Vascular Rings: Symptoms, Types, Causes and Treatment

Vascular rings are rare congenital anomalies of the major blood vessels near the heart, which encircle and compress the trachea and esophagus. This can lead to a variety of symptoms, ranging from mild to life-threatening, and the condition is notoriously difficult to diagnose due to its rarity and the non-specific nature of its symptoms. In this article, we take a comprehensive look at vascular rings, exploring their symptoms, different types, underlying causes, and available treatment options—all supported by up-to-date research and clinical experience.

Symptoms of Vascular Rings

When it comes to vascular rings, the symptoms can be deceptive. Many patients—especially infants and young children—present with common respiratory or feeding difficulties, which often leads to misdiagnosis. However, recognizing the hallmark signs is crucial for early detection and effective management.

Symptom	Description	Age Group Most Affected	Sources
Stridor	High-pitched, noisy breathing	Infants, children	2 4 5 16
Wheezing	Whistling sound during breathing	Infants, children	4 5 16
Dysphagia	Difficulty swallowing	Children, adults	1 3 4 8
Respiratory infections	Recurrent chest infections	Primarily children	4 14 16
Dyspnea	Shortness of breath	Children, adults	3 8
Cough	Persistent or chronic cough	Children, adults	6 12
Apnea/cyanosis	Breathing pauses/blue discoloration	Infants	1 5

Table 1: Key Symptoms

Common Presenting Features

Vascular rings most often reveal themselves through respiratory symptoms. Stridor—a harsh, vibratory breathing sound—is the most frequently reported sign in infants and young children. Wheezing and recurrent respiratory infections are also common, as the vascular ring compresses the trachea, making it more susceptible to airway collapse and infection 2 4 5 16. Feeding difficulties, particularly dysphagia (trouble swallowing), can also occur due to esophageal compression, and these are more noticeable as the child begins to eat solid foods 1 3 4.

Less Typical Symptoms

Some patients experience less typical symptoms such as apnea (temporary cessation of breathing), cyanosis (bluish skin due to lack of oxygen), or even syncope (fainting) 1 5. In adults, dysphagia is often more prominent than respiratory issues, although exertional shortness of breath and recurrent bronchitis can also present 3 8.

Symptom Variability and Misdiagnosis

Because these symptoms overlap with common pediatric illnesses—such as asthma, croup, or recurrent bronchitis—vascular rings are often overlooked or misdiagnosed. The time from symptom onset to diagnosis can range from months to even years, especially in cases without associated cardiac defects 4 5.

Types of Vascular Rings

Understanding the different types of vascular rings is crucial, as the anatomy determines both the symptoms and the best approach to treatment. These anomalies result from variations in the development of the aortic arch and surrounding structures.

Type of Ring	Key Anatomic Feature	Prevalence	Sources
Double Aortic Arch	Two aortic arches encircle trachea/esophagus	Most common	7 8 9 14
Right Aortic Arch with Aberrant Left Subclavian Artery	Right-sided arch with left subclavian artery and ligamentum arteriosum	Common	1 7 9 14
Left Aortic Arch with Aberrant Right Subclavian Artery	Left-sided arch with aberrant right subclavian artery	Common	1 8 14
Pulmonary Artery Sling	Left pulmonary artery passes between trachea/esophagus	Rare	1 7 14
Innominate Artery Compression	Innominate artery compresses trachea	Infants only	7
Circumflex Aorta	Aorta loops behind esophagus as it crosses	Very rare	7

Table 2: Main Types of Vascular Rings

Double Aortic Arch

This is the most classic and complete form of vascular ring, with two arches forming a tight circle around the trachea and esophagus. It is the most likely to cause severe, early symptoms and almost always requires surgical correction 7 8 9 14.

Right Aortic Arch with Aberrant Left Subclavian Artery

Here, the right aortic arch gives rise to an aberrant left subclavian artery, often with a ligamentum arteriosum completing the ring. This variant is commonly associated with a structure called a Kommerell diverticulum, which may necessitate special surgical consideration 1 7 9 14.

Left Aortic Arch with Aberrant Right Subclavian Artery

In this type, the left aortic arch persists, but the right subclavian artery arises abnormally, passing behind the esophagus. While sometimes incomplete, this arrangement can still create significant compression 1 8 14.

Pulmonary Artery Sling

A rarer anomaly, the left pulmonary artery arises from the right pulmonary artery and passes between the trachea and esophagus, compressing both. These cases often have associated tracheal stenosis 1 7 14.

Other Variants

• Innominate Artery Compression: Unique to infants, where the innominate artery crosses and compresses the trachea 7.
• Circumflex Aorta: Exceptionally rare, requiring specialized surgical approaches 7.

Associated Anomalies

Over half of patients with vascular rings have other congenital cardiovascular malformations, underscoring the need for thorough cardiovascular evaluation 1 4 5 7.

