Agenesis of the ductus venosus that is associated with extrahepatic umbilical vein drainage: Prenatal features and clinical outcome

Objective: We sought to study the clinical and ultrasonographic findings and outcomes of fetuses with ductus venosus agenesis as the result of extrahepatic umbilical vein drainage. Study Design: This was an observational study of 12 fetuses who were diagnosed with these anomalies between 1995 and 2001. Results: The umbilical vein drained either directly into the right atrium (n = 7 fetuses), the inferior vena cava (n = 3 fetuses), or the iliac vein (n = 2 fetuses). Combined cardiac output (n = 8 fetuses) was nearly 2-fold increased to 891 ± 210 mL/kg per minute. Other relevant findings were (1) considerable umbilical vein enlargement (12/12 cases; range, 8-13 mm) with an accelerated (peak, 0.53 ± 0.1 m/s), (2) pulsatile Doppler flow pattern within its intra-abdominal course, (3) cardiomegaly (12/12 fetuses), (4) secundum atrial septal defects (5/12 fetuses), (5) extracardiac anomalies (5/12 fetuses), (6) polyhydramnios (4/12 fetuses), and (7) progressive heart failure (3/12 fetuses). Two fetuses died: 1 death was unexplained at 29 weeks of gestation; the other death from hydrops occurred after delivery at 32 weeks of gestation. So far, none of the survivors have had other long-term sequelae that were related to ductus venosus agenesis. Conclusion: Careful assessment of the umbilical venous return and the ductus venosus should be part of the routine evaluation of every fetus with heart failure and polyhydramnios. (Am J Obstet Gynecol 2002;187:1031-7.)