What Is a Right Aortic Arch and How Is It Treated?

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

A right aortic arch is a rare congenital heart abnormality in which the aortic arch passes to the right of the trachea instead of following its usual course on the left side. The aorta is the main artery that carries oxygen-rich blood from the heart to the rest of the body.

Some people with a right aortic arch have no symptoms and the condition is discovered incidentally during imaging. In others, abnormal blood vessel arrangements can form a vascular ring that compresses the trachea or oesophagus, causing breathing or swallowing difficulties. A right aortic arch may also occur with other congenital heart defects.

What Are the Types of Right Aortic Arch?

Right aortic arch is classified according to the branching pattern of the major arteries and whether associated vascular abnormalities are present.

Right Aortic Arch With Mirror-Image Branching: The branches of the aortic arch follow a pattern that mirrors the normal left-sided arrangement. This type is often associated with congenital heart defects.
Right Aortic Arch With Aberrant Left Subclavian Artery: The left subclavian artery arises abnormally from the right-sided aorta and may pass behind the oesophagus.
Right Aortic Arch With Kommerell Diverticulum: An abnormal pouch-like enlargement develops near the origin of an aberrant subclavian artery and may contribute to compression of the trachea or oesophagus.
Right Aortic Arch With Isolated Left Subclavian Artery: The left subclavian artery is disconnected from the aortic arch and receives blood through other vessels. This is a rare form.

What Are the Symptoms of Right Aortic Arch?

Many people with a right aortic arch have no symptoms. When symptoms occur, they are usually caused by compression of the trachea or oesophagus by a vascular ring or by an associated congenital heart defect.

Difficulty swallowing
Feeding difficulties in infants
Choking or gagging during feeding
Persistent cough
Wheezing
Stridor
Recurrent respiratory infections
Breathing difficulties
Chest pain or discomfort in some cases
Cyanosis when an associated heart defect reduces oxygen levels

Breathing Problems

Compression of the trachea can cause noisy breathing, wheezing, persistent cough or recurrent respiratory infections. Symptoms may be more noticeable during feeding or respiratory illnesses.

Swallowing and Feeding Difficulties

Pressure on the oesophagus may cause difficulty swallowing, choking or feeding problems. Older children and adults may feel that solid food becomes stuck.

Symptoms of Associated Heart Defects

When a right aortic arch occurs with another congenital heart abnormality, symptoms may include cyanosis, poor feeding, poor growth, fatigue or shortness of breath.

What Causes a Right Aortic Arch?

A right aortic arch develops before birth when the embryonic aortic arches form differently from usual. Instead of the left-sided arch persisting, the right-sided arch remains and becomes the main pathway for blood leaving the heart.

Abnormal Fetal Development: Changes in the development of the embryonic aortic arches can result in a right-sided aortic arch.
Genetic Factors: Certain genetic or chromosomal abnormalities may increase the likelihood of developing the condition.
Associated Congenital Heart Defects: A right aortic arch may occur with conditions such as tetralogy of Fallot, truncus arteriosus or other structural heart abnormalities.
Chromosomal Abnormalities: Some cases are associated with chromosome 22q11.2 deletion and conditions such as DiGeorge syndrome.

When to See a Doctor for a Right Aortic Arch?

Medical evaluation by a Cardiologist or Pediatric Cardiologist is recommended when a child or adult has persistent breathing problems, difficulty swallowing, recurrent respiratory infections, or unexplained feeding difficulties. Prompt assessment is especially important when symptoms suggest significant airway compression or an associated congenital heart defect.

You should see a doctor if you have:

Persistent difficulty swallowing or feeding
Recurrent wheezing, stridor or respiratory infections
Ongoing cough or breathing discomfort

Get medical help immediately if:

Severe difficulty breathing or choking develops
The lips or skin become blue or grey
An infant cannot feed properly or shows signs of severe distress

These could be signs of serious airway compression or a heart defect, which needs urgent care.

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How Is a Right Aortic Arch Diagnosed?

