Gradual diagnosis and clinical importance of prenatally detected persistent left superior vena cava with absent right superior vena cava - a case report and literature review

Abstract

Persistent left superior vena cava is a malformation of cardinal veins. We report a case of a secundigravida who had many fetal ultrasound examinations - first performed by an obstetrician (who described fetal mediastinum as "abnormal"), second by other obstetrician, who performed basic fetal echocardiographic examination and diagnosed persistent left superior vena cava. The woman was referred to a tertiary center for detailed fetal echocardiography. The diagnosis of persistent left superior vena cava with agenesis of the right superior vena cava was confirmed. The anomaly had no influence on fetal hemodynamic stability, fetal life, delivery and early postnatal period. After delivery, the neonate was under observation for further anomalies, aortic coarctation in particular. Prenatal and postnatal management was summarized. Literature review is presented.

Persistent left superior vena cava is a malformation of cardinal veins. We report a case of a secundigravida who had many fetal ultrasound examinations – first performed by an obstetrician (who described fetal mediastinum as “abnormal”), second by other obstetrician, who performed basic fetal echocardiographic examination and diagnosed persistent left superior vena cava. The woman was referred to a tertiary center for detailed fetal echocardiography. The diagnosis of persistent left superior vena cava with agenesis of the right superior vena cava was confirmed. The anomaly had no influence on fetal hemodynamic stability, fetal life, delivery and early postnatal period. After delivery, the neonate was under observation for further anomalies, aortic coarctation in particular. Prenatal and postnatal management was summarized. Literature review is presented.

Fig. 1.

Fetal mediastinum- additional vessel, left to the main pulmonary artery. (AO – aorta, PA – main pulmonary artery, LSVC – left superior vena cava)

Fig. 2.

Aortic arch view (AscAo – ascending aorta, Aoisth – aortic isthmus, DscAo – descending aorta)

Fig. 3.

Flow through aortic arch

Fig. 4.

Left ventricular outflow tract. No disproportion between left and right heart (CS – coronary sinus)

Fig. 5.

Sketches of normal fetal heart (left) and fetal heart with PLSVC and agenesis of the right SVC. Arrows – flow through fetal heart (RA – right atrium, LA – left atrium, RV – right ventricle, LV – left ventricle, PA – main pulmonary artery, AO – aorta, DA – ductus arteriosus, RPVs – right pulmonary veins, LPVs – left pulmonary veins, FO – foramen ovale, IVC – inferior vena cava, LPSVC – left persistent vena cava)