Abstract
Right aortic arch presents a reported incidence of 0.1% of the general population; the aim of our study was to evaluate the risk of associated intracardiac (ICA), extracardiac (ECA), or chromosomal abnormalities in fetuses with right aortic arch (RAA) and concomitant right ductal arch (RDA). A systematic review of the literature selected 18 studies including 60 cases of RAA/RDA. A meta-analysis with a random effect model calculated for each outcome the pooled crude proportion of associated abnormal outcomes in cases of RAA/RDA and the pooled proportions and odds ratios in RAA with LDA or RDA. Quality assessment of the included studies was achieved using the NIH quality assessment tool for case series studies. RAA/RDA presents risk of associated conotruncal CHDs of about 30% and risk of 22q11 microdeletion in the region of 1%. Two-thirds of 22q11 microdeletions had concomitant thymic hypoplasia and no other chromosomal defects were described. Risks for ICA, ECA, 22q11 microdeletion, and aberrant left subclavian artery are not substantially different in RAA with right or left arterial duct. RAA increases the risk of associated cardiac defects regardless of laterality of the ductal arch. In isolated RDA/RAA cases, absolute risks of extracardiac associated problems or surgery are rather low, we would therefore recommend reassurance, particularly when the thymus and karyotype are normal.
Highlights
The prevalence of aortic arch anomalies, including right aortic arch and double aortic arch is estimated to be approximately 0.1% in the adult population and low-risk fetuses [1,2]
Current International Society of Ultrasound in Obstetrics and Gynecology (ISUOG) practice guidelines for sonographic screening examination of the fetal heart recommend the three-vessel and trachea view to be included in routine pregnancy screening in order to increase prenatal detection of many cardiac and vascular abnormalities, including right aortic arch (RAA) [5]
The primary aim of our study is to evaluate the risk of associated ICA, ECA or chromosomal abnormalities in fetuses prenatally diagnosed with RAA and concomitant right ductal arch (RDA), in order to optimize prenatal counseling and perinatal management
Summary
The prevalence of aortic arch anomalies, including right aortic arch and double aortic arch is estimated to be approximately 0.1% in the adult population and low-risk fetuses [1,2]. Right aortic arch (RAA) is characterized by abnormal laterality of the aorta and the brachiocephalic vessels. Classification of congenital aortic arch abnormalities in four groups was reported decades ago: group I includes double aortic arch, group II left aortic arch, group III right aortic arch, and group IV other and rare malformations of the aortic arch system. This classification wan challenged and enriched with several subgroups, in order to incorporate more recent definitions [2]. Current International Society of Ultrasound in Obstetrics and Gynecology (ISUOG) practice guidelines for sonographic screening examination of the fetal heart recommend the three-vessel and trachea view to be included in routine pregnancy screening in order to increase prenatal detection of many cardiac and vascular abnormalities, including RAA [5]
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