Causes of Vascular Rings

The root cause of vascular rings lies in abnormal development of the aortic arch system during embryogenesis. Understanding these developmental errors provides insight into why these anomalies form and how they may differ between patients.

Cause	Mechanism/Description	Typical Timing	Sources
Embryonic Arch Persistence	Failure of normal regression of aortic arch segments	Fetal development	9 10 12
Abnormal Involution	Selective regression of arch segments leads to abnormal vessel arrangement	Fetal development	9 10
Genetic/Unknown	No clear genetic cause identified in most cases	Congenital	5 10 12
Associated Malformations	Often found with other cardiac anomalies	Congenital	4 5 7

Table 3: Main Causes of Vascular Rings

Embryologic Origins

During fetal development, the aortic arch forms from a complex system of paired arches. Vascular rings arise when there is abnormal persistence or involution (regression) of certain segments. For example, in a double aortic arch, both right and left fourth aortic arches persist, forming a complete ring 9 10.

Variations in Regression

• If the right arch persists instead of regressing, or if the subclavian arteries take unusual courses, different vascular ring types result 9 10.
• Atretic or fibrous remnants, such as the ligamentum arteriosum, can complete the ring, even if some segments are non-patent 13.

Lack of Genetic or Environmental Cause

Most cases of vascular rings appear sporadically, with no clear genetic pattern or identifiable environmental trigger. However, due to frequent associations with other congenital heart defects, genetic counseling may be considered in select cases 5 10.

Associated Cardiac and Non-Cardiac Anomalies

Over half of patients may have additional heart defects or other congenital anomalies. These associations can complicate both diagnosis and management 4 5 7.

Treatment of Vascular Rings

The mainstay of treatment for symptomatic vascular rings is surgical intervention. Advances in imaging and surgical techniques have dramatically improved outcomes for these patients.

Treatment	Purpose/Goal	Outcome/Prognosis	Sources
Surgical Division	Relieve compression by dividing ring	Excellent symptom relief	1 7 10 14 15
Imaging (CT/MRI)	Delineate anatomy, plan surgery	Crucial for diagnosis & planning	1 2 6 7 13
Barium Swallow	Initial screening for compression	High sensitivity	1 2 4 16
Bronchoscopy	Assess tracheal involvement	Recommended pre-op	7 17
Medical Management	Supportive care (pre-op)	Temporary relief	5 12
Reoperation	Address persistent/recurrent symptoms	High success w/ individualized approach	17

Table 4: Treatment Approaches and Outcomes

Diagnostic Workup

• Barium Esophagography: Often the first diagnostic tool, showing characteristic indentations on the esophagus 1 2 4 16.
• CT and MRI: Provide detailed images of the vascular anatomy and its relation to the airway and esophagus, essential for planning surgery 1 2 6 7 13.
• Bronchoscopy: Used to evaluate the degree of airway compression and check for tracheomalacia 7 17.

Surgical Management

The definitive treatment for symptomatic vascular rings is surgical division of the ring, usually via thoracotomy. The specific approach depends on the anatomy, but the goal is always to relieve the compression 1 7 10 14 15.

• Timing: Early surgery is preferred to prevent complications such as airway damage or feeding issues 10 15 16.
• Outcomes: Most patients experience complete or significant symptom relief after surgery. Some residual symptoms, such as mild stridor, may persist but often improve over time 1 7 10 14 15 16.
• Risks: Mortality and serious complications are rare; most adverse outcomes relate to associated tracheal or cardiac anomalies 14 15 16.

Managing Persistent or Recurrent Symptoms

A minority of patients continue to have symptoms after surgery, often due to residual scarring, tracheomalacia, or incomplete division of the ring. Reoperation may be needed and is usually successful when tailored to the underlying cause 17.

Long-Term Prognosis

Long-term outcomes are excellent, with most patients leading normal lives after surgery. Continuous follow-up may be necessary, especially for those with associated anomalies or persistent symptoms 1 7 8 10 14 17.

Conclusion

Vascular rings, though rare, are significant causes of respiratory and feeding difficulties in infants and children, and can even present in adults. Their variable symptoms make diagnosis challenging, but advances in imaging and surgical techniques have led to excellent outcomes for most patients.

Key Takeaways:

• Symptoms are diverse but commonly include stridor, wheezing, dysphagia, and recurrent infections.
• Types of vascular rings are determined by the specific vascular anatomy involved, with double aortic arch and right aortic arch with aberrant left subclavian artery being the most common.
• Causes stem from abnormal development of the aortic arch system during embryogenesis.
• Treatment is primarily surgical, with high rates of symptom relief and low risk of serious complications.

Early recognition and multidisciplinary management are essential for optimal outcomes. Clinical awareness and a high index of suspicion are key—prolonged or unexplained respiratory or feeding symptoms should prompt consideration of vascular rings.