---
title: Vascular rings
description: What a vascular ring and a double aortic arch are, why the ring squeezes the trachea and oesophagus, how it is found, why an operation is needed and when to call an ambulance.
type: disease
aliases:
  - Vascular ring
  - Aortic vascular ring
  - Double aortic arch
  - DAA
sources:
  - tier: guideline
    org: minzdrav-rf
    title: Перерыв дуги аорты. Двойная дуга аорты
    year: 2024
    url: https://cr.minzdrav.gov.ru/view-cr/830_1
  - tier: reference
    org: msd-manuals
    title: Dysphagia Lusoria
    year: 2026
    url: https://www.msdmanuals.com/professional/gastrointestinal-disorders/esophageal-and-swallowing-disorders/dysphagia-lusoria
sources_checked: 2026-10-14
review: null
---

Congenital anomalies of the aortic arch are a group of defects in which the branching, position or number of the arches of the <Structure id="aorta">aorta</Structure> and its branches are abnormal.<Cite n={1} /> Some of them form a vascular ring around the trachea and oesophagus. The most common variant is a double aortic arch: both arches, the right and the left, persist and together close a complete ring around the trachea and oesophagus.<Cite n={1} />

A double aortic arch does not disturb the circulation by itself: the symptoms are caused by compression of the trachea and oesophagus, and how strong it is determines when symptoms appear and the outlook.<Cite n={1} />

<Diagram name="vascular-rings">
  <p slot="normal">
    Normally there is a single aortic arch. It joins the ascending and descending aorta and passes
    beside the trachea and oesophagus, without forming a complete ring around them.<Cite n={1} />
  </p>
  <p slot="disease">
    With a double aortic arch both arches, the right and the left, persist and form a complete ring
    around the trachea and oesophagus.<Cite n={1} /> According to the Russian Ministry of Health
    guideline, the right arch is more often the larger one, and this is how it is shown in the
    diagram.<Cite n={1} /> Blood flow is not disturbed, but the ring squeezes the trachea and
    oesophagus from both sides.<Cite n={1} /> The diagram shows them in cross-section.
  </p>
</Diagram>

## Why it happens

<Patient>

The heart forms in the first trimester of pregnancy, and problems with its development at this time are especially dangerous.<Cite n={1} /> The embryo develops two aortic arches, and normally part of them disappears. With a double aortic arch this process goes wrong, and both arches persist.<Cite n={1} />

According to the Russian Ministry of Health guideline, a double aortic arch is the most common vascular ring and occurs in about 1 in 15,000 newborns.<Cite n={1} /> In about 20% of cases the child also has a genetic change, the 22q11 deletion (DiGeorge syndrome).<Cite n={1} />

In some children the ring occurs together with other heart defects: according to the same guideline, in about 25%. Typical ones are [tetralogy of Fallot](../tetralogy-of-fallot/), [ventricular septal defect](../ventricular-septal-defect/), [truncus arteriosus](../truncus-arteriosus/) and [pulmonary atresia](../pulmonary-atresia-with-ventricular-septal-defect/). The ring is then often found during tests for that defect.<Cite n={1} />

</Patient>

<Student>

According to the international nomenclature cited by the Russian Ministry of Health guideline, aortic vascular rings are divided into complete and incomplete ("loops").<Cite n={1} /> Complete rings include, among others, double aortic arch (with a dominant right, dominant left or balanced arches) and right aortic arch with an aberrant left subclavian artery; incomplete rings include innominate artery compression syndrome, pulmonary artery sling and aberrant right subclavian artery.<Cite n={1} />

In double aortic arch the right arch gives rise to the right common carotid and right subclavian arteries, and the left arch to the left ones.<Cite n={1} /> If the lumen of one arch is completely closed, that arch (usually the left) may be represented by a ligament, which still takes part in forming the complete ring.<Cite n={1} />

Double aortic arch belongs to the same group of arch anomalies as [interrupted aortic arch](../interrupted-aortic-arch/), but unlike it, it does not depend on a patent ductus arteriosus.<Cite n={1} /> Narrowing of the aorta, [coarctation](../coarctation-of-the-aorta/), is a different congenital defect of the aorta and is covered in a separate article.

</Student>

<HeartModel highlight={['aorta']} />

The 3D model shows a normal heart with a single aortic arch; the trachea and oesophagus are not shown on it.

## Symptoms

<Patient>

With a double aortic arch, symptoms usually appear in the first months of life, often as early as the first month.<Cite n={1} /> As a rule, they start earlier and are more severe than with another type of ring, a right aortic arch with a left ligamentum arteriosum.<Cite n={1} />

Compression of the trachea shows up as noisy, whistling breathing when the baby breathes in (stridor), sometimes from birth, wheezing when breathing out, and fast breathing.<Cite n={1} /> Stridor may get worse when the baby lies on its back rather than on its side; children often tip the head back, stretching the neck, to breathe more easily.<Cite n={1} /> The cry may be hoarse, and a persistent "barking" cough is a common symptom.<Cite n={1} />

Compression of the oesophagus shows up as the baby feeding poorly, having difficulty swallowing liquids and choking; symptoms often get worse during feeds.<Cite n={1} /> The child has often had pneumonia in the past, and repeated respiratory infections make the breathing difficulty worse.<Cite n={1} />

