Double Aortic Arch: Symptoms, Types, Causes and Treatment

Double aortic arch is a rare but significant congenital anomaly where the aorta splits into two branches that encircle and compress the trachea and esophagus, often leading to potentially life-altering symptoms. This comprehensive article will guide you through the key aspects: symptoms, types, causes, and treatment, drawing upon leading research and clinical experience. Whether you are a patient, family member, or healthcare professional, understanding double aortic arch can help enable early diagnosis and effective management.

Symptoms of Double Aortic Arch

When the aorta forms two arches around the trachea and esophagus, it creates a vascular ring that can squeeze these vital structures. This compression usually begins to show symptoms in early infancy, but sometimes milder cases may be overlooked until later in childhood or even adulthood. Recognizing these symptoms early can be life-changing.

Symptom	Description	Prevalence	Source(s)
Stridor	High-pitched, noisy breathing	77–91%	1 2 3 4
Respiratory Distress	Difficulty breathing, especially during feeding	Common in infants	1 2 5
Dysphagia	Difficulty swallowing / choking	40–65%	1 2 4 5
Recurrent Infections	Frequent respiratory infections	Common	2 5
Feeding Problems	Choking, coughing with feeds	Common	1 2 3
Chronic Cough	Persistent cough	Common	2 5
Malnutrition	Poor weight gain, growth issues	Possible	2
Asymptomatic	No symptoms despite compression	15–35%	4 6

Table 1: Key Symptoms of Double Aortic Arch

Respiratory Symptoms

The most prominent symptoms of double aortic arch are respiratory. Stridor, a harsh, vibrating noise during breathing, is the classic sign and is present in most infants with this condition 1 2 3 4. Other breathing difficulties, such as noisy breathing, wheezing, and outright respiratory distress, especially during feeding or when lying down, are common.

• Chronic cough and recurrent respiratory infections such as bronchopneumonia may occur due to airway obstruction and poor clearance of secretions 2 5.
• Orthopnea (difficulty breathing when lying flat) can appear in severe cases, even in adults who may have been misdiagnosed for decades 5.

Feeding and Gastrointestinal Symptoms

Since the esophagus is also compressed, feeding difficulties are common:

• Dysphagia (difficulty swallowing), choking, and coughing during feeds are frequent, especially in infants 1 2 3 5.
• Malnutrition or failure to thrive can develop if feeding difficulties are severe or prolonged 2.

Age of Onset and Variability

• Symptoms often present in early infancy, sometimes immediately after birth 1 2 4.
• Some individuals, particularly if one arch is atretic (closed off), may remain asymptomatic until later childhood or even adulthood 6 9 15.
• In rare cases, adults may suddenly develop symptoms, often misattributed to asthma or other conditions 5 15.

Persistent Symptoms After Surgery

Even after surgical correction, some patients experience lingering respiratory symptoms due to longstanding compression and maldevelopment of the airway 1.

Types of Double Aortic Arch

Double aortic arch can manifest in several anatomical forms, which may influence both the severity of symptoms and the approach to treatment.

Type	Description	Frequency	Source(s)
Complete Double Arch	Both arches patent, forming a full ring	Most common	1 4 6 7
Right-Dominant	Right arch larger, left arch smaller or atretic	72–83%	1 4 6 13
Left-Dominant	Left arch larger, right arch smaller/atretic	Rare	6
Atretic Arch	One arch partially or fully closed (atretic)	Common in older children/adults	6 7 13
Kommerell Diverticulum	Bulge or diverticulum associated with an arch	Rare subset	13

Table 2: Types of Double Aortic Arch

Complete vs. Incomplete (Atretic) Double Aortic Arch

• Complete double aortic arch: Both arches are fully open (patent), forming a tight vascular ring. This is the classic and most symptomatic form, especially in infants 1 4 6.
• Atretic double aortic arch: One arch (usually the left) is partially or completely closed (atretic), resulting in a looser ring. Symptoms are often milder, and diagnosis may be delayed until later in life 6 7 9 13.

Dominant Arch Patterns

• Right-dominant double arch: The right arch is larger and carries most of the blood flow; this is the most common configuration (up to 72–83%) 1 4 6 13.
• Left-dominant double arch: The left arch is larger; this is much less common 6.

Associated Variants

• Kommerell Diverticulum: Some patients have a bulging outpouching (diverticulum) associated with one of the arches—most commonly the left—which can further compress the trachea and esophagus 13.
• The descending aorta is most commonly on the left side, but can be on the right in rare cases 6.

Symptom Correlation

• The anatomical type does not reliably predict the severity of tracheal compression or symptoms. Both complete and atretic forms can cause compression and symptoms 4 6 7.
• Imaging (CT, MRI) is crucial for distinguishing between types and planning surgery 3 7 14.

