Aortic arch abnormalities

Aortic arches are anastomoses that are in the early stages of the embryo. The function of these arches is to bridge the ventral aorta (aortic sac) and the dorsal aortas. Anomalies appear as alterations of these arches, deviations, etc.


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  • 1 Vascular rings
  • 2 Double aortic arch
    • 1 Radiological examination
    • 2 Diagnosis
  • 3 Aberrant Right Subclavian Artery
    • 1 Clinical picture
    • 2 Radiological examination
    • 3 Diagnosis
  • 4 Aberrant Left Subclavian Artery
    • 1 Radiological examination
    • 2 Diagnosis
  • 5 Interruption of the aortic arch
    • 1 Concept and pathological anatomy
    • 2 Clinical picture
    • 3 Diagnosis
  • 6 Sources

Vascular rings

The symptoms and signs of vascular rings depend on compression of the trachea and esophagus. The most frequent symptoms are: dysphagia and cough, present in 80% of cases. Poor weight development, circulation, stridor, dyspnea, cyanosis, vomiting, and more than 50% of patients have a normal electrocardiogram.

When it is suspected to be in the presence of a vascular ring, the following aspects should be kept in mind: determine if the vascular ring is present; if your symptoms and signs require surgery; the complete study of vascular anomalies and associated heart diseases. The vascular ring may be a double aortic arch, an aberrant right subclavian artery, and an aberrant left subclavian artery with a right aortic arch and others.

Double aortic arch

The developing fetus initially has an aortic arch on either side of the trachea and esophagus with the descending aorta on the left or right, which when it persists after birth is called a double aortic arch.

The double aortic arch presents as an isolated lesion, although it may be associated with other anomalies such as: tetralogy of Fallot, ventricular septal defect, complete transposition of the great vessels and the arterial trunk.

The left anterior arch crosses the left side of the trachea and esophagus and advances upward, and the right anterior arch crosses over the right main bronchus, attached to the esophagus , to join the left arch and form the descending aorta on the right side. or left. The size of the lumen of the vessel, the position of the descending aorta, and the side of the ductus arteriosus are variable.

Radiological examination

On a radiological view of the simple chest in a newborn with the presence of the thymus in the upper mediastinum, it is difficult to find signs of compression of the trachea. In a penetrated view it is possible to see the two aortic arches and in a lateral view to see the indentation of the trachea at the level of the aortic arch.

On examination of the barium esophagus, the degree of indentation and the size of the defect vary with the size of the aortic arch. In the lateral view of the esophagogram, the union of the two arches at the back produces a large indentation at the back of the esophagus. Complete the study with aortography and echocardiogram for the diagnosis of double aortic arch.


The diagnosis is made by the clinical picture and radiological study. Differential diagnosis with all those vascular pathologies that compress the esophagus and trachea. The study from the nose to the bronchi to eliminate other causes of dyspnea, stridor and cyanosis.

Aberrant right subclavian artery

The aberrant right subclavian artery arises as the last brachiocephalic branch of the aorta, crossing the mediastinum from left to right and from bottom to top, causing oblique dentation in the posterior wall of the esophagus. It rarely passes between the esophagus and trachea and rarely dysphagia.

Clinical picture

If the right subclavian artery passes behind the esophagus it can cause dysphagia or be asymptomatic. Other symptoms such as cough, shortness of breath, and regurgitation.

Radiological examination

In the front view of the esophagogram, an oblique line appears, and in the lateral view, an impression on the back of the esophagus.


It can be asymptomatic or with the symptoms of associated congenital heart disease. Newborn children may have respiratory distress. The complementary ones previously indicated in the double aortic arch.

Aberrant Left Subclavian Artery

The aberrant left subclavian artery arises from the last branch of the right aortic arch and crosses to the left behind the esophagus and bulbous dilatation at its point of origin.

Usually, the ductus on the left side and communicates the left subclavian artery with the left pulmonary artery. When the aortic arch is right, the left subclavian artery and ductus arteriosus form a ring that embraces the esophagus and trachea. If the ascending aorta is on the right side, the arch passes on the left behind the esophagus and the descending aorta on the left side. If the descending aorta is to the right of the spinal column , the retroesophageal component is small.

Radiological examination

Symptoms and signs depend on the degree of constriction of the trachea and esophagus, manifesting as dysphagia and respiratory distress.

The configuration of the heart is normal. The right aortic arch is higher than the normal left. The aberrant left subclavian artery bulb projects as a mass on the left side of the trachea in the normal position of the left aortic arch. On the esophagogram, a right impression of the aortic arch.


The presence of dyspnea, stridor, dysphagia, regurgitation, circulation, are the main symptoms that motivate us to think of an anomaly of the aortic arch or vascular ring. Complementary examinations to determine the anatomical variants by means of the echocardiogram and angiogram.

Interruption of the aortic arch

Concept and pathology

It is defined as an absent or atrial segment of the aortic arch, and aortic continuity is interrupted.

Celoria and Patton divide patients with interrupted aortic arch into three groups:

  1. Disruption distal to the left subclavian;
  2. Interruption between the left carotid and the left subclavian.
  3. Disruption proximal to the left carotid artery. This group is the least frequent, with approximately 4%; group A, 43% and B, 53.

All reported cases have ductus arteriosus and are associated with other congenital heart defects, such as ventricular septal defect, aortopulmonary window, bicuspid aortic valve, and muscular subaortic stenosis. The dilated pulmonary artery.

Clinical picture

When complete disruption of the aortic arch is isolated, the clinical manifestations are similar to postductal aortic coarctation. Symptoms can appear early in life and be severe. The average age of onset of symptoms in the first 10 days and if they are not treated, they die at approximately 8 months of age.

The clinical picture is variable, depending on the severity of intracardiac defects, the degree of increase in pulmonary resistance, the size of the ductus arteriosus, and myocardial failure.

Clinical signs of intracardiac defects may be auscultated, and cyanosis may or may not be visible. Blood pressure increased in the upper extremities and decreased in the lower ones. The electrocardiogram shows signs of left or combined ventricular growth. On radiological examination there are no typical signs of interruption of the aortic arch and increased pulmonary vascularity. Heart failure can appear in the first days or weeks of age.


The box is similar to coarctation of the aorta. Complete the study with angiogram and echocardiogram.

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