Umbilical Vein Connection to Right Atrium with Absent Ductus Venosus: A Rare Congenital Defect

Introduction: An abnormal course of the Umbilical Vein (UV) is a rare anomaly and this can be associated with the congenital absence of the Ductus Venosus (DV), cardiac and extra-cardiac anomalies. The major clinical importance of these anomalies is the need to recognize them during prenatal and postnatal diagnostic studies and at the time of cannulation or catheterization of the umbilical vein in sick neonates. Through targeted examination of the fetal heart and the venous system, a correct prenatal diagnosis is possible.

Case Report: A 25-year-old gravida 1 para 0 with type 1 diabetes mellitus at 21+6 weeks gestation referred for fetal echocardiogram because abnormal vessel was noted connecting the UV to the right atrium on the obstetric ultrasound by her maternal fetal medicine specialist. A diagnosis of drainage of the UV directly into the right atrium with absent DV and other minor cardia defects were made on the fetal echocardiogram. Preterm delivery occurred at 31+3 weeks gestation and had a favorable clinical course.

Conclusion: An abnormal course of the umbilical vein is a rare anomaly and this can be associated with the congenital absence of the Ductus Venosus (DV), cardiac and extra-cardiac anomalies. Through a thorough and meticulous ultrasonographic assessment, the diagnosis can be made and that will be helpful in guiding the management of the rest of the pregnancy and counsel the family what to expect.

Author(s):

Osei FA and Brewer J

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