of three. Many people with aortic Coarctation have double aortic valves.

Ductus arteriosus: Ductus arteriosus is a blood vessel that connects the aorta to the left pulmonary artery and allows blood to enter the lungs. This pathway is blocked shortly after birth, and if it remains open, it is called an arterial canal. Large open arterial ducts, if left untreated, can reduce the oxygen-carrying capacity of the blood and flow in the wrong direction, weakening the heart muscle, which can lead to heart failure and other complications. Holes in the wall between the right and left sides of the heart: A person may have a hole in the wall between the upper or lower chambers of the heart at birth. This condition causes the oxygen-rich blood on the left side of the heart to mix with the less oxygenated blood on the right side of the heart. Aortic valve stenosis: In this condition, the valve that separates the left ventricle of the heart from the aorta is narrow, causing narrowing of the aortic artery. This condition makes the heart work harder to get enough blood to the body. Over time, aortic stenosis can cause the heart muscle to thicken, leading to symptoms such as chest pain, fatigue, shortness of breath, or heart failure. Return of blood from the aortic valve (Aortic Regurgitation): This condition occurs when the aortic valves do not close properly due to failure, resulting in blood flowing backwards and to the left ventricle. Mitral valve stenosis: Mitral valve stenosis occurs between the upper part of the heart (left atrium) and the left ventricle, which allows blood to flow to the left side of the heart. In this condition, blood may return to the lungs, causing shortness of breath or lung congestion. Like aortic valve blockage, this condition can lead to heart failure.

Return of blood to the mitral valve (Mitral Regurgitation): When the mitral valve does not close properly due to failure, blood leaks into the left atrium.

Aortic Coarctation is common in people with certain genetic conditions like Turner syndrome. Women and girls with Turner syndrome have 45 chromosomes and X chromosome is defective or missing. So these people have 45 chromosomes instead of 46. About 10% of women with Turner syndrome develop aortic Coarctation

Complications of aortic Coarctation disease
Failure to treat aortic Coarctation can lead to complications. Some of these complications can be the result of high blood pressure that lasts for a long time. After treatment of aortic Coarctation, the possibility of complications remains. Complications of aortic Coarctation include:

• • Narrowing of the aortic valve (aortic valve stenosis)
• • High blood pressure
• • Stroke
• • Enlargement of parts of the aortic wall
• • Aortic rupture
• • Premature arterial disease: refers to the narrowing of the blood vessels that supply blood to the heart
• • Heart failure
• • Arterial weakness in the brain (cerebral aneurysm) or bleeding in the brain

In addition, if the aortic Coarctation is severe, the heart may not be able to pump enough blood to other organs. As a result, the heart is damaged and may lead to kidney failure or failure of other organs. If aortic Coarctation is treated in a child, we will experience a narrowing of the aorta over time. There will also be a risk of high blood pressure. As a result, due to aortic Coarctation, the patient should be monitored over time and further treatment may be needed.

Detection of aortic Coarctation

If aortic Coarctation is severe, the diagnosis is usually made in childhood. It is not possible to perform a test to detect prenatal aortic Coarctation.

Milder forms of aortic Coarctation are more likely to occur when aortic Coarctation occurs in adults and older children. These people usually look healthy unless their doctor identifies the following manifestations:

• High blood pressure in the arms
• Blood pressure difference between arms and legs: Increased blood pressure in the arms and decreased blood pressure in the legs
• Weakness or delayed pulse in the legs
• Abnormal sound due to rapid blood flow in narrowed areas

Diagnostic tests:

Tests performed to confirm the diagnosis of aortic Coarctation include:

Echocardiogram: An echocardiogram uses sound waves to create an image of the heart. Sound waves propagate through the heart to create moving images that can be seen on a video moni-tor.

Echocardiogram can show the location and severity of aortic Coarctation. Other heart de-fects, such as the double-leaf aortic valve, are also detected in this test. Doctors often use echo-cardiograms to diagnose aortic Coarctation and determine the most appropriate treatment op-tion based on the findings.