The relationship between umbilical–portal–systemic venous shunt and outcome in 11 fetuses

Liu Fang,He Deying,Ran Suzhen,Xiao Yangxue,Zhou Lan,Wang Xueyan,Zhang Xiaohang

doi:10.12688/f1000research.55600.1

Abstract

Objective: To investigate the relationship between congenital umbilical–portal–systemic venous shunt (UPSVs) and fetal outcome. Methods: The ultrasonographic and genetic characteristics of 11 cases of UPSVS were retrospectively analyzed and followed up to postnatal. Results: Four cases of ductus venosus -- systemic shunt (DVSS), one case of extrahepatic portal -- systemic shunt (EHPSS), and one case of umbilical systemic shunt (USS) combined with intrahepatic portal-systemic shunt (IHPSS), six cases of intrahepatic portal-systemic shunt (IHPSS) were observed. chromosomal abnormalities were observed in 9.1% (1/11), other ultrasonic abnormalities in 54.5% (6/11), cardiothoracic ratio increase in 45.5% (5/11), fetal growth restriction in 36.4% (4/11), edema was in 9.1% (1/11) and live birth was in 72.7% (8/11). Conclusion: The incidence of IHPSS is the highest and the outcome is the best. Shunt of DVSS and IHPSS can be closed spontaneously after birth. When the prenatal diagnosis is congenital UPSVs, chromosomal abnormalities and other ultrasonic abnormalities should be required further examination, and the growth and development of the fetus should be closely monitored.

Highlights

Congenital portosystemic shunt (CPSS) is associated with some complications, such as cholestasis, hyperammonemia, pulmonary arterial hypertension, hepatopulmonary syndrome, and benign and malignant liver tumors, in childhood
According to the umbilical-porta-systemic venous shunt (UPSVS) classification criteria, four cases were ductus venosus -- systemic shunt (DVSS), and six cases were intrahepatic portalsystemic shunt (IHPSS), including one case of umbilical systemic shunt (USS) combined with IHPSS and one case of extrahepatic portal -- systemic shunt (EHPSS)
According to the UPSVS classification criteria, four cases of DVSS, six cases of IHPSS, one case of USS combined with IHPSS and one case of EHPSS were recorded

Summary

Introduction

Congenital portosystemic shunt (CPSS) is associated with some complications, such as cholestasis, hyperammonemia, pulmonary arterial hypertension, hepatopulmonary syndrome, and benign and malignant liver tumors, in childhood. Portal systemic shunts were divided into extrahepatic and intrahepatic shunts They have been used to study fetal umbilical-portal system shunt and pediatric congenital portal systemic shunt.[2,3,4,5,6] The recent systematic reclassification of the term “umbilical-porta-systemic venous shunt (UPSVS)” has been considered as the best choice for fetal prognosis analysis. This classification is based on the theory of UV-PV-DV as an intact structure.

Methods

Results

Stringer MD