Ultrasound Obstet Gynecol. 2023 Mar 2. doi: 10.1002/uog.26186. Online ahead of print.
OBJECTIVES: This study aimed to evaluate the prenatal characteristics of double aortic arch (DAA), assess the relative size of the arches and their growth during pregnancy, describe associated cardiac, extracardiac and chromosomal/genetic abnormalities and review postnatal presentation and clinical outcome.
METHODS: All fetuses with a confirmed diagnosis of DAA seen in five specialized referral centers between November 2012 and November 2019 were retrospectively identified from the hospitals’ fetal databases. Fetal echocardiographic findings, intracardiac and extracardiac abnormalities, genetic defects, computed tomography (CT) findings and postnatal clinical presentation and outcome were evaluated.
RESULTS: A total of 79 fetal cases of DAA were included. Of the whole cohort, 48.6% had an atretic left aortic arch (LAA) postnatally, with 5.1% being atretic at 1st 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.