The aorta narrows, causing the heart to pump harder to push blood through the narrowed aorta to get adequate blood flow to the lower half of the body.
Infants with severe coarctation become suddenly ill when they are a few days old, showing signs of heart failure and decreased blood flow to the lower body. Older children with coarctation often have no symptoms.
The diagnosis is suspected based on symptoms and findings during a doctor's examination and is confirmed with echocardiography.
Most children with coarctation need corrective surgery or balloon angioplasty of the narrowed aorta.
(See also Overview of Heart Defects Overview of Heart Defects About one in 100 babies is born with a heart defect. Some are severe, but many are not. Defects may involve abnormal formation of the heart's walls or valves or of the blood vessels that enter... read more .)
Coarctation, a heart birth defect, is narrowing of just one segment of the aorta, not the whole aorta. The narrowing is typically located across from the point where the ductus arteriosus joins the aorta. The ductus arteriosus is a blood vessel connecting the two great arteries leaving the heart, the pulmonary artery and the aorta (see Normal Fetal Circulation Normal Fetal Circulation About one in 100 babies is born with a heart defect. Some are severe, but many are not. Defects may involve abnormal formation of the heart's walls or valves or of the blood vessels that enter... read more ). In the womb and during the first few days of life, the ductus is open, so blood flowing through it bypasses the narrowed section of aorta.
Coarctation may reduce blood flow to the lower half of the body, including the kidneys, liver, and other organs in the abdomen. The blood pressure is lower than normal in the legs and tends to be higher than normal in the arms. Coarctation is a serious but treatable cause of high blood pressure High Blood Pressure High blood pressure (hypertension) is persistently high pressure in the arteries. Often no cause for high blood pressure can be identified, but sometimes it occurs as a result of an underlying... read more . A heart murmur (a sound created by turbulent blood flow through narrowed or leaking heart valves or through abnormal heart structures) is sometimes present.
Without treatment, severe coarctation can be fatal in newborns. Less severe coarctation eventually strains and enlarges the heart and causes high blood pressure. Coarctation makes the child susceptible to heart infection (bacterial endocarditis Infective Endocarditis Infective endocarditis is an infection of the lining of the heart (endocardium) and usually also of the heart valves. Infective endocarditis occurs when bacteria enter the bloodstream and travel... read more ) and bleeding in the brain. Children with coarctation often have other heart defects, such as aortic valve stenosis Aortic Valve Stenosis in Children Aortic valve stenosis is a narrowing of the valve that opens to allow blood to flow from the left ventricle into the aorta and then to the body. This defect makes the heart work harder to pump... read more or an atrial or a ventricular septal defect Atrial and Ventricular Septal Defects Atrial and ventricular septal defects are holes in the walls (septa) that separate the heart into the left and right sides. Holes can be present in the walls of the heart between the upper heart... read more .
Infants with mild coarctation usually have few or no symptoms. Infants with moderate coarctation may have rapid, sometimes labored breathing, a fast heart beat, poor feeding, grayish skin coloration, decreased number of wet diapers, and irritability or lethargy.
With a severe coarctation in infancy, blood can flow to the lower portion of the aorta (at a point past its narrowing) through the open connection between the aorta and the pulmonary artery, the ductus arteriosus. Symptoms usually do not occur until the ductus closes, usually when the newborn is a few days old. After the closure, the blood supplied through the ductus disappears, sometimes causing sudden loss of almost the entire blood supply to the lower body. Sudden, catastrophic heart failure Heart Failure (HF) Heart failure is a disorder in which the heart is unable to keep up with the demands of the body, leading to reduced blood flow, back-up (congestion) of blood in the veins and lungs, and/or... read more and low blood pressure Low Blood Pressure Low blood pressure is blood pressure low enough to cause symptoms such as dizziness and fainting. Very low blood pressure can cause damage to organs, a process called shock. Various drugs and... read more can result.
Most older children with coarctation do not have symptoms. Occasionally, children with coarctation have headaches or nosebleeds because of high blood pressure in the upper body or leg pains during exercise because the blood supply to the legs is insufficient.
Coarctation is usually suspected when a doctor notices a heart murmur or differences in pulses or blood pressures between the arms and legs when doing a physical examination. A heart murmur is a sound created by turbulent blood flow through narrowed or leaking heart valves or through abnormal heart structures. In addition, the blood pressure measured in the arms may be quite high.
Echocardiography Echocardiography and Other Ultrasound Procedures Ultrasonography uses high-frequency (ultrasound) waves bounced off internal structures to produce a moving image. It uses no x-rays. Ultrasonography of the heart (echocardiography) is one of... read more (ultrasonography of the heart) and, sometimes, CT or MRI confirms the diagnosis.
Electrocardiography Electrocardiography Electrocardiography (ECG) is a quick, simple, painless procedure in which the heart’s electrical impulses are amplified and recorded. This record, the electrocardiogram (also known as an ECG)... read more (ECG) and chest x-rays X-Rays of the Chest Anyone thought to have a heart disorder has chest x-rays taken from the front and the side. Typically, the person is standing upright, but chest x-rays can be done with people lying in bed if... read more are typically done. They may show an enlarged heart
Treatment depends on the severity of the coarctation and the symptoms it causes.
Infants with severe symptoms due to coarctation require emergency treatment, including
Surgery is the preferred treatment for moderate or severe coarctation in infants and small children. The narrow portion of the aorta may be cut out and the two normal sections then sewn together or a patch can be used to enlarge the narrow portion of the aorta. Sometimes, tissue from the blood vessel supplying the left arm (the subclavian artery) is used to create this patch.
A very mild coarctation that causes no symptoms may not get detected early in life. A very mild coarctation may get noticed when children are somewhat older and the aorta has become narrower. A doctor may notice stronger pulses and a higher blood pressure in the arm than the leg. In such children, surgery and balloon angioplasty are options for repairing the narrowed aorta. Balloon angioplasty is done during cardiac catheterization, in which a thin tube (catheter) with a balloon at its tip is passed through a blood vessel in the arm or leg into the narrowed aorta. The balloon is inflated, widening the narrowed aorta. Sometimes, an expandable flexible tube (stent) is inserted to help keep the aorta from narrowing again.
Some children who had surgery for coarctation develop scar tissue in the aorta at the site of repair. Such scar tissue narrows the aorta and develops most commonly in infants who needed emergency surgery very early in life. Balloon angioplasty with or without stent placement is usually a very effective treatment for this recurrent narrowing.
Children need to take antibiotics before visits to the dentist and before certain surgeries (such as on the respiratory tract) for the 6-month period following repair. These antibiotics are used to prevent serious heart infections called endocarditis Infective Endocarditis Infective endocarditis is an infection of the lining of the heart (endocardium) and usually also of the heart valves. Infective endocarditis occurs when bacteria enter the bloodstream and travel... read more .
The following are some English-language resources that may be useful. Please note that THE MANUAL is not responsible for the content of these resources.
American Heart Association: Common Heart Defects: Provides an overview of common birth defects of the heart for parents and caregivers
American Heart Association: Infective Endocarditis: Provides an overview of infective endocarditis, including summarizing antibiotic use, for parents and caregivers