Amirhossein Moaddab1, Gabriele Tonni2, Gianpaolo Grisolia3, Maria Paola Bonasoni4, Edward Araujo Júnior5, Lilliam Cristine Rolo5, Federico Prefumo6, Sergio de la Fuente7, Waldo Sepulveda7, Nancy Ayres8, Rodrigo Ruano1. 1. a Department of Obstetrics and Gynecology , Texas Children's Hospital Fetal Center and Baylor College of Medicine , Houston , TX , USA . 2. b Prenatal Diagnostic Service, Department of Obstetrics and Gynecology, AUSL , Reggio Emilia , Italy . 3. c Prenatal Diagnostic Service, Department of Obstetrics and Gynecology, "Carlo Poma" Hospital , Mantua , Italy . 4. d Pathology Service, IRCCS Arcispedale "Santa Maria Nuova" , Reggio Emilia , Italy . 5. e Department of Obstetrics , Paulista School of Medicine - São Paulo Federal University of São Paulo (EPM-UNIFESP) , São Paulo , Brazil . 6. f Department of Obstetrics and Gynecology , University of Brescia , Brescia , Italy . 7. g Fetalmed - Maternal Fetal Diagnostic Center , Santiago , Chile , and. 8. h Department of Pediatric Cardiology , Texas Children's Hospital Fetal Center and Baylor College of Medicine , Houston , TX , USA.
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. CONCLUSION: Fetal hydrops, the presence of associated congenital anomalies and premature delivery are associated with poor prognosis in fetuses with ADV.
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. CONCLUSION: Fetal hydrops, the presence of associated congenital anomalies and premature delivery are associated with poor prognosis in fetuses with ADV.