As with many congenital abnormalities, coarctation of the aorta is associated with other congenital anomalies. Coarctation of the aorta. On the left another patient with an aberrant right subclavian. On the axial image there is a right arch with the left subclavian artery that comes off on the posterior side and runs behind the trachea and the esophagus.
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Classification[ edit ] Illustration showing a heart with a coarctation of the aorta Sketch showing heart with coarctation of the aorta. A: Coarctation narrowing of the aorta. 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 kind usually appears when the ductus arteriosus closes. 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.
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 arteriosis, whereas aortic stenosis occurs in the aortic root, at or near the aortic valve. This naturally could present the question of the dividing line between a postvalvular stenosis and a preductal coarctation; nonetheless, the dichotomy has practical use, as most defects are either one or the other.
Signs and symptoms[ edit ] 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. Coarctation is about twice as common in boys as it is in girls. It is common in girls who have Turner syndrome. 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. 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. 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. Diagnosis[ edit ] 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.
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. In teenagers and adults echocardiograms may not be conclusive. 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.
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. The first operations to treat coarctation were carried out by Clarence Crafoord in Sweden in 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. Some patients may, however, develop 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. 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. 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.
Undetected chronic hypertension can lead to sudden death among coarctation repair patients, at higher rates as time progresses. 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. This can be fixed by either another coarctectomy[ citation needed ].
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. This procedure is most common in infant patients and is uncommon in adult patients. It is important to visit the cardiologist on a regular basis.
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. History[ edit ] 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 in Beirut, the reference manuscript still exists in one of the Maronite monasteries in Mount Lebanon.
Coarctation of the aorta
Coartación de la aorta