title: Fetuses with right aortic arch: a multicenter cohort study and meta-analysis.
abstract: OBJECTIVE
  Use of recent antenatal screening guidelines for cardiac abnormalities has increased fetal diagnoses of right aortic arch (RAA). We aimed to establish the outcome of fetal RAA without intracardiac abnormalities (ICA) to guide postnatal management.
  
  
  METHODS
  In the retrospective cohort part of our study, outcome measures were rates of chromosomal abnormalities, 22q11.2 deletion, fetal extracardiac abnormalities (ECA), postnatal ICA and ECA, and symptoms of and surgery for vascular ring. A systematic review and meta-analysis was also 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 rates of chromosomal abnormalities and 22q11.2 deletion were 9.0% (95% CI, 6.0-12.5%) and 6.1% (95% CI, 3.6-9.3%), whilst the respective 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 in 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 of vascular rings (follow-up ≥ 24 months postpartum) was 25.2% (95% CI, 16.6-35.0%), and 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 with 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 after delivery.
authors.affiliation: Fetal Medicine Unit, St George's University Hospital NHS Foundation Trust and Institute of Cardiovascular and Cell Sciences, St George's, University of London, London, UK.
authors.forename: F
authors.initials: F
authors.lastname: D'Antonio
authors.affiliation: Fetal Medicine Unit, St George's University Hospital NHS Foundation Trust and Institute of Cardiovascular and Cell Sciences, St George's, University of London, London, UK.
authors.forename: A
authors.initials: A
authors.lastname: Khalil
authors.affiliation: King's College Hospital, London, UK.
authors.forename: V
authors.initials: V
authors.lastname: Zidere
authors.affiliation: Fetal Medicine Unit, St George's University Hospital NHS Foundation Trust and Institute of Cardiovascular and Cell Sciences, St George's, University of London, London, UK.|Brompton Centre for Fetal Cardiology, Royal Brompton Hospital NHS Trust, London, UK.
authors.forename: J S
authors.initials: JS
authors.lastname: Carvalho
mesh_terms: D000015:Abnormalities, Multiple / Q000453:epidemiology; D001013:Aorta, Thoracic / Q000002:abnormalities* / Q000000981:diagnostic imaging / Q000196:embryology; D001015:Aortic Arch Syndromes / Q000000981:diagnostic imaging / Q000196:embryology / Q000209:etiology*; D002869:Chromosome Aberrations / Q000706:statistics & numerical data; D015331:Cohort Studies; D004062:DiGeorge Syndrome / Q000150:complications / Q000453:epidemiology; D005260:Female; D005315:Fetal Diseases / Q000000981:diagnostic imaging / Q000453:epidemiology / Q000209:etiology*; D005318:Fetal Heart / Q000002:abnormalities* / Q000000981:diagnostic imaging / Q000473:pathology; D006330:Heart Defects, Congenital / Q000150:complications / Q000453:epidemiology; D006801:Humans; D053208:Kaplan-Meier Estimate; D011247:Pregnancy; D011296:Prenatal Diagnosis; D012189:Retrospective Studies; D016216:Ultrasonography, Prenatal / Q000379:methods
keywords: aortic arch syndromes; echocardiography; fetus; meta-analysis; prenatal diagnosis