Right aortic arch and double arch assessed by prenatal echocardiography: a population-based study
Authors' conclusion
Incidence of RAA/DAA is higher than previously reported. Genetic anomaly is identified in at least 7.6%, particularly in the presence of ECA. Liveborn with DAA have earlier and more surgical intervention compared to those with RAA-LAD-ALSA.
Study context
- Population
- Foetuses diagnosed with RAA/DAA in England, Scotland and Wales, 1 April 2015–31 March 2019, excluding major congenital heart diseaseuncategorized in 7
- Participants
- 1,046 participants
- Exposure
- RAA vs DAA
- RAAn = 898
- DAAn = 141
- Outcome
- Incidence, associations and medium-term outcome of RAA/DAA
Methodological detailspopulation-based, multicentre, Variation in management pathways
- Population-based
- Multicentre
- Variation in management pathways
Main finding
As reported in the abstract
Estimated incidence per 10 000 pregnancies screened/year
- Combined incidence
- 0.23%
Observational data: associations, not effects.
Further findings
| Outcome | Risk ratio (95% CI) | P value | |
|---|---|---|---|
| Abnormal genetic resultsPrenatal ECAvs No prenatal ECARisk of genetic anomaly | 3.39 (1.89–5.73) | P < 0.0001 | |
| 0.20.5125Risk ratio (log scale) |
| Outcome | DAA |
|---|---|
| Surgery by arch type | 74.6% |
| Median age at surgery | 5.29 months (IQR: 2.99-11.26 months) |
- Abnormal genetic results — Abnormal genetic results
- 80 of 1046 (7.6%)
- Abnormal genetic results — 22q11.2 microdeletion
- 28 (35%)
- Surgery by arch type: RAA-LAD-ALSA 24.4% · Median age at surgery 12.62 months (IQR: 8.68-17.32 months)
- Surgery by arch type: age at surgery P < .0001
Isolated cases
- Isolated RAA/DAA
- 940 (89.9%)
Liveborn and follow-up · mean follow-up 4.1 years (SD 1.4)
- Liveborn
- 986 (94.3%)
- Postnatal deaths from unrelated problems
- 5
- Symptoms of tracheoesophageal compression
- 278 of 986 (28.2%)
- Vascular ring relief
- 234 of 986 (23.7%)
Deaths after vascular ring relief
- Deaths after vascular ring relief
- 0
Original publication
Right aortic arch and double arch assessed by prenatal echocardiography: a population-based study. Eur Heart J. Published 28 May 2026. doi:10.
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