Right aortic arch with aberrant subclavian and left ligamentum
Causes, incidence, and risk factors
Vascular ring is rare. It accounts for less than 1% of all congenital heart problems. The condition occurs as often in males as females. Some infants with vascular ring also have another congenital heart problem.
Vascular ring occurs very early in the baby's development in the womb. Normally, the aorta develops from one of several curved pieces of tissue (arches). The body breaks down some of the remaining arches, while others form into arteries. Some arteries that should break down do not; this forms vascular rings.
With vascular ring, some of the arches and vessels that should have changed into arteries or disappeared are still present when the baby is born. These arches form a ring of blood vessels, which encircles and presses down on the windpipe (trachea) and esophagus.
Several different types of vascular ring exist. In some types, the vascular ring only partially encircles the trachea and esophagus, but it still can cause symptoms.
Some children with a vascular ring never develop symptoms. However, in most cases, symptoms are seen during infancy. Pressure on the windpipe (trachea) and esophagus can lead to breathing and digestive problems. The more the ring presses down, the more severe the symptoms will be.
Breathing problems may include:
Loud breathing (stridor)
Repeated pneumonias or respiratory infections
Eating may make breathing symptoms worse.
Digestive symptoms are rare, but may include:
Difficulty eating solid foods
Difficulty swallowing (dysphagia)
Gastroesophageal reflux (GERD)
Slow breast or bottle feeding
Signs and tests
The doctor will listen to the baby's breathing to rule out other breathing disorders such as asthma. Listening to the child's heart through a stethoscope can help identify murmurs and other heart problems.
The following tests can help diagnose vascular ring:
Computed tomography (CT) scan of the heart
Camera down the throat to examine the airways (bronchoscopy)
Magnetic resonance imaging (MRI) of the heart
Ultrasound examination (echocardiogram) of heart
X-ray of blood vessels (angiography)
X-ray of the esophagus using a special dye to better highlight the area (esophagram or barium swallow)
Surgery is usually performed as soon as possible on children with symptoms. The goal of surgery is to split the vascular ring and relieve pressure on the surrounding structures. The surgery is not very invasive. The procedure is usually done through a small surgical cut in the left side of the chest between the ribs.
Changing the child's diet may help relieve the digestive symptoms of vascular ring. The doctor will prescribe medications (such as antibiotics) to treat any respiratory tract infections, if they occur.
Children who don't have symptoms may not need treatment, but should be carefully watched to make sure the condition doesn't become worse.
How well the infant does depends on how much pressure the vascular ring is putting on the esophagus and trachea and how quickly the infant is diagnosed and treated.
Surgery works well in most cases and often relieves symptoms right away. Severe breathing problems may take months to go away. Some children may continue to have loud breathing, especially when they are very active or have respiratory infections.
Delaying surgery can lead to serious complications such as damage to the trachea and even death.
Calling your health care provider
Call your health care provider if your baby has symptoms of vascular ring. Getting diagnosed and treated quickly can prevent serious complications.
There is no known way to prevent this condition.
Bernstein D. Other congenital heart and vascular malformations. In: Kliegman RM, Behrman RE, Jenson HB, Stanton BF, eds. Nelson Textbook of Pediatrics. 19th ed. Philadelphia, Pa: Saunders Elsevier; 2011;chap 426.
Neil K. Kaneshiro, MD, MHA, Clinical Assistant Professor of Pediatrics, University of Washington School of Medicine; and Michael A. Chen, MD, PhD, Assistant Professor of Medicine, Division of Cardiology, Harborview Medical Center, University of Washington Medical School, Seattle, Washington. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.