Vascular ring is an abnormal formation of the aorta, the great artery that carries blood from the heart to the rest of the body. It is a congenital problem, which means it is present at birth.
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- 1 Causes, incidence and risk factors
- 2 Symptoms
- 3 Signs and tests
- 4 Treatment
- 5 Expectations
- 6 Complications
- 7 Prevention
- 8 Alternative names
- 9 Source
Causes, incidence and risk factors
The vascular ring is rare and represents less than 1% of all congenital heart problems. The condition occurs with equal frequency in men and women. Some babies with a vascular ring also have another congenital heart problem.
The vascular ring occurs very early in the development of the baby in the womb . Normally, the aorta develops from one of several curved pieces of tissue (arches). The body breaks some of the remaining arches, while others become arteries. Some arteries that must be broken do not; this forms vascular rings.
With the vascular ring, some of the arches and vessels that should have become arteries or disappeared are still present when the baby is born. These arches form a ring of blood vessels that surrounds and puts pressure on the trachea and esophagus .
There are several different types of vascular ring. In some types, the vascular ring surrounds the trachea and esophagus only partially, but it can still cause symptoms.
Some children with a vascular ring will never show symptoms. However, in most cases, symptoms are observed during lactation. Pressure on the trachea and esophagus can lead to respiratory and digestive problems. The more pressure the ring exerts, the more severe the symptoms become.
Respiratory problems can include:
- Squeaky cough
- Noisy breathing (stridor)
- Pneumonia or repetitive respiratory infections
- Breathing difficulty
Eating can worsen respiratory symptoms. Digestive symptoms are rare, but may include:
- Difficulty eating solid food
- Difficulty swallowing ( dysphagia)
- Gastroesophageal reflux (GERD)
- Slow breastfeeding or bottle feeding
Signs and tests
The doctor will listen to the baby’s breathing to rule out other respiratory 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 a vascular ring:
- Chest x-ray
- Computed tomography (CT) of the heart
- Downstream camera to examine the airways (bronchoscopy)
- Magnetic resonance imaging (MRI) of the heart
- Ultrasound (echocardiography) of the heart
- X-ray of the blood vessels (angiography)
- X-ray of the esophagus using a special dye to better highlight the area (esophagogram or esophagography)
Surgery is usually carried out as soon as possible in children with symptoms and the goal is to divide the vascular ring and relieve pressure on the surrounding structures. The surgery is not very invasive. The procedure is normally done through a small surgical incision on the left side of the chest between the ribs.
Changing the child’s diet can help alleviate the digestive symptoms of the vascular ring. Also, the doctor will prescribe medications (such as antibiotics) to treat any respiratory tract infection, if it occurs. Asymptomatic children may not need treatment, but they should be carefully watched to make sure the condition does not worsen.
The baby’s outlook depends on how much pressure the vascular ring is putting on the esophagus and trachea and how quickly it is diagnosed and treated.
Surgery works well in most cases and often relieves symptoms immediately. Serious breathing problems can take months to go away. Some children may continue to have noisy 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.
There is no known way to prevent this condition.
Right aortic arch with abnormal subclavian and left ligament