AORTIC COARCTATION. Synonyms: - Coarctation of the aorta

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1 AORTIC COARCTATION Synonyms: - Coarctation of the aorta Definition: Aortic coarctation is a congenital narrowing of the aorta, usually located after the left subclavian artery, near the ductus or the ligamentum arteriosus. This disease is usually associated to other congenital cardiac malformations, which appear in up to 75% of the cases diagnosed in the neonatal period. The most frequent ones are patent ductus arteriosus, bicuspid aortic valve, ventricular septal defect and mitral valve anomalies. The coexistence of several of these defects on the left side of the heart usually determines the Shone's syndrome. Clinical Picture: This disease shows a great variability in its clinical presentation. Newborn babies with severe aortic coarctation usually present congestive heart failure (See Clinical Picture in Diagnosis and Treatment), which gets worse once the ductus is closed. Given the fact that in these babies the coarctation is usually located before the ductus, the lower part of the body receives blood from the right ventricle through it. Radial pulses (from the wrists) are usually strong, while femoral pulses (from the groins) are very weak or absent. Older children with this disease may present scarce symptoms, and the diagnosis is usually carried out in a routine check, in which femoral pulses are considered reduced. Diagnosis:

2 Chest x-ray in more affected newborn babies usually shows cardiomegaly (widening of heart shape) and pulmonary congestion (See Diagnosis in Diagnosis and treatment). The x-ray may be almost normal for older children with aortic coarctation of scarce clinical significance. The Roessler sign (inferior rib notching) usually appears in late stages of the disease. The echocardiogram shows the area of the coarctation, its extension and the severity of the obstruction. It also helps to evaluate the presence of other related malformations, such as ventricular septal defect. Nuclear magnetic resonance may be useful for specific cases as a complementary method to define aortic arch anatomy more clearly. Treatment: Treatment differs according to the situation. In case of a newborn baby with severe symptoms, a very careful management in a Pediatric Intensive Care Unit or Neonatology is needed. An intravenous prostaglandin infusion must be started immediately. This medication prevents the ductus from closing and, therefore, maintains the blood flow to the lower part of the body. Furthermore, the baby may require inotropic medication (to improve heart contractility) and to be connected to a ventilator until the clinical condition is improved. For these patients, corrective surgery of the aortic coarctation shall be indicated immediately. In those cases where there is no other associated malformation, surgery can be carried out through an incision made below the left scapula (See Surgery in Diagnosis and treatment).

3 The narrowest section of the aorta along with the ductus are cut and removed. Continuity of the aorta is then restablished with a single continuous suture joining the upper and the lower portions. In order to do this, blood flow to the lower half of the body should be stopped during the repair, as shown in the figure.

4 Sometimes, when the coarctation involves the aortic arch, its lower side is opened a bit in order to enlarge this area and prevent its future constriction (extended end-to-end anastomosis). Other correction techniques such as the subclavian flap or the Dacron patch are almost no longer used. The main risks of the surgery are bleeding, post-operative arterial hypertension, renal failure in newborns and chylothorax (injury of lymphatic vessels with accumulation of a fat-rich liquid in the thorax). Another important risk related to this procedure is paraplegia (definitive inability to move the legs). This is due to lack of blood flow in the lower half of the body that takes place during aortic reparation. It approximately happens in 0.5% of the cases. Because of this, it is important to perform the surgery in a quick way, keeping the child s temperature in low values (34-35 C) during the procedure. During the past few years, stents have been developed (small wire braiding tubes which are extended to obtain different diameters), which can be placed into the aorta by catheterization (See Haemodynamics in Diagnosis and Treatment). Access to the aorta is by means of tubes introduced through the groin. Then, the stent is placed in the coarctation, dilating it and maintaining the normal diameter thanks to the strength of the material with which it is made. These stents can only be used in children heavier than kg approximately. When the aortic coarctation is associated to another cardiac malformation, such as ventricular septal defect, the correction is carried out through an incision in the sternum with the aid of cardiopulmonary bypass.

5 Prognosis: Prognosis and quality of life are good once the surgery and the post-operative period are overcome (See Postoperative in Diagnosis and treatment). Heart failure symptoms disappear. Heart dilation typically regresses. However, a small percentage of patients shall remain hypertense and with medication requirements for a long time. A frequent complication is the recurrence of the obstruction, known as recoarctation. It can be treated with ballooning by catheterization or with a stent, in those patients who have already undergone surgery, although some of them may require a new surgery to repair the recoarctation (See Follow-up and control in Diagnosis and treatment).

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