Coarctation of the aorta

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Aortic coarctation
Other namesAortic narrowing
Blausen 0243 CoarctationofAorta CloseUp.png
Illustration depicting coarctation of the aorta
Specialty Interventional Cardiology

Coarctation of the aorta (CoA [1] [2] or CoAo), also called aortic narrowing, is a congenital condition whereby the aorta is narrow, usually in the area where the ductus arteriosus (ligamentum arteriosum after regression) inserts. The word coarctation means "pressing or drawing together; narrowing". Coarctations are most common in the aortic arch. The arch may be small in babies with coarctations. Other heart defects may also occur when coarctation is present, typically occurring on the left side of the heart. When a patient has a coarctation, the left ventricle has to work harder. Since the aorta is narrowed, the left ventricle must generate a much higher pressure than normal in order to force enough blood through the aorta to deliver blood to the lower part of the body. If the narrowing is severe enough, the left ventricle may not be strong enough to push blood through the coarctation, thus resulting in a lack of blood to the lower half of the body. Physiologically its complete form is manifested as interrupted aortic arch.[ citation needed ]



Illustration showing a heart with a coarctation of the aorta Coarctation Of Aorta.png
Illustration showing a heart with a coarctation of the aorta
Sketch showing heart with coarctation of the aorta. A: Coarctation (narrowing) of the aorta. 1:Inferior vena cava, 2:Right pulmonary veins, 3: Right pulmonary artery, 4:Superior vena cava, 5:Left pulmonary artery, 6:Left pulmonary veins, 7:Right ventricle, 8:Left ventricle, 9:Pulmonary artery, 10:Aorta Coarctation.png
Sketch showing heart with coarctation of the aorta. A: Coarctation (narrowing) of the aorta. 1:Inferior vena cava, 2:Right pulmonary veins, 3: Right pulmonary artery, 4:Superior vena cava, 5:Left pulmonary artery, 6:Left pulmonary veins, 7:Right ventricle, 8:Left ventricle, 9:Pulmonary artery, 10:Aorta
Schematic drawing of alternative locations of coarctation of the aorta, relative to the ductus arteriosus. A: Ductal coarctation, B: Preductal coarctation, C: Postductal coarctation. 1: Aorta ascendens, 2: Arteria pulmonalis, 3: Ductus arteriosus, 4: Aorta descendens, 5: Truncus brachiocephalicus, 6: Arteria carotis communis sinistra, 7: Arteria subclavia sinistra Coarctation and PDA.png
Schematic drawing of alternative locations of coarctation of the aorta, relative to the ductus arteriosus. A: Ductal coarctation, B: Preductal coarctation, C: Postductal coarctation. 1: Aorta ascendens, 2: Arteria pulmonalis, 3: Ductus arteriosus, 4: Aorta descendens, 5: Truncus brachiocephalicus, 6: Arteria carotis communis sinistra, 7: Arteria subclavia sinistra
3D model of coarctation of aorta Coarctation of aorta 3DSR File Nevit Dilmen.stl
3D model of coarctation of aorta

There are three types of aortic coarctations: [3]

  1. Preductal coarctation: The narrowing is proximal to the ductus arteriosus. Blood flow to the aorta that is distal to the narrowing is dependent on the ductus arteriosus; therefore severe coarctation can be life-threatening. Preductal coarctation results when an intracardiac anomaly during fetal life decreases blood flow through the left side of the heart, leading to hypoplastic development of the aorta. This is the type seen in approximately 5% of infants with Turner syndrome. [4] [5]
  2. Ductal coarctation: The narrowing occurs at the insertion of the ductus arteriosus. This kind usually appears when the ductus arteriosus closes.
  3. Postductal coarctation: The narrowing is distal to the insertion of the ductus arteriosus. Even with an open ductus arteriosus, blood flow to the lower body can be impaired. This type is most common in adults. It is associated with notching of the ribs (because of collateral circulation), hypertension in the upper extremities, and weak pulses in the lower extremities. Postductal coarctation is most likely the result of the extension of a muscular artery (ductus arteriosus) into an elastic artery (aorta) during fetal life, where the contraction and fibrosis of the ductus arteriosus upon birth subsequently narrows the aortic lumen. [6]

