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Updated: May 31, 2026

A Pipeline to Characterize Structural Heart Defects in the Fetal Mouse
Published on: December 16, 2022
Right aortic arch and double arch assessed by prenatal echocardiography: a population-based study
Anna N Seale1,2, Trisha V Vigneswaran3, Lindsey E Hunter4
1Cardiac Department, Birmingham Women's and Children's Hospital, Steelhouse Lane, Birmingham B4 6NH, UK.
The incidence of right (RAA) and double (DAA) aortic arch is higher than previously thought. Genetic anomalies are associated with these conditions, especially when extracardiac anomalies are present.
Area of Science:
- Cardiology
- Prenatal Diagnosis
- Genetics
Background:
- The three-vessel trachea view improved detection of right (RAA) and double (DAA) aortic arch.
- RAA and DAA are vascular anomalies affecting the aortic arch.
Purpose of the Study:
- To investigate the incidence, associations, and medium-term outcomes of RAA and DAA.
- To understand the clinical significance of these aortic arch anomalies.
Main Methods:
- Retrospective, population-based, multicentre observational study.
- Involved cardiology units across England, Scotland, and Wales.
- Included fetuses diagnosed with RAA/DAA between April 2015 and March 2019, excluding major congenital heart disease.
Main Results:
- The estimated incidence of RAA and DAA was 17.98 and 4.58 per 10,000 pregnancies, respectively.
- Genetic anomalies, particularly 22q11.2 microdeletion, were found in 7.6% of cases, with higher risk associated with extracardiac anomalies.
- 28.2% of liveborn infants experienced symptoms of tracheoesophageal compression, requiring surgery in 23.7%.
Conclusions:
- The incidence of RAA/DAA is higher than previously reported.
- Genetic anomalies are associated with RAA/DAA, especially in the presence of extracardiac anomalies.
- Infants with DAA underwent earlier surgical intervention compared to those with RAA-LAD-ALSA.
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