Double Aortic Arch: Symptoms, Causes, Diagnosis and Treatment

Written by Medicover Team and Medically Reviewed by Dr Jagadeesh Chandra Bose Y , Cardiologists

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1. Causes 2. Find a Doctor 3. Diagnosis 4. Treatment 5. FAQs

Double Aortic Arch is a rare congenital cardiovascular abnormality in which the aorta forms two arches that encircle the trachea and esophagus, creating a vascular ring. This ring can compress the airway and food pipe, leading to breathing difficulties, noisy breathing, recurrent respiratory infections, and swallowing problems, especially in infants and young children. Early diagnosis and timely treatment are important to relieve compression, improve symptoms, and prevent complications.

What are the Types of Double Aortic Arch?

Double Aortic Arch is classified according to the dominance of the aortic arch.

Right-Dominant Double Aortic Arch

This is the most common type, in which the right aortic arch is larger than the left.

Most common form
Right arch is larger
Causes varying degrees of airway compression

Left-Dominant Double Aortic Arch

In this type, the left aortic arch is larger and supplies most of the blood flow.

Less common
Left arch is dominant
Produces similar symptoms

Balanced Double Aortic Arch

Both arches are of similar size and contribute equally to blood flow.

Rare type
Both arches are equally developed
May produce significant vascular ring compression

What are the Symptoms of a Double Aortic Arch?

Symptoms of a double aortic arch usually manifest early, often in the first few months of life. These symptoms can vary in severity and may include:

Common Symptoms of Double Aortic Arch

A double aortic arch often causes symptoms in infancy or early childhood, due to the vascular ring pressing on the trachea (windpipe) and esophagus (food pipe). Symptoms vary in severity depending on the tightness of the ring.

Respiratory Symptoms

These are often the first and most noticeable signs:

Noisy breathing (stridor), especially when inhaling
A chronic cough that doesn't go away
Frequent respiratory infections (like bronchitis or pneumonia)
Wheezing not linked to asthma
Shortness of breath or trouble breathing during feeding or activity
Bluish skin or lips in severe cases (cyanosis)

Gastrointestinal Symptoms

Compression of the esophagus can lead to feeding difficulties, including:

Trouble swallowing (dysphagia), primarily solids
Gagging or choking while feeding
Vomiting or spitting up frequently
Poor weight gain or growth (failure to thrive)
Refusal to eat or irritability during feeds

Heart-Related Symptoms (Less Common)

Though the aorta is affected, direct heart symptoms are uncommon. However:

A heart murmur may be detected during examination
Abnormal pulse in the neck or arms (rare)
If the condition is associated with other congenital heart defects, additional cardiac symptoms may occur

Most infants with a double aortic arch show breathing and feeding difficulties early on. Prompt diagnosis and treatment can significantly improve symptoms and prevent long-term complications.

What are the Common Causes and Risk Factors of Double Aortic Arch?

Double Aortic Arch results from abnormal development of the embryonic aortic arches during fetal life. The exact cause is usually unknown, although some cases occur in association with genetic syndromes and other congenital heart defects.

Prenatal detection and early postnatal evaluation improve treatment planning and outcomes.

Causes

Abnormal persistence of both embryonic aortic arches
Congenital developmental defect during fetal growth
Occasionally associated with genetic abnormalities

Risk Factors

Congenital heart defects
Certain chromosomal or genetic syndromes, including 22q11.2 deletion syndrome in some cases
Family history of congenital cardiovascular abnormalities (rare)

When to See a Doctor for Double Aortic Arch?

Infants or children with persistent noisy breathing, feeding difficulties, recurrent respiratory infections, or swallowing problems should be evaluated promptly by a Cardiologist or Pediatric Cardiothoracic Surgeon.

You should see a doctor if your child has:

Persistent stridor or noisy breathing
Difficulty feeding or swallowing
Repeated respiratory infections or chronic cough

Seek immediate medical attention if your child:

Develops severe breathing difficulty or pauses in breathing
Shows bluish discoloration of the lips or skin (cyanosis)
Experiences choking with inability to breathe or feed properly

These symptoms may indicate significant airway compression requiring urgent medical evaluation and treatment.