Aortic coarctation and aortic stenosis are both forms of aortic narrowing. In terms of word root meanings, the names are not different, but a conventional distinction in their usage allows differentiation of clinical aspects. This spectrum is dichotomized by the idea that aortic coarctation occurs in the aortic arch, at or near the ductus arteriosus, whereas aortic stenosis occurs in the aortic root, at or near the aortic valve. [7] This naturally could present the question of the dividing line between a post valvular stenosis and a preductal coarctation; nonetheless, the dichotomy has a practical use, as most defects are either one or the other.[ citation needed ]

Signs and symptoms

Illustration of an aortic coarctation Coarctationlayoutv2-575px.jpg
Illustration of an aortic coarctation

In mild cases, children may show no signs or symptoms at first and their condition may not be diagnosed until later in life. Some children born with coarctation of the aorta have additional heart defects, such as aortic stenosis, ventricular septal defect, patent ductus arteriosus or mitral valve abnormalities.[ citation needed ]

Coarctation is about twice as common in boys as it is in girls. It is frequently found in girls who have Turner syndrome. [8]

Symptoms may be absent with mild narrowings (coarctation). When present, they include breathing difficulties, poor appetite or trouble feeding, and failure to thrive. Later on, children may develop symptoms related to problems with blood flow and an enlarged heart. They may experience dizziness or shortness of breath, fainting or near-fainting episodes, chest pain, abnormal tiredness or fatigue, headaches, or nosebleeds. They have cold legs and feet or have pain in their legs with exercise (intermittent claudication). [8]

In cases of more severe coarctations, babies may develop serious problems soon after birth because not enough blood can get through the aorta to the rest of their body. Arterial hypertension in the arms with low blood pressure in the lower extremities is classic. In the lower extremities, weak pulses in the femoral arteries and arteries of the feet are found. [8]

The coarctation typically occurs after the left subclavian artery. However, if situated before it, blood flow to the left arm is compromised and asynchronous or radial pulses of different "strength" may be detected (normal on the right arm, weak or delayed on the left), termed radio-radial delay. In these cases, a difference between the normal radial pulse in the right arm and the delayed femoral pulse in the legs (either side) may be apparent, whilst no such delay would be appreciated with palpation of both delayed left arm and either femoral pulses. On the other hand, a coarctation occurring after the left subclavian artery will produce synchronous radial pulses, but radio-femoral delay will be present under palpation in either arm (both arm pulses are normal compared to the delayed leg pulses).[ citation needed ]


With imaging, resorption of the lower part of the ribs may be seen, due to increased blood flow over the neurovascular bundle that runs there. Prestenotic dilatation of the aortic arch and left subclavian artery, as well as indentation at the site of coarctation results in a classic 'figure 3 sign' on x-ray. The characteristic bulging of the sign is caused by dilatation of the aorta due to an indrawing of the aortic wall at the site of cervical rib obstruction, with consequent poststenotic dilatation. This physiology results in the '3' image for which the sign is named. [9] [10] [11] When the esophagus is filled with barium, a reverse 3 or E sign is often seen and represents a mirror image of the areas of prestenotic and poststenotic dilatation. [12]

Coarctation of the aorta can be accurately diagnosed with magnetic resonance angiography. In teenagers and adults echocardiograms may not be conclusive. [ citation needed ]

The severity of coarctation of the aorta can be rated by a combination of the smallest aortic cross-sectional area of the aorta (adjusted for body surface area) as measured by 3D-rendered contrast MRI, as well as mean heart rate–corrected flow deceleration in the descending aorta as measured by phase contrast magnetic resonance imaging. [13]


Unfortunately, coarctations can not be prevented because they are usually present at birth. The best thing for patients who are affected by coarctations is early detection. Some signs that can lead to a coarctation have been linked to pathologies such as Turner syndrome, bicuspid aortic valve, and other family heart conditions. [5]


In adults and children found to have coarctation, treatment is conservative if asymptomatic, but may require surgical resection of the narrow segment if there is arterial hypertension. The first operations to treat coarctation were carried out by Clarence Crafoord in Sweden in 1944. [14] In some cases angioplasty can be performed to dilate the narrowed artery, with or without the placement of a stent graft.[ citation needed ]

For fetuses at high risk for developing coarctation, a novel experimental treatment approach is being investigated, wherein the mother inhales 45% oxygen three times a day (3 x 3–4 hours) beyond 34 weeks of gestation. The oxygen is transferred via the placenta to the fetus and results in dilatation of the fetal lung vessels. As a consequence, the flow of blood through the fetal circulatory system increases, including that through the underdeveloped arch. In suitable fetuses, marked increases in aortic arch dimensions have been observed over treatment periods of about two to three weeks. [15]

