What is a Vascular Ring?
A vascular ring is a type of congenital (present at birth) defect. This happens when normal vessels are in the wrong place. This may cause pressure on the esophagus or airway.
View a graphic summary of vascular rings.
These vessels known as arteries may carry blood to the body, to the lungs or both. The three most common types of vascular rings are: 1) double aortic arch, 2) right aortic arch with abnormal connection of the left subclavian artery and 3) pulmonary arterial slings.
Vascular rings can vary. They can affect the aortic arch, forming a ring going around the trachea (windpipe) and esophagus. Some affect the arteries that carry blood to the lungs.
The aorta is the largest artery in the body. The aorta delivers oxygen to all parts of the body. When a baby is developing, parts of the arch may not form correctly.
The two most common types of true vascular rings are persistent double aortic arch and right aortic arch with abnormal connection of the left subclavian artery.
The left-sided ligamentum arteriosum (scar tissue formed as a remainder of the ductus arteriosus within three weeks of birth) completes the vascular ring.
What Symptoms are Caused by Vascular Rings?
There are many symptoms that may develop when vascular rings are present. Some vascular rings can be found before birth.
Many patients do not have symptoms or have only mild symptoms. Vascular rings diagnosed in patients less than 6 months of age are usually found because a baby has noisy breathing (stridor). Worsening of breathing, problems with feeding, or upper respiratory infections may be seen.
Children with double aortic arch defects often have symptoms earlier than those with right aortic arch defects. Swallowing problems are uncommon in the first months of life. But choking or swallowing problems are more common in older children as the main symptoms. A vascular ring can be discovered by chance during an exam for an unrelated problem.
How are Vascular Rings Diagnosed?
Physical examination may help find the reason for "noisy breathing." This may help separate it from other more common problems such as a floppy airway or asthma.
With a vascular ring, the noisy breathing may be heard both during inspiration (breathing in) and expiration (breathing out), while in asthma, the noise is at the end of expiration.
Sometimes, physical examination will find a weak pulse in an arm or legs. Listening to the chest for murmurs is often part of the exam.
A chest X-ray is often done as part of the initial evaluation. If the aortic arch is on the right side, a vascular ring may be suspected. Seeing the aortic arch on the plain chest X-ray may be hard in some children.
Patients with swallowing problems may do a barium swallow study as part of the first evaluation. This will typically show abnormal compression of the middle part of the esophagus.
A barium study that shows classic features of a vascular ring, along with a chest X-ray showing a right-sided aortic arch, makes it likely that a vascular ring is present. Often an echocardiogram (ultrasound of the heart) is ordered to see the anatomy of the aortic arch and the arteries that come from it.
A chest CT or MRI will often be ordered to show the relationship of the vessels, the trachea and the esophagus.
When breathing symptoms are moderate to severe, an airway scope or bronchoscopy may be done.
How are Vascular Rings Treated?
Surgery to divide the vascular ring is recommended in all symptomatic cases.
The goal of surgery for vascular rings is to make sure blood can flow through the vessels freely and to avoid compression of the trachea or esophagus. When the ring is open in at least one direction, symptoms will be relieved.
In most cases, the surgery is done using a small cut on the left side of the chest. This allows entry 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 putting pressure on the esophagus or airway.
With a right aortic arch and abnormal 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. Patients usually stay in the hospital for a day or two after surgery.
Complete relief of symptoms may happen right away after surgery.
In infants, there may be some tracheomalacia (floppiness of the trachea). This may be seen during physical activity or during upper respiratory infections.
Improvement of swallowing symptoms may be seen gradually.
Adult and Adolescent Management
Vascular rings can rarely show up in adult life. When they do, they are assessed and managed by surgeons. If they have been repaired, the symptoms usually go away. These patients typically do not need ongoing follow up with a doctor.
Learn more about the Adolescent and Adult Congenital Heart Disease Program.
