Incidence of Persistent Left Superior Vena Cava in the Normal Population and in Patients with Congenital Heart Diseases Detected Using Echocardiography

Abstract

Reports on the incidence of persistent left superior vena cava (PLSVC) in the normal population are limited to studies involving pacemaker implantation candidates and cadavers. The incidence in patients with congenital heart diseases (CHDs) is estimated to be higher than that in the normal population; however, the details are unclear. To investigate the incidence of PLSVC in the normal population and in patients with CHDs, subjects were examined prospectively using echocardiography. Normal subjects consisted of 2841 successive neonates without intra-cardiac or congenital anomalies born in Gifu Prefectural General Medical Center. Additionally, 1920 patients with CHDs were evaluated. The incidence of PLSVC in normal neonates was 0.21% (95% confidence interval 0.042-0.38%). A high incidence (more than 7.0 times the incidence in normal subjects) was observed in all CHD patients. The high incidence group included coarctation of the aorta (CoA) (23.7%) and double outlet right ventricle (DORV) patients (24.6%). The second group consisted of CHD patients with ventricular septal defect (VSD), with an incidence ranging from 5.1 to 6.1%. The low incidence group comprised patients with other CHDs, with an incidence between 1.5 and 3.1%. The incidence of PLSVC in trisomy 21 and atrial septal defect patients was significantly higher than that in normal neonates. The incidence of PLSVC in the normal population and in patients with CHDs was systematically evaluated for the first time. The incidence in CHD patients appeared to be positively influenced by the type of CHD, particularly by DORV, CoA, and VSD.

Persistent left superior vena cava: clinical importance and differential diagnoses

“…Generally, the left common CV and the caudal part of the left superior CV will regress. If these veins do not regress, then they will persist as PLSVC [2,3,[8][9][10][11].…”

“…Additionally, it is informed that 45% of patients with PLSVC in the antenatal period have accompanying heterotaxy [15]. In a study, DSVC is detected in nearly half of patients with heterotaxy [8]. Another study reported that 72% of heterotaxy patients with SVC anomaly have DSVC, while the remaining have IPLSVC [23].…”

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

“…Increased risk of coarctation of aorta (CoA) was noticed in up to 21% of fetuses with PLSVC ( 3 ) . The ratio of the incidence of PLSVC in CHD patients to that in the normal population was very high (over hundredfold) in CoA and DORV group ( 6 ) . There is a high prevalence of ventricular septal defect, atrial septal defect, endocardial cushion defect, cortriatriatum and tetralogy of Fallot in patients with PLSVC and agenesis of the right SVC ( 15 ) .…”

“…This is the most common variant of anomalous systemic venous return in adults ( 3 ) . PLSVC is observed in 0,21% of the general population and 4–8% of patients with congenital heart disease (CHD) ( 5 , 6 ) . There is no sex difference in the incidence of PLSVC ( 6 ) .…”

Cardiac implantable electronic devices in patients with persistent left superior vena cava—A single center experience

“…The prevalence of persistent left superior vena cava (PLSVC) is approximately 0.2%‐0.6% in the general population 1 and is higher in patients with congenital heart disease 2 . The PLSVC most commonly drains into the right atrium through an enlarged coronary sinus (CS) 3 .…”