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Anomalies of Systemic Venous Return
Introduction
Congenital anomalies of systemic venous drainage represent a heterogeneous group of malformations. The most frequently reported anomalies are persistent left superior vena cava (PLSVC), absence of inferior vena cava (IVC), and agenesis of the ductus venosus. More rarely, right absent superior vena cava, persistent right umbilical vein, and total anomalous systemic venous return toward the coronary sinus have also been reported prenatally.
Abnormal systemic venous drainage may be isolated or associated with complex heart diseases, mainly heterotaxy syndromes ( Chapter 91 ), and chromosomal defects. Prognosis depends mainly on the association with other anomalies.
Disease
Definition
Anomalies of systemic venous return include abnormalities of the umbilical, portal, hepatic, and caval venous systems.
Prevalence and Epidemiology
Anomalous systemic venous return is rarely seen prenatally. The estimated prevalence is 0.3% to 0.5%.
Etiology and Pathophysiology
Although knowledge of the normal anatomy of the fetal venous system has increased enormously, little information is available on the mechanisms leading to abnormal in utero development. Anomalies of systemic venous drainage represent a heterogeneous group of malformations including abnormalities in cardinal, umbilical, and vitelline veins. The most common anomalies are described next.
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