D'Antonio, F; Khalil, A; Zidere, V; Carvalho, JS
(2016)
Fetuses with right aortic arch Multicentre cohort study and meta-analysis.
Ultrasound in Obstetetrics and Gynecology, 47 (4).
pp. 423-432.
ISSN 1469-0705
https://doi.org/10.1002/uog.15805
SGUL Authors: Khalil, Asma Carvalho, Julene
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Abstract
OBJECTIVES: Recent antenatal screening guidelines for cardiac abnormalities has increased fetal diagnosis of right aortic arch (RAA). We aimed to establish outcome of fetal RAA without intra-cardiac abnormalities (ICA) to guide postnatal management. METHODOLOGY: Retrospective cohort study. Outcome measures were rates of chromosomal abnormalities, 22q11.2 deletion, fetal extra-cardiac abnormalities (ECA), postnatal ICA and ECA, symptoms and surgery for vascular ring. A systematic review and meta-analysis (reference: CRD42015016097) was performed; results are reported as proportions. Kaplan Meier analysis of vascular ring cases with surgery as endpoint was performed. RESULTS: Our cohort included 86 cases; 41 had a vascular ring. Rates of chromosomal abnormalities, 22q11.2 deletion, and fetal ECA were 14.1%, 6.4% and 17.4% respectively. Sixteen studies including our cohort (312 fetuses) were included in the systematic review. Overall chromosomal abnormalities and 22q11.2 deletion rates were 9.0% (95% CI 6.0-12.5) and 6.1% (95% CI 3.6-9.3) whilst rates for cases with no ECA were 4.6% (95% CI 2.3-7.8) and 5.1% (95% CI 2.4-8.6). ECA were seen in 14.6% (95% CI 10.6-19.0) prenatally and 4.0% (95%CI 1.5-7.6) after birth. Postnatal ICA were identified in 5.0% (95% CI 2.7-7.9). Rate of symptoms (follow up ≥24 months) was 25.2% (95% CI 16.6-35.0) while 17.1% (95% CI 9.9-25.7) had surgery. Two-year freedom from surgery was 83.0% (95% CI 74.3-90.1) CONCLUSIONS: Fetal RAA without ICA is more frequently associated with ECA than chromosomal abnormalities. Most cases however, are isolated. Vascular ring symptoms occur in about 25% of cases. Postnatal surveillance is required mainly in the first 2 years of life.
Item Type: | Article | ||||
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Additional Information: | Copyright © 2015 ISUOG. Published by John Wiley & Sons Ltd. | ||||
Keywords: | Prenatal diagnosis, aortic arch syndromes, echocardiography, fetus, meta-analysis, Obstetrics & Reproductive Medicine, 1114 Paediatrics And Reproductive Medicine | ||||
SGUL Research Institute / Research Centre: | Academic Structure > Molecular and Clinical Sciences Research Institute (MCS) Academic Structure > Molecular and Clinical Sciences Research Institute (MCS) > Cardiac (INCCCA) Academic Structure > Molecular and Clinical Sciences Research Institute (MCS) > Vascular (INCCVA) |
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Journal or Publication Title: | Ultrasound in Obstetetrics and Gynecology | ||||
ISSN: | 1469-0705 | ||||
Language: | ENG | ||||
Dates: |
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Publisher License: | Publisher's own licence | ||||
PubMed ID: | 26643657 | ||||
Go to PubMed abstract | |||||
URI: | https://openaccess.sgul.ac.uk/id/eprint/107593 | ||||
Publisher's version: | https://doi.org/10.1002/uog.15805 |
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