Vascular Rings

The symptoms seen with vascular rings can be quite varied. They may occasionally be noted in the newborn, but more often are recognized later.

Many patients are asymptomatic or have very mild symptoms. Vascular rings diagnosed in patients less than 6 months of age are often associated with symptoms of airway obstruction, such as stridor (noisy breathing). Worsening of breathing difficulties with feeding or during upper respiratory infections may be seen.

Children with double aortic arch anomalies tend to present earlier than those with right aortic arch variants. Swallowing problems are uncommon in the first months of life while children are on a liquid diet.

Choking or swallowing difficulties are more common in older children as the predominant symptoms. Occasionally, a vascular ring is discovered incidentally during evaluation of a seemingly unrelated problem.

Physical examination may help characterize the "noisy breathing" and help differentiate it from other more common problems such as asthma.

With a vascular ring, the noisy breathing may be heard both during inspiration and expiration, while in asthma, the noise is mainly at the end of expiration.

Occasionally, physical examination will detect an abnormally weak pulse in an arm or the legs due to narrowing in a part of the anomalous blood vessels. Listening to the chest for murmurs is often included to assess the need for more thorough evaluation for associated cardiac anomalies (which are uncommon).

A chest X-ray is often performed as a part of the initial evaluation, and if the aortic arch appears to be right-sided, a vascular ring should be suspected. The identification of the side of the aortic arch on the plain chest X-ray, though, may be difficult in some children, particularly infants.

Patients with swallowing difficulties should undergo a barium swallow as part of the initial evaluation. This will typically show abnormal compression of the middle part of the esophagus characteristic for a vascular ring.

A barium study that shows classic features of a vascular ring, along with a chest X-ray showing a right-sided aortic arch is generally all that is necessary to proceed with operation.

A chest CT or MRI will often be ordered to show the relationship of the vessels, the trachea and the esophagus.

When breathing symptoms predominate, bronchoscopy may be performed that will often demonstrate extrinsic, sometime pulsatile compression of the trachea.

An operation to divide the vascular ring is indicated in all symptomatic cases. It may be argued that when the vascular ring anomaly is found "incidentally" that continued observation is appropriate.

While the symptoms prompting evaluation leading to the diagnosis may not be "classic" stridor or dysphagia (swallowing difficulty), many feel that unusual presentations such as a "barky" cough or frequent upper respiratory infections may be associated with the vascular anomaly and constitute indications for surgery.

Given the low risk associated with surgical division of a vascular ring, it is difficult to recommend continued observation, particularly in younger patients.

The goal of surgical intervention for vascular rings is to convert a restrictive, closed ring into one that is open realizing that there may still be an abnormal course of some of the blood vessels. With the ring open in at least one direction, symptoms related to esophageal and tracheal compression will be relieved.

In most cases the operation is performed using an incision on the left side of the chest, entering between the ribs. In the case of double aortic arch, the left side of the ring (which is usually the smaller side) is divided where it is compressing the esophagus.

With a right aortic arch and anomalous left subclavian artery, the ligamentum arteriosum (a ligament that was a blood vessel during fetal life) is divided between the descending aorta and the pulmonary artery. Hospitalization after surgery is rarely more than a day or two.

Complete relief of symptoms may be noted immediately following the operation, although persistence of some findings is not uncommon.

In infants, there may be some degree of tracheomalacia (floppiness of the trachea) associated with the vascular anomaly and persistence of some stridor, particularly during times of great activity or during upper respiratory infections.

Depending on the specific anatomy, division of the ring may still leave either the subclavian artery or a segment of the aorta itself in an abnormal position behind the esophagus. Improvement of swallowing symptoms in such cases may be seen only gradually.

Vascular rings seldom present in adult life. When they do, they are assessed and managed by surgeons.  If they have been repaired, the patient has usually become asymptomatic, and does not require expert ongoing surveillance. 

Learn more about the Adolescent and Adult Congenital Heart Disease Program.