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A vascular ring occurs when the aorta grows incorrectly during fetal development. It is a congenital heart defect. The deformity strains the trachea, esophagus, or both, causing breathing or swallowing difficulties.
The aorta is the primary artery that transports oxygenated blood from the heart to the rest of the body. In a typical scenario, the aorta originates from the left ventricle of a baby’s heart and travels upward alongside the trachea, forming the aortic arch. From there, it descends on the side of the trachea, which is known as the descending aorta. With a vascular ring, aorta or its branches partially or completely form a ring around your baby’s trachea, esophagus, or both.
A vascular ring can be complete or incomplete. A complete vascular ring surrounds both the esophagus and the trachea. An incomplete vascular ring fails to surround the esophagus or trachea. A vascular ring may also develop in a variety of ways. The three most prevalent are:
- Double aortic arch: The aorta begins properly, but soon separates into two arches and on the other side, the arches rejoin as one blood vessel. This wraps and squeezes the infant’s trachea and esophagus.
- Right aortic arch: In this case, the aorta of the baby originates to the right of their trachea instead of the left. One of its branches, which develops unusually, passes behind the baby’s esophagus and gives rise to another artery that goes to their left arm (known as the left subclavian artery). The ring is completed by the presence of a remnant of a blood vessel that was open during fetal development, which is now known as the ligamentum arteriosum.
These blood arteries have the potential to restrict your baby’s trachea and/or esophagus.
- Aberrant (abnormal) right subclavian artery (RSA): Also known as arteria lusoria. This artery usually arises from the brachiocephalic trunk, one of the three arteries that arise from the aortic arch. But an abnormal RSA develops directly from the arch. This form of vascular ring is not a complete ring and may not cause symptoms.
Since the trachea is the tube that transports air to and from the lungs, a vascular ring might cause breathing problems. It might also create feeding and digestion problems when the esophagus cannot efficiently transport food as it should be. In most cases, a vascular ring may be treated with surgery.
Symptoms
Depending on the type and degree, vascular ring symptoms may develop during childhood, later in life, or never. Some people who are born with a vascular ring may also have additional heart abnormalities. The type of heart issues present determines the specific symptoms.
Several symptoms are associated with vascular ring, such as:
- Cough
- Respiratory infections
- Wheezing or noisy breathing
- Swallowing difficulties
- Having difficulty eating
- Vomiting
- Inability to gain weight normally
Cause
The cause of vascular ring, a medical condition, is unknown and it develops during fetal growth. Usually, certain portions of the aorta that are meant to disappear during fetal development do not undergo regression.
Risk factor
Vascular rings are a rare occurrence, affecting fewer than 1% of infants and representing only 1% to 3% of all cases of congenital heart defects. Although anyone can develop a vascular ring, it appears to be more common in male newborns compared to females.
Diagnosis
Diagnostic procedures for vascular ring may involve evaluating symptoms, conducting a physical examination, and performing various tests. Some of the tests that may be performed include:
- Imaging tests: such as chest X-ray, CT scan, an echocardiogram, CT angiogram, or an MRI scan. These tests may be used by doctors to plan treatment.
A chest X-ray produces images of the inside of the child’s body. It is frequently one of the first tests ordered throughout the diagnostic process. The exam might reveal which side of the body the aortic arch is located on. A CT scan captures internal structures such as the baby’s heart, trachea, and esophagus. An echocardiogram, often known as an echo, produces animated images of the baby’s heart and aorta.
- Barium swallow: A vascular ring indentation can be detected using this test. This test requires swallowing a chemical called barium. X-rays show the path of the barium through the stomach.
- Upper endoscopy: Using an endoscope, a medical equipment with a tiny light and a camera on it, the doctor will be able to see and evaluate the esophagus. During the procedure, the tube is passed into the mouth and into the throat.
- Bronchoscopy: This test can determine whether a vascular ring is pressing against the trachea. A bronchoscopy involves a healthcare provider inserting a tube into the bronchial tubes through the mouth or nose. The tube has a light and a small camera that allows the doctor to see into the airways and the windpipe.
Treatment
The treatment for a vascular ring usually involves surgery. The procedure used is determined by the nature and severity of the child’s condition as well as the pre-existing heart illness. Surgery is typically performed to relieve a vascular ring pressing against the trachea or esophagus.
Surgery is frequently performed as an open operation via the side of the child’s chest, also known as thoracotomy, which necessitates larger incisions. Vascular ring surgery often entails dividing the part of the vascular ring to alleviate pressure against the windpipe and food pipe. The surgery may also involve taking off a section of the aorta or artery.
Although the child may experience symptoms for a few months following surgery, the majority are symptom-free within a year. Most patients do not require additional procedures. However, children born with a vascular ring must have regular health examinations for the rest of their lives to prevent complications.