The long-term outcome is very good. Some patients may, however, develop a narrowing (stenosis) or dilatation at the previous coarctation site. All patients with unrepaired or repaired aortic coarctation require follow-up in specialized Congenital Heart Disease centers.[ citation needed ]

Complications of surgery

Surgical treatment involves resection of the stenosed segment and re-anastomosis. Two complications specific to this surgery are left recurrent nerve palsy and chylothorax, as the recurrent laryngeal nerve and thoracic duct are in the vicinity. Chylothorax is a troublesome complication and is usually managed conservatively by adjusting the diet to eliminate long-chain fatty acids and supplementing medium-chain triglycerides. When conservative management fails surgical intervention is then most often required. [17] Fluorescein dye can aid in the localisation of chyle leak. [18]


Side effects

Previously, hypertension was defined as a blood pressure of 140/90 mm Hg but has since been revised by the American College of Cardiology/American Heart Association Task Force to a blood pressure of 130/80 mm Hg or higher in adults. [19] This is a severe problem for the heart and can cause many other complications. In a study of 120 coarctation repair recipients done in Groningen, The Netherlands, twenty-nine patients (25%) experienced hypertension in the later years of life due to the repair. While hypertension has many different factors that lead to this stage of blood pressure, people who have had a coarctation repair — regardless of the age at which the operation was performed — are at much higher risk than the general public of hypertension later in life. [20] Undetected chronic hypertension may result earlier atherosclerosis in the arterial area and can lead to earlier death among coarctation repair patients, at higher rates as time progresses. [20]

Angioplasty is a procedure done to dilate an abnormally narrow section of a blood vessel to allow better blood flow. This is done in a cardiac catheterization laboratory. Typically taking two to three hours, the procedure may take longer but usually patients are able to leave the hospital the same day. After a coarctation repair 20-60% of infant patients may experience reoccurring stenosis at the site of the original operation. This can be fixed by either another coarctectomy[ citation needed ]. [21]

Coronary artery disease (CAD) is a major issue for patients who have undergone a coarctation repair. Many years after the procedure is done, heart disease not only has an increased chance of affecting coarctation patients, but also progresses through the levels of severity at an alarmingly increased rate. In one study, one fourth of the patients who experienced a coarctation later died of heart disease, some at a relatively young age. [22] [23]

Clinical criteria are used in most studies when defining recurrence of coarctation (recoarctation) when blood pressure is at a difference of >20 mmHg between the lower and upper limbs. This procedure is most common in infant patients and is uncommon in adult patients. 10.8% of infant patients underwent recoarctations at less than two years of age while another 3.1% of older children received a recoarctation. [24]

People who have had a coarctation of the aorta are likely to have bicuspid aortic valve disease. Between 20% and 85% of patients are affected by this disease. Bicuspid aortic valve disease is a big contributor to cardiac failure, which in turn makes up roughly 20% of late deaths to coarctation patients. [24]


Because of the risk of recoarctation and late hypertension, check-ups are needed once a year or less frequently depending on the individual case. It is important to visit the cardiologist on a regular basis. [20] Depending on the severity of the patient's condition, which is evaluated on a case-by-case level, visiting a cardiologist can be a once a year or less frequent surveillance check-up. Keeping a regular schedule of appointments with a cardiologist after a coarctation procedure is complete helps increase the chances of optimal health for the patients. Nowadays, life expectancy is considered normal given the repair was successfully done in early childhood. Treatment of recoarctation is usually successfully done without the need for open-heart surgery. Recoarctation is increasingly less common in the modern era. Late hypertension does also seem to be much less of a problem if the coarctation repair was performed within the first 5 years of life. Life expectancy and quality of life are therefore the same or very close to that of the normal population, but check ups are recommended so that those few percent who need further treatment get it in time. [25]


The condition was largely unidentified until the mid-20th century. History of the condition prior to 1945 has been understood via post-mortem records, the first series of which was published in 1928, which examined cases as far back as 1791. The first surgery for coarctation of the aorta was performed by Clarence Crafoord and G. Nylin on October 19, 1944 in Stockholm, Sweden on a 12-year old boy. [26] [27] [28]

An anecdotal history statement describes the first diagnosed case of the coarctation of the aorta in Julia the daughter of the French poet Alphonse de Lamartine after the autopsy in 1832 in Beirut, the referenced manuscript still exists in one of the Maronite monasteries in Mount Lebanon.[ citation needed ]

Related Research Articles

Aorta Largest artery in the human body

The aorta is the main and largest artery in the human body, originating from the left ventricle of the heart and extending down to the abdomen, where it splits into two smaller arteries. The aorta distributes oxygenated blood to all parts of the body through the systemic circulation.

