What is vascular ring pathology?

From the Guidelines

Vascular ring pathology is a congenital cardiovascular anomaly where blood vessels surrounding the trachea and esophagus form a complete ring, potentially causing compression of these structures, and is diagnosed using imaging studies such as CT angiography, MRI, or barium esophagram, with surgical correction recommended for symptomatic patients 1.

Definition and Causes

Vascular ring pathology occurs during embryonic development when the aortic arch and its branches fail to develop normally, resulting in a complete ring of blood vessels surrounding the trachea and esophagus. The most common types include double aortic arch and right aortic arch with aberrant left subclavian artery and left ligamentum arteriosum.

Symptoms and Diagnosis

Symptoms typically include respiratory difficulties such as stridor, wheezing, recurrent respiratory infections, and feeding problems including difficulty swallowing and vomiting. The severity varies based on the degree of compression. Diagnosis involves imaging studies such as CT angiography, MRI, or barium esophagram to visualize the vascular structures and their relationship to the airway and esophagus, with CMR providing many advantages for the assessment of vascular rings and slings, including imaging of the airway and allowing a conclusive diagnosis to guide in therapeutic management 1.

Treatment and Management

Treatment is surgical correction to relieve compression, typically by dividing the non-dominant portion of the vascular ring. Surgery is generally recommended for symptomatic patients, while asymptomatic cases might be monitored. Early intervention is important to prevent long-term complications such as tracheomalacia or recurrent pneumonia. CMR has been demonstrated to successfully diagnose patent double arches and those with atretic portions and is superior to cardiac catheterization angiography in its ability to demonstrate associated compression of the airway 1.

Key Points

• Vascular ring pathology is a congenital cardiovascular anomaly that can cause compression of the trachea and esophagus.
• Diagnosis involves imaging studies such as CT angiography, MRI, or barium esophagram.
• Surgical correction is recommended for symptomatic patients to relieve compression and prevent long-term complications.
• CMR is a useful diagnostic tool for assessing vascular rings and slings, providing many advantages for therapeutic management 1.

From the Research

Definition of Vascular Ring Pathology

• A vascular ring is a rare congenital cardiovascular anomaly that encircles and compresses the trachea or esophagus, or both 2.
• It is a form of congenital malformation that completely encircles the trachea and esophagus with vascular structures 3.
• The term vascular ring refers to congenital vascular anomalies of the aortic arch system that compress the esophagus and trachea, causing symptoms related to those two structures 4.

Types of Vascular Rings

• The most common forms of vascular rings are double aortic arch and right aortic arch with an aberrant left subclavian artery and a left-sided ligamentum arteriosum 3.
• Right aortic arch with aberrant left subclavian artery is one of the most common forms of vascular ring 5.
• Other types of vascular rings include pulmonary artery sling and innominate artery compression syndrome 4.

Symptoms and Diagnosis

• Patients with vascular rings may present with symptoms of airway or esophageal impingement, including "noisy breathing," stridor, respiratory distress, difficulty feeding, or dysphagia 3.
• Diagnosis can be made using CT angiography or magnetic resonance angiography, which can accurately delineate the anatomy of the vascular ring and associated tracheal pathology 4.
• Echocardiography can also be used to assess for associated congenital heart disease, but is less useful for imaging vascular structures when atretic segments comprise part of the vascular ring 3.

Treatment and Outcomes

• Surgical division of the structures contributing to the vascular ring is the typical treatment for vascular rings 3.
• Outcomes of surgical intervention are excellent, with most patients having complete resolution of symptoms over a period of time 4.
• Some patients may require additional procedures, such as resection and transfer of the left subclavian artery to the left carotid artery, or aortic uncrossing operation 4.