From Routine to Rare: Uncovering Double Aortic Arch During Prenatal Care

Document Type

Conference Proceeding

Publication Date

10-2025

Publication Title

Journal of Ultrasound in Medicine

Abstract

Objectives: A double aortic arch (DAA) is a rare con-genital cardiac anomaly, a form of vascular ring, accounting for less than 1% of cardiac anomalies and present approximately in 1 in 10,000 babies. After arising from the ascending aorta, the two arches surround the trachea and esophagus to form a complete vascular ring. A vascular ring may lead to variable degrees of compression of the trachea and/or the esophagus. Fetal DAA is generally diagnosed based on the characteristic complete vascular ring in the three-vessel tracheal view on ultrasound but may also be diagnosed in a neonate or pediatric age group as an incidental finding following evaluation for respiratory or swallowing difficulties. Methods: This is institutional review board (IRB)exempt because it is a single case report. Results: A 20 year-old African-American femaleG2P1001 presented at 12 weeks of gestation for routine prenatal care. Her obstetric history included prior full term vaginal delivery of a healthy female. Medical history included bipolar disorder and chronic hypertension controlled with Nifedipine. She denied being on any medication for her bipolar disorder and had no personal or family history of congenital cardiac disorder or DiGeorge syndrome. Non-invasive prenatal testing (NIPT) with the cell-free DNA (cf-DNA) showing low risk female and low risk for chromosome 22q11.2 deletion –DiGeroge syndrome. At 24 weeks and 4 days gestation, anatomy ultrasound revealed right sided aortic arch with a left  sided ductus arteriosus forming a loose vascular ring. Presence of a double aortic arch could not be ruled out and the rest of the anatomy evaluation was normal. A fetal echocardiogram showed normal cardiac position, size and rhythm but the 3-vessel trachea view revealed a double aortic arch with a dominant right arch and small left arch forming an aortic ring around the trachea and esophagus. The pediatric cardiologist recommended post-partum echocardiogram and surgery if necessary. At 39 weeks of gestation, induction of labor secondary to chronic hypertension was started with uneventful vaginal delivery of a baby girl weighing 3950 gram with neonatal intensive care unit (NICU) team present at the time of delivery. Neonatal evaluation revealed a warm, well perfused infant with no upper limb to lower limb pressure gradient. Auscultation revealed dual heart sounds with normal rate and rhythm. Mother and baby were discharged home in stable condition. Outpatient follow up with pediatric cardiology was arranged for further evaluation. Conclusions: Fetal double aortic arch (DAA) presents distinctive characteristics on prenatal echocardiography. A thorough and systematic ultrasound examination can significantly enhance the accuracy of prenatal diagnoses. While DAA is primarily an isolated anomaly, some cases are linked to intracardiac and extracardiac abnormalities. The likelihood of chromosomal abnormalities is quite low, particularly in isolated DAA cases. Most affected infants are asymptomatic afterbirth, and clinical outcomes are generally positive, even if surgery is needed to address tracheal or esophageal compression .It is essential to assess for additional malformations or chromosomal anomalies for accurate prognostic evaluations and effective prenatal counseling.

Volume

44

Issue

S1

First Page

S127

Comments

2025 American Institute of Ultrasound in Medicine (AIUM) Annual Convention, March 29 - April 1, 2025, Orlando, FL

Last Page

S127

DOI

10.1002/jum.180_70067

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