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How is Double Aortic Arch Diagnosed?

Diagnosing a double aortic arch involves a combination of clinical evaluation and imaging techniques. Given the rarity of the condition, a high index of suspicion is necessary when presented with the characteristic symptoms.

Clinical Evaluation

The initial assessment involves a thorough medical history and a comprehensive physical examination. Physicians look for signs such as respiratory distress, feeding difficulties, and stridor, which can suggest a vascular ring anomaly.

Imaging Techniques

Imaging plays a critical role in confirming the diagnosis of a double aortic arch. The following techniques are commonly used:

Chest X-ray: While not definitive, it can suggest a vascular anomaly if there is evidence of tracheal compression.
Barium Swallow: This test can show indentations on the esophagus caused by the vascular ring.
Echocardiography: A non-invasive ultrasound of the heart that can provide initial insights into the presence of a vascular ring.
CT or MRI Angiography: These imaging modalities offer detailed visualisation of the aortic arch anatomy and are crucial for definitive diagnosis.

What are the Treatment Options for Double Aortic Arch?

Treatment is focused on relieving pressure on the windpipe and food pipe caused by the vascular ring. The main approach is surgery, which is highly effective in most cases.

Surgical Treatment (Definitive Management)

Vascular ring division surgery is the standard treatment for this condition.
A cardiothoracic surgeon cuts and removes the smaller arch to open the ring.
This relieves compression on the trachea and esophagus.
Most children recover well and can breathe and eat normally afterwards.

Supportive Care Before Surgery

If symptoms are mild or surgery is delayed, supportive care may be used:

Oxygen therapy for breathing support
Feeding assistance (e.g., thickened feeds, positioning)
Medications to reduce airway swelling if needed

Post-Surgical Follow-Up

After surgery, follow-up appointments are crucial to monitor the healing process.
Some children may require breathing therapy or speech and swallowing support.
Rarely, if airway damage is severe, further treatment may be required.

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What is the Recovery Process for Double Aortic Arch?

Most children recover well after surgery, with gradual improvement in breathing and swallowing over several weeks to months. Some children may continue to have mild airway symptoms for a period due to residual tracheal weakness (tracheomalacia), which usually improves with time.

Regular follow-up with the healthcare team ensures proper healing, monitors growth and development, and detects any long-term complications.

Recovery Includes

Regular follow-up with a pediatric cardiologist and cardiothoracic surgeon.
Monitoring breathing and swallowing after surgery.
Gradual return to normal feeding and activities.
Treatment of any persistent respiratory symptoms.
Routine assessment of growth and development.
Long-term monitoring for associated congenital heart conditions when present.

Frequently Asked Questions

1. Which type of double aortic arch is most common?

The most common form is the right-dominant double aortic arch, where the right arch is larger and more developed than the left. This variant forms a complete vascular ring around the trachea and esophagus.

2. How do you fix a double aortic arch?

Surgical correction is the standard treatment. It involves dividing and closing the smaller arch to release pressure on the trachea and esophagus, relieving breathing and swallowing difficulties.

3. What is the difference between a double aortic arch and a vascular ring?

A double aortic arch is a type of vascular ring, where both arches encircle the trachea and esophagus. "Vascular ring" is a broader term that includes several types of abnormal blood vessel formations.

4. What is the recovery time for double aortic arch surgery?

Recovery usually takes a few weeks. Most children stay in the hospital for about 5-7 days after surgery, followed by home rest and gradual return to normal activities as symptoms improve.

5. What is the life expectancy with a double aortic arch?

With early diagnosis and successful surgical treatment, life expectancy is normal. Most children recover well and lead healthy lives with no long-term complications if treated promptly.

6. What does a double aortic arch look like on a fetal ultrasound?

On fetal ultrasound, a double aortic arch may show as two vessels encircling the trachea in a ring-like shape. Further confirmation is typically done using fetal echocardiography or MRI for detailed imaging.