A right aortic arch may be detected before birth, during evaluation of breathing or swallowing symptoms, or incidentally on imaging performed for another reason. Tests help define the position of the aorta and identify associated vascular or heart abnormalities.

Echocardiogram

An echocardiogram uses sound waves to assess the heart and major blood vessels. It can identify associated congenital heart defects and may show the position of the aortic arch.

Chest X-Ray

A chest X-ray may suggest that the aortic arch is located on the right side, although additional imaging is usually needed to define the anatomy.

CT Angiography

A CT angiogram provides detailed images of the aorta and its branches. It can show vascular rings, airway compression and abnormalities such as Kommerell diverticulum.

Magnetic Resonance Angiography

MRI or magnetic resonance angiography can provide detailed images of the heart and blood vessels without using ionising radiation.

Additional Tests

Bronchoscopy, swallowing studies or genetic testing may be recommended in selected cases when airway compression, swallowing problems or an associated genetic condition is suspected.

How Is a Right Aortic Arch Treated?

Treatment for a right aortic arch depends on whether symptoms, vascular compression or associated congenital heart defects are present. People without symptoms may not require treatment but may need periodic monitoring.

Observation and Monitoring

People with an isolated right aortic arch and no symptoms may only require regular medical follow-up.
Imaging may be repeated when necessary to monitor associated vascular abnormalities.

Surgical Treatment

Surgery may be needed when a vascular ring significantly compresses the trachea or oesophagus.
The procedure aims to relieve pressure on the airway or oesophagus by dividing or repositioning the structures causing compression.

Treatment of Associated Heart Defects

Congenital heart abnormalities occurring with a right aortic arch may require specialised medical management or corrective surgery.

Feeding and Nutritional Support

Infants or children with significant swallowing difficulties may need temporary feeding adjustments and nutritional support.
Growth and weight should be monitored until compression-related symptoms improve.

Multidisciplinary Care

Care may involve paediatric cardiologists, cardiothoracic surgeons, vascular surgeons, pulmonologists and other specialists depending on the person's symptoms and associated abnormalities.

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What Are the Risk Factors for a Right Aortic Arch?

A right aortic arch is a congenital condition, meaning it develops before birth. Certain genetic and cardiac abnormalities may be associated with an increased likelihood of this aortic arch pattern.

Chromosome 22q11.2 deletion
DiGeorge syndrome
Family history of congenital heart abnormalities
Other congenital heart defects
Certain genetic or chromosomal syndromes

What Are the Complications of a Right Aortic Arch?

Complications mainly occur when abnormal vessels compress nearby structures or when the condition is associated with another congenital heart defect.

Tracheal compression
Oesophageal compression
Persistent breathing difficulties
Recurrent respiratory infections
Feeding difficulties and poor growth
Complications related to associated congenital heart defects
Enlargement or aneurysmal changes involving Kommerell diverticulum in some individuals

What Is the Outlook for a Right Aortic Arch?

The outlook depends on the specific anatomy and whether other heart or vascular abnormalities are present. People with an isolated right aortic arch and no symptoms may lead normal lives without treatment.

When a vascular ring causes significant breathing or swallowing problems, surgical treatment can relieve compression. Regular follow-up is important for people with associated congenital heart defects or abnormalities such as Kommerell diverticulum.

Frequently Asked Questions

1. What is a right aortic arch?

A right aortic arch is a rare anatomical variation where the aorta curves to the right side of the body instead of the left.

2. What are the symptoms of a right aortic arch?

Most individuals with a right aortic arch do not experience any symptoms. In some cases, it may be associated with congenital heart defects or abnormalities in nearby structures.

3. How is a right aortic arch diagnosed?

A right aortic arch can be diagnosed through imaging tests such as X-rays, CT scans, or MRIs which can show the abnormal positioning of the aorta.

4. What are the potential complications of a right aortic arch?

Complications may include compression of nearby structures like the esophagus or trachea, leading to difficulty swallowing or breathing in some cases.

5. How is a right aortic arch treated?

Treatment depends on associated conditions and symptoms. In some cases, no treatment is necessary. However, surgical intervention may be required if complications arise.