Double aortic arch: implications of antenatal diagnosis, differential growth of arches during pregnancy, associated abnormalities and postnatal outcome.

fetal scan and diagnosed antenatally as right aortic arch (RAA). Among those who had CT scan, the LAA was atretic in 55.7%. DAA was an isolated abnormality in 91.1% of the cases, 8.9% had intracardiac (ICA) and 2.5% had extracardiac abnormalities (ECA). Among those tested, 11.5% had genetic abnormalities and specifically 22q11 microdeletion was identified in 3.8% of the patients. At a median follow up of 993.5 days, 42.5% of the patients had developed symptoms of tracheo-esophageal compression (5.5% during the first month of life) and 56.2% underwent intervention. Statistical analysis using Chi-square test showed no statistically significant correlation between patency of both aortic arches or not and need for intervention (P-value 0.134), development of vascular ring symptoms (P-value 0.350) or evidence of airway compression on CT (P-value 0.193) CONCLUSIONS: Most DAA cases can be easily diagnosed in mid-gestation as both arches are patent with a dominant RAA. However, posnatally the LAA has become atretic in approximately half of the cases, supporting the theory of differential growth during pregnancy. DAA is usually an isolated abnormality; however, a thorough assessment is required, to exclude ICA and ECA and to discuss invasive prenatal genetic testing. Postnatally, early clinical assessment is needed, and CT scan should be considered, irrespective of the presence of symptoms or not. This article is protected by copyright. All rights reserved.