Causes of Double Aortic Arch

Understanding the origins of double aortic arch helps clarify why this anomaly develops and when to consider genetic counseling.

Cause	Description	Relevance	Source(s)
Embryological Error	Failure of normal regression of aortic arches	Universal	8 10
Genetic Syndromes	Chromosome 22q11 deletion (CATCH-22/DiGeorge)	Some cases	10 4
Familial/Hereditary	Rare familial occurrence	Rare	4 10
Associated Anomalies	Other heart/vascular malformations	18% cases	1 6 14

Table 3: Causes of Double Aortic Arch

Embryological Basis

Double aortic arch arises due to failure of the normal regression of the right dorsal aorta during fetal development. Normally, only one side (usually the left) remains to form the aortic arch, while the other regresses. If both persist, a double arch forms, encircling the trachea and esophagus 8 10.

• This anomaly is linked to the fourth branchial arch derivatives in embryology 8 10.
• The posterior arch is often larger and carries more blood flow 8.

Genetic Factors

• Chromosome 22q11 deletion syndrome (also known as CATCH-22 or DiGeorge syndrome) is reported in some cases of double aortic arch. This genetic variant can also be associated with other congenital heart defects 10 4.
• Genetic testing is advised when double aortic arch is identified, especially if other anomalies are present 4.

Association with Other Anomalies

• About 18% of cases have other congenital heart anomalies, such as ventricular or atrial septal defects, patent ductus arteriosus, or tetralogy of Fallot 1 6 14.
• Double aortic arch can also occur with other vascular variants, such as Kommerell diverticulum 13.

Familial and Sporadic Cases

• Most cases are sporadic, but rare familial patterns have been documented 4 10.

Treatment of Double Aortic Arch

Prompt treatment of double aortic arch is essential to relieve airway and esophageal compression and prevent long-term complications.

Treatment	Approach	Outcome	Source(s)
Surgical Division	Cutting the smaller/atretic arch	Excellent, 96% survival	1 5 11 14
Resection of Diverticulum	Removal of Kommerell diverticulum if present	Good	13
Simultaneous Repair	Correction of associated defects	Possible	14
Imaging Follow-up	CT/MRI for diagnosis & planning	Essential	3 7 14
Neonatal Support	Delivery in centers with neonatal ICU	Recommended	4

Table 4: Treatment Approaches for Double Aortic Arch

Surgical Management

• Surgical division of the lesser or atretic arch is the standard treatment 1 5 11 14.

  • Performed via a left thoracotomy (incision on the left side of the chest).
  • The goal is to break the vascular ring, relieving pressure on the trachea and esophagus.
  • If a Kommerell diverticulum is present, it is also resected to prevent residual compression 13.

• Simultaneous repair of associated cardiac defects may be performed in appropriately equipped centers 14.

Outcomes and Prognosis

• Excellent survival rates: 96% at 5 years post-surgery 1.
• Most patients experience significant relief of symptoms, although some may have persistent mild respiratory symptoms due to earlier airway damage 1 13.
• Low surgical mortality due to advances in postoperative care 11 14.

Postoperative Considerations

• Complications: May include chylothorax (9%), tracheal stenosis (14%), and tracheomalacia (7%) postoperatively 1.
• Reoperation is rare: Only a small fraction require further surgery 1.
• Long-term follow-up may be needed for airway issues.

Diagnostic and Preoperative Planning

• Contrast-enhanced CT or MRI: Essential for diagnosis, surgical planning, and distinguishing from similar anomalies 3 7 14.
• Flexible bronchoscopy: May be used to assess the degree of tracheal compression 4.

Special Considerations

• Significant tracheal compression can exist even without symptoms. Delivery at centers with neonatal intensive care is recommended when prenatal diagnosis is made 4.
• In adults, management is similar, though surgery may be considered only if symptoms are present 15.

Conclusion

Double aortic arch is a rare but potentially serious congenital vascular anomaly. Early recognition and treatment are critical to prevent complications and improve quality of life. Here's a summary of the key points:

• Symptoms: Most common are stridor, respiratory distress, feeding difficulties, and recurrent respiratory infections, predominantly in infants but occasionally in adults.
• Types: Several anatomical variants exist, with right-dominant double arch being the most frequent; both complete and incomplete forms can cause significant symptoms.
• Causes: The condition results from abnormal persistence of embryonic aortic arches, sometimes linked to genetic syndromes such as 22q11 deletion.
• Treatment: Surgical division of the smaller or atretic arch is highly effective, with excellent long-term outcomes; imaging is vital for diagnosis and planning.

Double aortic arch, though rare, is a treatable condition. With advances in imaging, surgical techniques, and postoperative care, most patients experience significant improvement and lead healthy lives. Early diagnosis and multidisciplinary care make all the difference.