title: Are congenital malformations more frequent in fetuses with intrahepatic persistent right umbilical vein? A comparative study.
abstract: OBJECTIVE
  Persistent right umbilical vein (PRUV) is a vascular anomaly where the right umbilical vein remains as the only conduit that returns oxygenated blood to the fetus. It has classically been described as associated with numerous defects. We distinguish the intrahepatic variant (better prognosis) and the extrahepatic variant (associated with worse prognosis). The objective of this study was to compare rates of congenital malformations in fetuses with intrahepatic PRUV (I-PRUV) versus singleton pregnancies without risk factors.
  
  
  METHODS
  A multicenter, crossover design, comparative study was performed between 2003 and 2013 on fetuses diagnosed with I-PRUV (n=56), and singleton pregnancies without congenital malformation risk factors (n=4050).
  
  
  RESULTS
  Fifty-six cases of I-PRUV were diagnosed (incidence 1:770). A statistically significant association between I-PRUV and the presence of congenital malformations (odds ratio 4.321; 95% confidence interval 2.15-8.69) was found. This positive association was only observed with genitourinary malformations (odds ratio 3.038; 95% confidence interval 1.08-8.56).
  
  
  CONCLUSIONS
  Our rate of malformations associated with I-PRUV (17.9%) is similar to previously published rates. I-PRUV has shown a significant increase in the rate of associated malformations, although this association has only been found to be statistically significant in the genitourinary system. Noteworthy is the fact that this comparative study has not pointed to a significant increase in the congenital heart malformation rate. Diagnosis of isolated I-PRUV does not carry a worse prognosis.
authors.affiliation: Department of Obstetrics and Gynecology, Hospital Universitario Miguel Servet, Zaragoza, Spain. Electronic address: ignacioadiego@gmail.com.
authors.forename: Ignacio
authors.initials: I
authors.lastname: Adiego-Calvo
authors.affiliation: Department of Obstetrics and Gynecology, Hospital Universitario Miguel Servet, Zaragoza, Spain.
authors.forename: Ricardo
authors.initials: R
authors.lastname: Saviron-Cornudella
authors.affiliation: Department of Obstetrics and Gynecology, Hospital Universitario Puerta de Hierro-Majadahonda, Madrid, Spain.
authors.forename: Cristina
authors.initials: C
authors.lastname: Martinez-Payo
authors.affiliation: Department of Biostatistics, School of Medicine, University of Zaragoza, Zaragoza, Spain.
authors.forename: Encarna
authors.initials: E
authors.lastname: Rubio-Aranda
authors.affiliation: Department of Obstetrics and Gynecology, Hospital Universitario Puerta de Hierro-Majadahonda, Madrid, Spain.
authors.forename: Javier
authors.initials: J
authors.lastname: Sancho-Sauco
authors.affiliation: Department of Human Anatomy and Histology, School of Medicine, University of Zaragoza, Zaragoza, Spain.
authors.forename: Ana Isabel
authors.initials: AI
authors.lastname: Cisneros-Gimeno
authors.affiliation: Department of Obstetrics and Gynecology, Hospital Universitario Miguel Servet, Zaragoza, Spain.
authors.forename: Pilar
authors.initials: P
authors.lastname: Perez-Perez
authors.affiliation: Department of Obstetrics and Gynecology, Hospital Universitario Miguel Servet, Zaragoza, Spain.
authors.forename: Diego
authors.initials: D
authors.lastname: Lerma-Puertas
authors.affiliation: Department of Human Anatomy and Histology, School of Medicine, University of Zaragoza, Zaragoza, Spain.
authors.forename: Jaime
authors.initials: J
authors.lastname: Whyte-Orozco
mesh_terms: D000013:Congenital Abnormalities / Q000453:epidemiology*; D018592:Cross-Over Studies; D005260:Female; D006801:Humans; D015994:Incidence; D011247:Pregnancy; D012307:Risk Factors; D016216:Ultrasonography, Prenatal; D014471:Umbilical Veins / Q000002:abnormalities* / Q000000981:diagnostic imaging; D055815:Young Adult
keywords: congenital heart defects; congenital malformation; prenatal diagnosis; umbilical cord; umbilical veins