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What Is A Double Aortic Arch?
Written by Medicover Team and Medically Reviewed by Dr Bharath Reddy D , Cardiologist
A double aortic arch is a rare congenital disability where the aorta, the large artery that carries blood from the heart, splits into two separate arches instead of forming a single arch. These two arches loop around and encircle the trachea (windpipe) and esophagus (food pipe), creating a tight ring. This is known as a vascular ring.
This ring can press on the airway and esophagus, making it hard to breathe or swallow, especially in babies and young children.
Vascular Ring
A vascular ring is a term used when blood vessels from the heart form a loop or ring around the trachea and esophagus. This happens due to abnormal development of the aortic arch system in the womb.
While some vascular rings cause no symptoms, others can compress the windpipe or food pipe and lead to significant respiratory or feeding issues.
Types of Vascular Rings
Here are the most common types of vascular rings:
Double Aortic Arch (Most common complete ring)
- Two aortic arches form a complete ring around the trachea and esophagus.
- Usually presents in infancy with noisy breathing (stridor), coughing, and difficulty feeding.
- Surgery is often required to divide the smaller arch and relieve pressure.
Right Aortic Arch with Aberrant Left Subclavian Artery
- The aorta arches to the right instead of the left.
- An extra artery (left subclavian) arises in a way that may create a partial ring.
- May or may not cause symptoms; surgery is needed if there's compression.
Pulmonary Sling
- The left pulmonary artery takes an unusual path behind the trachea and can compress it.
- Commonly associated with breathing problems in infants.
Innominate Artery Compression
- The brachiocephalic (innominate) artery compresses the trachea from the front.
- Usually causes stridor (noisy breathing) in children, but it is not a complete ring.
Types of Double Aortic Arches
A double aortic arch can be classified based on which of the two arches, right or left, is dominant or whether both arches are similar in size. The classification helps guide treatment and surgical planning.
- Right-Dominant Double Aortic Arch
- Left-Dominant Double Aortic Arch
- Balanced or Codominant Double Aortic Arch
- Incomplete or Atretic Arch
Why It Matters?
Identifying the type of double aortic arch helps surgeons plan the safest way to divide the smaller or non-dominant arch and relieve pressure on the windpipe and food pipe.
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 Causes of a Double Aortic Arch?
The exact cause is often unknown, but it results from an error in the formation of the aorta during embryonic development.
- Congenital malformation during fetal development
- Failure of the embryonic aortic arch system to regress normally
- Persistence of both left and right aortic arches, forming a vascular ring
- Isolated developmental defect (most common)
- May occur as part of a genetic syndrome (e.g., DiGeorge syndrome)
- Occasionally linked to chromosomal abnormalities.
Risk Factors for Double Aortic Arch
While many cases occur randomly, certain factors may increase the likelihood of a baby being born with a double aortic arch:
- Family history of congenital heart defects
- Genetic syndromes, such as:
- 22q11.2 deletion syndrome (DiGeorge syndrome)
- CHARGE syndrome
- Other heart defects present at birth
- Chromosomal abnormalities detected during pregnancy
- Environmental exposures during pregnancy (rare but possible)
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Get Second OpinionHow 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.
When to See a Doctor?
See a doctor if your child shows any of the following signs, especially in infancy:
- Noisy breathing (stridor), especially when feeding or lying down
- Trouble swallowing, gagging, or choking during feeds
- Frequent respiratory infections
- Unexplained weight loss or poor growth
- Bluish skin or lips (a sign of low oxygen)
Seek urgent care if your child has severe breathing difficulty, pauses in breathing, or fainting.
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Can You Prevent a Double Aortic Arch?
A double aortic arch happens before birth, so it can't be entirely prevented. But you can lower the risk of birth problems by:
- Getting regular prenatal checkups
- Avoiding smoking, alcohol, or drugs
- Taking prenatal vitamins with folic acid
- Managing existing health problems like diabetes
- Talking to a doctor if you have a family history of heart defects
Most cases happen without an apparent reason. Early diagnosis and care help keep your child healthy.
Our Experience Treating Double Aortic Arch
At Medicover Hospitals, we specialise in diagnosing and treating rare congenital conditions, such as a double aortic arch. Our team of expert pediatric cardiologists and cardiothoracic surgeons use advanced imaging tools to detect the condition early, often in newborns and infants.
We perform minimally invasive surgeries whenever possible to relieve airway and esophagus pressure safely. From pre-surgical care to post-operative follow-up, we support every child and family with compassion and clarity.
Our high success rates and family-focused approach have helped many children recover fully and return to healthy, active lives.
