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Double aortic valve — Clinical Guidelines

Overview

Double aortic arch is a congenital vascular anomaly characterized by the presence of two aortic arches encircling the trachea and esophagus, potentially leading to respiratory and esophageal symptoms depending on the degree of compression. 1 2 3

Diagnosis

Prenatal Diagnosis: Feasible via early transvaginal ultrasound between 12-17 weeks of gestation with a prevalence of approximately 1:2387. 1

Postnatal Imaging: MRI and esophagography useful for detailed anatomical assessment, especially in adults presenting with symptoms. 3

Symptom Presentation: Neonatal respiratory failure, esophageal symptoms, and airway obstruction may indicate the need for imaging. 2 3

Management

Surgical Intervention: Recommended for symptomatic patients or those with severe obstruction identified via imaging. 1 2

Symptom Resolution: Surgical repair typically resolves symptoms, though outcomes can vary; one case noted incomplete resolution. 1

Emergent Care: Neonates may require emergent intubation due to critical airway obstruction. 2

Special Populations

Pregnancy: Prenatal diagnosis possible with transvaginal ultrasound; associated anomalies and genetic abnormalities should be evaluated. 1

Pediatrics: Neonates often present with respiratory failure requiring immediate intervention. 2

Adults: Rare presentation; imaging like MRI crucial for diagnosis in symptomatic adults. 3

Key Recommendations

Prenatal Screening: Implement early transvaginal ultrasound screening for double aortic arch during 12-17 weeks of gestation to identify cases and associated anomalies. (Evidence: Moderate) 1

Immediate Neonatal Assessment: Neonates with respiratory distress should undergo urgent imaging to rule out critical airway obstruction due to double aortic arch. (Evidence: Weak) 2

Surgical Evaluation for Symptomatic Patients: Symptomatic patients, including those with severe obstruction, should be evaluated for surgical intervention to alleviate symptoms. (Evidence: Moderate) 1 2

References

1 Zmora O, Beloosesky R, Khatib N, Ginsberg Y, Khoury A, Bronshtein M. Early Prenatal Diagnosis of Double Aortic Arch: Prevalence, Associated Anomalies and Outcome. Ultraschall in der Medizin (Stuttgart, Germany : 1980) 2021. link 2 Lee M, Kumar TKS, Paudel G. Double aortic arch presenting as neonatal respiratory failure in the delivery room. Cardiology in the young 2018. link 3 Ito K, Kogure T, Hayashi S, Kaneko I, Shimada M, Tubuku M et al.. A case of the incomplete double aortic arch diagnosed in adulthood by MR imaging. Radiation medicine 1995. link

Double aortic valve