Vascular Ring

What is a Vascular Ring?

A vascular ring is a congenital condition in which the large blood vessel is responsible for carrying blood away from the heart (aorta) or the associated vessels branching off the aortic arch form in a way that surrounds the trachea (airway) esophagus.

This results in the formation of a complete or incomplete ring around them. There can be wide variability in the group of structural abnormalities; however, all involve some degree of compression of the airway or esophagus. The location and severity of the compression depend on the anatomic location of the vessels. Some variations may be referred to as a double aortic arch or a right aortic arch with variable branch arterial anatomy.

What are the Symptoms of a Vascular Ring?

Symptoms of a vascular ring will typically, but not always, become apparent in infancy or early childhood. Symptoms may include:

• stridor (noisy breathing)
• cyanosis (bluing of the skin)
• recurrent episodes of pneumonia
• diagnosis of asthma
• and dysphagia (difficulty feeding or swallowing)

How is a Vascular Ring Treated?

The surgical approach is typically performed by a thoracotomy approach involving an incision through the chest wall side. The side of the thoracotomy depends on where the lesion is located. Cardiopulmonary bypass is generally not required for most cases.

Vessels are identified, and the non-dominant portion of the vessel is removed, ligated (tied off) and divided. Sometimes repair involves the relocation of these aberrant vessels. Careful attention is given to avoid interfering with the other blood vessels that arise from the arch while improving symptoms and preventing future complications.

Recovery After Surgery

Monitoring in the post-surgery recovery period following a vascular ring repair will most often include invasive lines, such as an arterial line, to monitor blood pressure. It may consist of a central venous line to deliver medications. Medications may be needed to control hemodynamics (blood pressure and heart rate), provide both pain and sedation medications and maintain nutrition/hydration during recovery. Non-invasive measures (stuck to the skin or placed externally) to monitor perfusion include pulse oximetry and NIRS (near-infrared spectroscopy) probes. The breathing tube (endotracheal tube) can be removed before leaving the operating room. Most of these lines and medications are stopped and removed the next day. A chest tube will be present to remove air, blood or fluid from around the heart or lungs. This tube will be removed in the ICU as soon as possible, typically the following day.

The average length of hospital stay is around 3-5 days.