Prenatal Detection and Postnatal Outcome of Persistent Left Superior Vena Cava and Agenesis of Ductus Venosus Associated with Postnatal Bovine Aortic Arch

Abstract

Background Isolated agenesis of ductus venosus (ADV) is usually a benign condition, but it may be associated with cardiovascular defects, hydrops, growth restriction, and chromosomal abnormalities. Additionally, persistent left superior vena cava (PLSVC) and bovine aortic arch are relatively common fetal anomalies. To the author’s knowledge, this is the first report of prenatal detection of DV agenesis and PLSVC associated with the postnatal bovine aortic arch with a hypoplastic transverse aortic arch.

Case A 25-year-old, G2P1 woman was referred to our department at 31 weeks due to fetal growth restriction and short femur. On fetal echocardiography, DV could not be viewed via two-dimensional (2D) and Doppler ultrasound (US) imaging; there was also evidence of the co-occurrence of PLSVC and an aortic arch anomaly. We revealed the intrahepatic continuation of the umbilical vein. A weekly follow-up program was scheduled for the patient and the rest of the pregnancy was uneventful. Postnatal, thorax computer tomography and transthoracic echocardiography (TTE) demonstrated PLSVC and bovine aortic arch associated with hypoplastic transverse aortic arch. Routine echocardiographic examinations revealed that the blood flow of the aortic arch had increased gradually, and the male infant’s aortic arch had significantly widened and reached the normal range until the baby was discharged from the hospital.

Conclusion DV agenesis and PLSVC are usually benign conditions but underlying serious heart diseases may accompany them. Therefore, in situations like ours, a prenatal aortic arch evaluation is of capital importance. Postnatal hemodynamic changes should be taken into consideration in the management of these cases. This is the first example in the literature that these abnormalities co-existed in one case.

Key words

Ductus Venosus Agenesis - Transverse Arch Hypoplasia - Bovine Aortic Arch - Fetal Echocardiography

• References

• 1 Yagel S, Kivilevitch Z, Cohen SM. et al. The fetal venous system, part I: normal embryology, anatomy, hemodynamic, ultrasound evaluation and Doppler investigation. Ultrasound Obstet Gynecol 2010; 35: 741-750
• 2 Nagy RD, Iliescu DG. Prenatal diagnosis and outcome of umbilical-portal-systemic venous shunts: Experience of a tertiary center and proposal for a new complex type. Diagnostics (Basel) 2022; 12: 873 Published online 2022 Mar 31
• 3 Acherman RJ, Evans WN, Galindo A. et al. Diagnosis of absent ductus venosus in a population referred for fetal echocardiography: association with a persistent portosystemic shunt requiring postnatal device occlusion. J Ultrasound Med 2007; 26: 1077-1082
• 4 Han BH, Park SB, Song MJ. et al. Congenital portosystemic shunts: prenatal manifestations with postnatal confirmation and follow-up. J Ultrasound Med 2013; 32: 45-52
• 5 Pacheco D, Brandão O, Montenegro N. et al. Ductus venosus agenesis and fetal malformations: what can we expect? – A systematic review of the literature. J Perinat Med 2018; 47: 1-11
• 6 Nagy RD, Cernea N, Dijmarescu AL. et al. ductus venosus agenesis and portal system anomalies-association and outcome. Biology (Basel) 2022; 11: 548
• 7 Contratti G, Banzi C, Ghi T. et al. Absence of ductus venosus: report of 10 new cases and review of the literature. Ultrasound Obstet Gynecol 2001; 18: 605-609
• 8 Shen O, Valsky DV, Messing B. et al. Shunt diameter in agenesis of the ductus venosus with extrahepatic portosystemic shunt impacts on prognosis. Ultrasound Obstet Gynecol 2011; 37: 184-190
• 9 Gembruch U, Baschat AA, Caliebe A. et al. Prenatal diagnosis of ductus venosus agenesis: a report of two cases and review of the literature. Ultrasound Obstet Gynecol 1998; 11: 185-189