title: Predicting outcome in 259 fetuses with agenesis of ductus venosus - a multicenter experience and systematic review of the literature (.).
abstract: OBJECTIVE
  To evaluate prenatal predictors of postnatal survival in fetuses with agenesis of ductus venosus (ADV).
  
  
  METHODS
  This retrospective study reviewed our experience and the literature between 1991 and 2015. Prenatal findings were evaluated and perinatal morbidity and mortality was documented.
  
  
  RESULTS
  A total of 259 cases were included in the present analysis from our centers and 49 published studies (15 patients from our retrospective cohort review and 244 from literature review). The intrahepatic and extrahepatic shunts were present in 32.0% (73/226) and 67.7% (153/226), respectively. Cardiomegaly (n = 64/259, 24.7%), hydrops (n = 31/259, 12.0%) and amniotic fluid abnormalities (n = 22/259, 8.5%) were among the most frequent initial ultrasound findings. One hundred and forty-seven fetuses (56.8%) had ADV without structural anomalies while 112 (43.2%) had associated anomalies (cardiac anomalies (n = 66), extra-cardiac anomalies (n = 19) and both cardiac and extra-cardiac anomalies (n = 27)). The mean gestational age (GA) at ultrasound diagnosis was 22.9 ± 6.9 weeks while the mean GA at delivery was 34 ± 7.5 weeks. The overall neonatal survival was 57.1% (n = 148/259). The following factors were associated with survival: advanced maternal age, earlier GA at diagnosis, prematurity, increased nuchal translucency, pericardial effusion, associated cardiac defects (especially AVSD), chromosomal abnormalities, hydrops, hygroma and limb anomalies.
  
  
  CONCLUSIONS
  Fetal hydrops, the presence of associated congenital anomalies and premature delivery are associated with poor prognosis in fetuses with ADV.
authors.affiliation: a Department of Obstetrics and Gynecology , Texas Children's Hospital Fetal Center and Baylor College of Medicine , Houston , TX , USA .
authors.forename: Amirhossein
authors.initials: A
authors.lastname: Moaddab
authors.affiliation: b Prenatal Diagnostic Service, Department of Obstetrics and Gynecology, AUSL , Reggio Emilia , Italy .
authors.forename: Gabriele
authors.initials: G
authors.lastname: Tonni
authors.affiliation: c Prenatal Diagnostic Service, Department of Obstetrics and Gynecology, "Carlo Poma" Hospital , Mantua , Italy .
authors.forename: Gianpaolo
authors.initials: G
authors.lastname: Grisolia
authors.affiliation: d Pathology Service, IRCCS Arcispedale "Santa Maria Nuova" , Reggio Emilia , Italy .
authors.forename: Maria Paola
authors.initials: MP
authors.lastname: Bonasoni
authors.affiliation: e Department of Obstetrics , Paulista School of Medicine - São Paulo Federal University of São Paulo (EPM-UNIFESP) , São Paulo , Brazil .
authors.forename: Edward
authors.initials: E
authors.lastname: Araujo Júnior
authors.affiliation: e Department of Obstetrics , Paulista School of Medicine - São Paulo Federal University of São Paulo (EPM-UNIFESP) , São Paulo , Brazil .
authors.forename: Lilliam Cristine
authors.initials: LC
authors.lastname: Rolo
authors.affiliation: f Department of Obstetrics and Gynecology , University of Brescia , Brescia , Italy .
authors.forename: Federico
authors.initials: F
authors.lastname: Prefumo
authors.affiliation: g Fetalmed - Maternal Fetal Diagnostic Center , Santiago , Chile , and.
authors.forename: Sergio
authors.initials: S
authors.lastname: de la Fuente
authors.affiliation: g Fetalmed - Maternal Fetal Diagnostic Center , Santiago , Chile , and.
authors.forename: Waldo
authors.initials: W
authors.lastname: Sepulveda
authors.affiliation: h Department of Pediatric Cardiology , Texas Children's Hospital Fetal Center and Baylor College of Medicine , Houston , TX , USA.
authors.forename: Nancy
authors.initials: N
authors.lastname: Ayres
authors.affiliation: a Department of Obstetrics and Gynecology , Texas Children's Hospital Fetal Center and Baylor College of Medicine , Houston , TX , USA .
authors.forename: Rodrigo
authors.initials: R
authors.lastname: Ruano
mesh_terms: D005260:Female; D006801:Humans; D007231:Infant, Newborn; D011247:Pregnancy; D000015:Abnormalities, Multiple / Q000000981:diagnostic imaging / Q000453:epidemiology; D006330:Heart Defects, Congenital / Q000000981:diagnostic imaging / Q000453:epidemiology; D015160:Hydrops Fetalis / Q000000981:diagnostic imaging / Q000401:mortality; D047928:Premature Birth; D011379:Prognosis; D012189:Retrospective Studies; D012307:Risk Factors; D018608:Ultrasonography, Doppler; D016216:Ultrasonography, Prenatal; D014471:Umbilical Veins / Q000002:abnormalities / Q000000981:diagnostic imaging; D054079:Vascular Malformations / Q000000981:diagnostic imaging / Q000401:mortality
keywords: Agenesis of ductus venosus; congenital anomalies; hydrops; outcome; prenatal diagnosis