Aortic stenosis Medical condition

Aortic stenosis is the narrowing of the exit of the left ventricle of the heart, such that problems result. It may occur at the aortic valve as well as above and below this level. It typically gets worse over time. Symptoms often come on gradually with a decreased ability to exercise often occurring first. If heart failure, loss of consciousness, or heart related chest pain occur due to AS the outcomes are worse. Loss of consciousness typically occurs with standing or exercising. Signs of heart failure include shortness of breath especially when lying down, at night, or with exercise, and swelling of the legs. Thickening of the valve without narrowing is known as aortic sclerosis.

Heart murmur Medical condition

Heart murmurs are unique heart sounds produced when blood flows across a heart valve or blood vessel. This occurs when turbulent blood flow creates a sound loud enough to hear with a stethoscope. Turbulent blood flow is not smooth. The sound differs from normal heart sounds by their characteristics. For example, heart murmurs may have a distinct pitch, duration and timing. The major way health care providers examine the heart on physical exam is heart auscultation; another clinical technique is palpation, which can detect by touch when such turbulence causes the vibrations called cardiac thrill. A murmur is a sign found during the cardiovascular exam. Murmurs are of various types and are important in the detection of cardiac and valvular pathologies.

Tetralogy of Fallot Type of congenital heart defect

Tetralogy of Fallot (TOF), formerly known as Steno-Fallot tetralogy, is a congenital heart defect characterized by four specific cardiac defects. Classically, the four defects are:

Pulse pressure Difference between systolic and diastolic blood pressure

Pulse pressure is the difference between systolic and diastolic blood pressure. It is measured in millimeters of mercury (mmHg). It represents the force that the heart generates each time it contracts. Resting blood pressure is normally approximately 120/80 mmHg, which yields a pulse pressure of approximately 40 mmHg.

Patent ductus arteriosus Condition wherein the ductus arteriosus fails to close after birth

Patent ductus arteriosus (PDA) is a medical condition in which the ductus arteriosus fails to close after birth: this allows a portion of oxygenated blood from the left heart to flow back to the lungs by flowing from the aorta, which has a higher pressure, to the pulmonary artery. Symptoms are uncommon at birth and shortly thereafter, but later in the first year of life there is often the onset of an increased work of breathing and failure to gain weight at a normal rate. With time, an uncorrected PDA usually leads to pulmonary hypertension followed by right-sided heart failure.

Vascular surgery Medical specialty, operative procedures for the treatment of vascular disorders

Vascular surgery is a surgical subspecialty in which diseases of the vascular system, or arteries, veins and lymphatic circulation, are managed by medical therapy, minimally-invasive catheter procedures and surgical reconstruction. The specialty evolved from general and cardiac surgery and includes treatment of the body's other major and essential veins and arteries. Open surgery techniques, as well as endovascular techniques are used to treat vascular diseases. The vascular surgeon is trained in the diagnosis and management of diseases affecting all parts of the vascular system excluding the coronaries and intracranial vasculature. Vascular surgeons often assist other physicians to address traumatic vascular injury, hemorrhage control, and safe exposure of vascular structures.

dextro-Transposition of the great arteries Medical condition

dextro-Transposition of the great arteries is a potentially life-threatening birth defect in the large arteries of the heart. The primary arteries are transposed.

Ductus arteriosus Blood vessel connecting the pulmonary artery to the proximal descending aorta

The ductus arteriosus, also called the ductus Botalli, named after the Italian physiologist Leonardo Botallo, is a blood vessel in the developing fetus connecting the trunk of the pulmonary artery to the proximal descending aorta. It allows most of the blood from the right ventricle to bypass the fetus's fluid-filled non-functioning lungs. Upon closure at birth, it becomes the ligamentum arteriosum.