If the symptoms are mild, the defect can "hide" behind what looks like respiratory illness, and the diagnosis is sometimes made much later, even in adulthood.<Cite n={1} />

</Patient>

<Student>

With severe obstruction there may be episodes of apnoea, marked cyanosis and loss of consciousness, often with visible subcostal retraction.<Cite n={1} /> Without surgery, children with obvious symptoms of compression develop hypoxic spells, apnoeic spells and, later, damage to the trachea and bronchi.<Cite n={1} />

Symptomatic vascular rings are rare in adults; dysphagia predominates in reported cases, and the ring can mimic chronic asthma.<Cite n={1} /> MSD Manuals calls difficulty swallowing caused by compression of the oesophagus by a congenital vascular anomaly dysphagia lusoria; it is usually caused by an aberrant right subclavian artery, a double aortic arch, or a right aortic arch with an aberrant left subclavian artery or a left ligamentum arteriosum.<Cite n={2} />

</Student>

## How it is diagnosed

<Patient>

A double aortic arch can be suspected before birth on an ultrasound scan of the baby's heart. The pregnant woman can then be referred in advance to give birth where staff are ready to help a newborn with breathing problems.<Cite n={1} />

The doctor asks whether the noisy breathing changes with body position, and whether there is choking, spitting up, or frequent bronchitis and pneumonia.<Cite n={1} /> Examination often finds nothing except wheezing; the double arch itself does not cause heart murmurs, and they are heard only when there are associated defects.<Cite n={1} />

After birth, a heart ultrasound (echocardiography) is done: it shows the type of double arch, the sites of narrowing and the size of both arches, but other tests are often needed as well.<Cite n={1} /> To clarify the anatomy and plan the operation, a contrast CT scan is done, and in some cases an MRI scan.<Cite n={1} /> With marked compression, a chest X-ray can show narrowing of the trachea, and if CT cannot be done, a barium swallow X-ray helps: it shows indentations on both sides of the oesophagus.<Cite n={1} />

If DiGeorge syndrome is suspected, the blood is checked for calcium and hormones and genetic testing is done.<Cite n={1} />

</Patient>

<Student>

The ECG in double aortic arch is within normal limits unless there are associated defects.<Cite n={1} />

Flexible bronchoscopy is not done routinely in isolated double aortic arch; it is reserved for difficult cases, to assess the degree of compression and deformity of the trachea and bronchi and to rule out other causes of respiratory problems.<Cite n={1} />

According to MSD Manuals, in adults the definitive diagnosis is usually made with CT angiography or MRI; bronchoscopy can reveal the pulsatile compression typical of a vascular ring.<Cite n={2} />

</Student>

## How it is treated

<Patient>

The ring can only be removed by an operation.<Cite n={1} /> The Russian Ministry of Health guideline recommends it for all patients with a double aortic arch and marked airway compression; when symptoms of tracheal or oesophageal compression appear soon after birth, it recommends operating early, in the first months of life, and advises against putting the operation off for long.<Cite n={1} /> The operation may be open or thoracoscopic; the doctors decide which approach to use.<Cite n={1} />

If there are no symptoms, the operation is done mainly for prevention. Only in adults whose ring was found by chance and who have no symptoms may the operation not be needed.<Cite n={1} /> According to MSD Manuals, most people whose vascular ring is first diagnosed in adulthood need no treatment, and in children the decision to operate depends on the symptoms, the type of ring and the specific anatomy.<Cite n={2} />

According to the Russian Ministry of Health guideline, in severe forms the patient is cared for by a team of specialists, including thoracic surgeons and endoscopists when needed.<Cite n={1} /> Overall the outlook with a double aortic arch is favourable, but it becomes much worse with marked compression of the trachea and bronchi. If there is no marked deformity of the trachea and bronchi, the outlook after the operation is usually favourable.<Cite n={1} />

After the operation, follow-up is needed for life: until the age of 18 with a paediatric cardiologist, then with a cardiologist, a general practitioner or a general physician.<Cite n={1} /> The doctor decides whether sport is allowed after the operation: according to the guideline, it is possible if there is no compression of the trachea and oesophagus and no other complications.<Cite n={1} />

</Patient>

## When to call an ambulance

According to the Russian Ministry of Health guideline, a double aortic arch does not cause direct circulatory problems, but severe compression of the trachea is dangerous.<Cite n={1} /> Call your local emergency number (112 in the EU, 911 in the US and Canada, 999 in the UK) if a child has pauses in breathing, turns very blue, loses consciousness, or has very noisy, laboured breathing with the skin under the ribs drawing in.<Cite n={1} />

If these signs appear in a child who has already had the operation, also call your local emergency number (112 in the EU, 911 in the US and Canada, 999 in the UK) straight away: life-threatening complications after the operation are a reason for emergency admission to hospital.<Cite n={1} /> Noisy breathing, a barking cough or choking during feeds without these signs are a reason to take the child to a paediatrician without delay. As with any heart condition, call your local emergency number if someone faints, has chest pain or is severely short of breath: this is the site's general safety rule, repeated at the bottom of every page.

<Quiz />