Vascular Ring Anomalies

The vascular ring presents itself as a heart complication at birth. It is a rare condition which accounts for approximately 1-3% of all congenital heart diseases. A person born as male is 1.4 to 2 times more likely to have a vascular ring than a born female.¹ This disease can be life-threatening. However, in many cases, surgical intervention for vascular rings is successful.  

The vascular ring occurs when a part of the body’s largest artery that supplies oxygenated blood to the rest of the body structure, called the aorta or its branches, forms a ring around the trachea (windpipe, and oesophagus (food swallowing tube) or both which then constricts these tube-like structures causing breathing and swallowing problems.

A good understanding of this condition, provided by this article, will help you to acquire knowledge on the vascular ring. Knowing can empower you to enhance your quality of life or identify the signs in others. 

For instance, reading to explore the different types of vascular rings, understand the underlying causes, recognise the associated symptoms, learn about the diagnostic methods used for accurate identification, and discover the available treatment options that can alleviate symptoms and improve one’s overall well-being.

Overview

Vascular ring results in the aortic arch being anomalies which arise during foetal development.^2,3 

This vascular anomaly can then potentially form a  ring around the trachea and oesophagus. The cause can then induce breathing and swallowing problems as the vascular ring squashes the oesophagus or the trachea.⁴

Fortunately enough, surgery has been proven to be successful and relieving the aortic artery with a significantly decreased risk of morbidity and mortality, improving one’s well-being.⁵

What types of vascular rings are there?

Double aortic arch 

In this vascular arrangement, the aorta divides into two branches. One branch passes to the right side of the trachea, whereas the other branch passes to the left side of the oesophagus. As a result, a vascular ring forms around these structures with the risk of causing compression over time. 

Right aortic arch with left ligamentum arteriosum 

In this arrangement, the aortic arch curves to the right side instead of the left side of the trachea while the left subclavian artery, which branches from the aortic arch passes behind the oesophagus and trachea. 

The blood vessel ligamentum arteriosum, also uses the term the ductus arteriolsus ( a vessel that links the pulmonary artery with the aorta’s arch), then passes between the left subclavian artery and the left pulmonary artery (main blood vessel which supplies deoxygenated blood to the lungs), completing the vascular ring. 

Right aortic arch with symmetrical image branching 

In this arrangement, the ductus arteriosus (a blood vessel which connects the pulmonary artery to the aorta) completes the vascular ring. ⁴

What causes the vascular ring to develop?

The cause of developing vascular rings is not precisely understood yet. However, doctors believe that vascular rings occur early in pregnancies as the baby's blood vessels materialise in the womb. 

Abnormalities do not influence the birth mother’s genetics and her actions related to pregnancy.  

In a vascular system, the aorta has six arches in the chest area that shrink and disappear during a baby’s development. On the other hand,  due to the malformation of the aorta’s arches during the vascular development and are abnormally formed. 

For instance, vascular rings can lead to chromosome conditions such as Down syndrome or Di-George syndrome.⁴

What are signs and symptoms of vascular ring?

The signs and symptoms of vascular ring may not become apparent immediately after the baby's birth and can slowly develop over months or even years

The most common symptoms include.⁶

• Noisy breathing, the description of wheezing or rattling occurs
• Regular chest infections like recurrent pneumonia or bronchitis
• Swallowing difficulties, choking episodes or food getting stuck in the throat
• Failure to gain weight is common in babies because they have trouble digesting due to the narrowing or blockage of the oesophagus; it is challenging to receive proper nutrients

Management and treatment for vascular ring

Surgical treatment is the only method currently used on babies experiencing vascular rings and their symptoms. Vascular rings can further deteriorate and cause complications like sudden death or damage to the trachea.

However, if someone has a vascular ring but displays few or no symptoms, surgery may not be necessary. 

Video-assisted thoracoscopic surgery (VATS) can treat surgical procedures similarto vascular rings in paediatric patients. While VATS is more commonly associated with other thoracic surgeries, its potential application in vascular ring procedures is an ongoing study and research development. 

VATS offers the potential for minimally invasive surgical interventions, which could reduce surgical trauma, improve patient recovery, and provide favourable outcomes for individuals with vascular rings. However, the specific use of VATS in vascular ring cases may vary depending on  the patient’s symptoms and  the  surgical team's expertise.⁷

What tools are used to diagnose vascular rings?

Chest radiography

The imaging test is a principal tool that helps doctors evaluate breathing difficulties in children. It can show the position of the aortic arch and detect its location to visualise the position of a suspected vascular ring.  Chest X-rays may not always provide a clear diagnosis.

Barium esophagography

This test involves swallowing a liquid containing barium to help with the diagnosis . It helps doctors visualise abnormalities associated with vascular rings, such as double aortic arch or anomalous left pulmonary artery.

Echocardiography and color flow doppler

These are ultrasound tests that create images of the heart and blood vessels. They can detect blood flow and structural issues related to vascular rings and also help evaluate any associated heart defects.

Computed tomography scan (CT scan), magnetic resonance imaging (MRI), and digital subtraction angiography (DSA)

These imaging techniques provide detailed pictures of the blood vessels, airways, and surrounding structures. Scans are scheduled by a health expert if there is no precise evidence of the diagnosis..

 CT scans schedule after a computer interprets X-rays, while MRI uses strong magnetic fields and radio waves. DSA involves injecting contrast dye into the blood vessels to visualise them.

Bronchoscopy

This procedure involves inserting a thin, flexible tube with a camera into the airways to check for obstructions or compression. It helps doctors assess abnormalities like an incorrectly positioned blood vessel.

These tests allow doctors to identify and understand the presence of a vascular ring, enabling them to determine the most appropriate treatment plan.⁷

FAQs

How can I prevent vascular rings?

Vascular ring is typically a congenital condition occurring during foetal development and cannot be prevented. There are no known preventive measures specifically for vascular rings . However, maintaining a healthy lifestyle during pregnancy, including regular prenatal care and avoiding harmful substances, can contribute to overall foetal well-being.

How frequently are vascular rings formed?

Vascular ring is a relatively rare condition, accounting for a small percentage of congenital heart defects. The exact prevalence is not well-established, but it is estimated to occur in approximately 1 in 1,000 to 1 in 3,000 live births. The defects are common in infants and young children.

Who is at risk of vascular rings?

A vascular ring is typically present at birth and is not influenced by specific risk factors or behaviours. There is a hypothesis that a sporadic occurrence (no detection of specific pattern regarding inheritance or familial transmission of this condition). Any infant can be born with a vascular ring, regardless of the parent's health or medical history.

When should I see a doctor?

If a patient notices any concerning symptoms in your child, such as difficulty breathing, recurrent respiratory infections, feeding difficulties, or poor weight gain, please consult a doctor.

Summary

Vascular ring is a rare condition where abnormal blood vessels compress the trachea and oesophagus, causing swallowing and breathing difficulties. Early detection and intervention are crucial for managing and improving quality of life. Trust your healthcare team to guide you through this process.