Bicuspid aortic valve Medical condition

Bicuspid aortic valve is a form of heart disease in which two of the leaflets of the aortic valve fuse during development in the womb resulting in a two-leaflet (bicuspid) valve instead of the normal three-leaflet (tricuspid) valve. BAV is the most common cause of heart disease present at birth and affects approximately 1.3% of adults. Normally, the mitral valve is the only bicuspid valve and this is situated between the heart's left atrium and left ventricle. Heart valves play a crucial role in ensuring the unidirectional flow of blood from the atrium to the ventricles, or from the ventricle to the aorta or pulmonary trunk. BAV is normally inherited.

Persistent truncus arteriosus Medical condition

Persistent truncus arteriosus (PTA), often referred to simply as truncus arteriosus, is a rare form of congenital heart disease that presents at birth. In this condition, the embryological structure known as the truncus arteriosus fails to properly divide into the pulmonary trunk and aorta. This results in one arterial trunk arising from the heart and providing mixed blood to the coronary arteries, pulmonary arteries, and systemic circulation. For the International Classification of Diseases (ICD-11), the International Paediatric and Congenital Cardiac Code (IPCCC) was developed to standardize the nomenclature of congenital heart disease. Under this system, English is now the official language, and persistent truncus arteriosus should properly be termed common arterial trunk.

Aortic arch

The aortic arch, arch of the aorta, or transverse aortic arch is the part of the aorta between the ascending and descending aorta. The arch travels backward, so that it ultimately runs to the left of the trachea.

Ortners syndrome Medical condition

Ortner's syndrome is a rare cardiovocal syndrome and refers to recurrent laryngeal nerve palsy from cardiovascular disease. It was first described by Norbert Ortner (1865–1935), an Austrian physician, in 1897.

Interrupted aortic arch is a very rare heart defect in which the aorta is not completely developed. There is a gap between the ascending and descending thoracic aorta. In a sense it is the complete form of a coarctation of the aorta. Almost all patients also have other cardiac anomalies, including a ventricular septal defect (VSD), aorto-pulmonary window, and truncus arteriosus. There are three types of interrupted aortic arch, with type B being the most common. Interrupted aortic arch is often associated with DiGeorge syndrome.

The following outline is provided as an overview of and topical guide to cardiology, the branch of medicine dealing with disorders of the human heart. The field includes medical diagnosis and treatment of congenital heart defects, coronary artery disease, heart failure, valvular heart disease and electrophysiology. Physicians who specialize in cardiology are called cardiologists.

Hypoplastic right heart syndrome is a congenital heart defect in which the right atrium and right ventricle are underdeveloped. This defect causes inadequate blood flow to the lungs and thus, a blue or cyanotic infant.

Double aortic arch is a relatively rare congenital cardiovascular malformation. DAA is an anomaly of the aortic arch in which two aortic arches form a complete vascular ring that can compress the trachea and/or esophagus. Most commonly there is a larger (dominant) right arch behind and a smaller (hypoplastic) left aortic arch in front of the trachea/esophagus. The two arches join to form the descending aorta which is usually on the left side. In some cases the end of the smaller left aortic arch closes and the vascular tissue becomes a fibrous cord. Although in these cases a complete ring of two patent aortic arches is not present, the term ‘vascular ring’ is the accepted generic term even in these anomalies.

Fetal aortic stenosis is a disorder that occurs when the fetus’ aortic valve does not fully open during development. The aortic valve is a one way valve that is located between the left ventricle and the aorta, keeping blood from leaking back into the ventricle. It has three leaflets that separate when the ventricle contracts to allow blood to move from the ventricle to the aorta. These leaflets come together when the ventricle relaxes.

The Yasui procedure is a pediatric heart operation used to bypass the left ventricular outflow tract (LVOT) that combines the aortic repair of the Norwood procedure and a shunt similar to that used in the Rastelli procedure in a single operation. It is used to repair defects that result in the physiology of hypoplastic left heart syndrome even though both ventricles are functioning normally. These defects are common in DiGeorge syndrome and include interrupted aortic arch and LVOT obstruction (IAA/LVOTO); aortic atresia-severe stenosis with ventricular septal defect (AA/VSD); and aortic atresia with interrupted aortic arch and aortopulmonary window. This procedure allows the surgeon to keep the left ventricle connected to the systemic circulation while using the pulmonary valve as its outflow valve, by connecting them through the ventricular septal defect. The Yasui procedure includes a modified Damus–Kaye–Stansel procedure to connect the aortic and pulmonary roots, allowing the coronary arteries to remain perfused. It was first described in 1987.


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Further reading