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.