Agenesis Of Ductus Venosus Research Articles

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.)

Prenatal diagnosis of ductus venosus agenesis and its association with cytogenetic/congenital anomalies.

We present an observational study of 12 cases of anomalies of the umbilical and portal vein systems associated with absence of the ductus venosus (DV) diagnosed over the past 5 years. The hemodynamic implications of each pattern of umbilico-portal system anomalies associated with absence of the DV have been investigated, as well as the frequency and types of associated anomalies and their embryological origin. In all cases ultrasound, color Doppler, and cytogenetic investigations were performed. Four main patterns of abnormal venous circulation were documented: (1). the umbilical vein (UV) bypasses the liver and drains into the right atrium directly or through a dilated coronary sinus (three cases); (2). the UV bypasses the liver, with an infrahepatic or suprahepatic connection directly to the inferior vena cava (IVC) (two cases); (3). the UV bypasses the liver and drains directly into the iliac or renal veins (four cases); and (4). the UV drains directly into the portal veins (three cases). Among seven cases with other associated anomalies (58%), there were three cases of Turner's and Noonan's syndromes. Two fetuses and two neonates died and there were two terminations of pregnancy (TOP). In utero diagnosis of ultrasound patterns associated with DV anomalies is feasible. Fetal karyotyping should be considered, serial ultrasound examinations recommended and, in the presence of heart failure, delivery can be anticipated.

Ductus venosus agenesis prevents transmission of central venous pulsations to the umbilical vein in fetal sheep

Velocity pulsations in the umbilical vein are widely used as a marker for congestive heart failure in the fetus. It has been suggested that the atrial pressure waves are transmitted to the umbilical vein mainly through the ductus venosus. In a case of ductus venosus agenesis in a fetal sheep of 110 days' gestation, we recorded pressures in the inferior vena cava and umbilical vein simultaneously with Doppler velocimetry in the medial hepatic vein and umbilical vein, under general anesthesia. During fetal respiratory acidosis (pH 7.01) and varying degrees of umbilical clamping, we did not observe the typical pulsations in the umbilical vein (deflections of the maximum velocity during atrial contraction) in spite of varying degrees of reversed flow in the hepatic vein (5-16%). The systolic-diastolic pressure amplitude in the inferior vena cava was 5.5-6.4 mmHg, sometimes reaching 9.19-11.03 mmHg. Varying degrees of reversed portocaval pressure gradient during atrial contraction (1.40-9.51 mmHg), periods of reversed portocaval pressure gradient throughout the entire cardiac cycle, tachycardia (240 beats/min) or bradycardia (99 beats/min) also did not produce the type of pulsation in the umbilical vein that reflects augmented atrial contractions. The results support the hypothesis that the ductus venosus is essential for the transmission of atrial pressure waves to the umbilical vein.