Vascular Rings

What Are Vascular Rings? 

Vascular rings occur during fetal development due to an abnormal formation of the aorta (the artery that delivers oxygen to the body) and the surrounding blood vessels. These vessels form a ring around the trachea (brings air to the lungs) and the esophagus (brings food to the stomach). Vessels in the wrong position may cause pressure on these structures.

Our team treats the following types of vascular rings:  

  • Right aortic arch and left ligamentum – Infants with a right aortic arch are born with an aorta which arches right instead of the typical left arch. After birth, the remainder of the patent ductus arteriosus, a vessel used for fetal circulation, can form scar tissue. With this scar tissue, the right aortic arch forms a circle around the trachea and creates a vascular ring. 
  • Double aortic arch – In this type of vascular ring, there are both right and left sides of the aorta (typically, the aorta is either left or right sided) leading to the aorta encircling the trachea and esophagus These two branch-like structures encircle the trachea and esophagus.   
  • Pulmonary artery sling – The pulmonary artery has two main branches, right and left, which usually sit in front of the trachea and esophagus. A pulmonary artery sling occurs when the left pulmonary artery circles around behind the trachea and in front of the esophagus. This traps the trachea between the aorta rising from the heart and the left pulmonary artery.   

Normal Anatomy

Right Aortic Arch and Left Ligamentum

Double Aortic Arch

Pulmonary Artery Sling

What Are the Symptoms of a Vascular Ring?

Symptoms of a vascular ring can vary. Some children never develop symptoms. The more pressure the rings apply to the trachea and esophagus, the more severe the symptoms will be.

Vascular rings can cause noisy breathing "stridor" while inhaling and exhaling. Other problems can include breathing or swallowing difficulties and choking. Some infants have difficulty breathing at birth and may need to be intubated (have a breathing tube inserted) in order to bring air to the lungs. Swallowing difficulties tend to occur when children are older and not as infants. 

How Are Vascular Rings Diagnosed?

Vascular rings are diagnosed in several different ways. A vascular ring may be diagnosed pre-natally (before birth), or post-natally (after birth), during an echocardiogram (ultrasound of the heart). During this test, heart vessels are scanned for their placement in relation to the airway and esophagus.

All patients require a CT (computerized tomography) scan or cardiac MRI (Magnetic resonance imaging) evaluation of the chest and a bronchoscopy by an Otolaryngology (ENT) specialist. 

Typically, these studies can be accomplished within one day. All patients are evaluated by a multidisciplinary team which includes Otolaryngology, Medical Imaging and Cardiology. 

How Are Vascular Rings Treated?

Lurie Children’s surgeons have pioneered several procedures to advance the methods used to correct many kinds of vascular rings. Vascular rings must be surgically corrected. This can be done either through a left thoracotomy or a median sternotomy. If a left thoracotomy is used, you should expect a 2-day hospital stay. A median sternotomy requires cardiopulmonary bypass and therefore, has a longer recovery of 5-7 days.  

Pulmonary Artery Sling Repair

Vascular Ring Palliation

To learn more about treatment offered at Lurie Children's, visit our Vascular Rings Program page

Make an Appointment

If you’d like to request an appointment with one of our specialists, call 1.800.543.7362 (1.800.KIDS DOC®).

Second Opinions

For families or providers seeking a second opinion, we offer a Priority Second Opinion Clinic.

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Heart Center Family Resource Guide

To help prepare families for their care with Lurie Children's Heart Center, we have compiled a list of resources about treatment and recovery. Learn how to get ready for an inpatient stay or outpatient visit, and read about our support services for patients and families.