nach oben

Erschienen in:

Open Access 01.12.2020 | Educational Review

Persistent left superior vena cava: clinical importance and differential diagnoses

verfasst von: Aynur Azizova, Omer Onder, Sevtap Arslan, Selin Ardali, Tuncay Hazirolan

Erschienen in: Insights into Imaging | Ausgabe 1/2020

Abstract

Persistent left superior vena cava (PLSVC) is the most common thoracic venous anomaly and may be a component of the complex cardiac pathologies. While it is often asymptomatic, it can lead to significant problems such as arrhythmias and cyanosis. Besides, it can cause serious complications during vascular interventional procedures or the surgical treatment of cardiac anomalies (CA). The clinical significance of PLSVC depends on the drainage site and the accompanying CA. In this article, we will describe the epidemiology, embryology, and anatomic variations of PLSVC. Possible accompanying CA and heterotaxy spectrum will be reviewed with the help of multidetector computed tomography (MDCT) images. Radiological pitfalls, differential diagnoses, and the clinical importance of PLSVC will be highlighted.

A correction to this article is available online at https://doi.org/10.1186/s13244-021-00983-x.

Publisher’s Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

3D VRT

Three-dimensional volume rendering technique

Atrial fibrillation

ALBV

Aberrant left brachiocephalic vein

ALSA

Aberrant left subclavian artery

APVD

Anomalous pulmonary venous drainage

ARSA

Aberrant right subclavian artery

ASD

Atrial septal defect

AVSD

Atrioventricular septal defect

AzV

Azygos vein

BT shunt

Blalock-Taussig shunt

Bridging vein

Cardiac anomalies

CCV

Common cardinal vein

CHD

Congenital heart disease

CoA

Coarctation of the aorta

Coronary sinus

CTMs

Conotruncal malformations

Cardinal vein

CVC

Central venous catheter

DORV

Double outlet right ventricle

DSVC

Double SVC

IPLSVC

Isolated PLSVC

ITVP

Inferior transverse venous plexus

IVC

Inferior vena cava

Internal jugular vein

L/D-TGA

Levo/dextro-transposition of the great arteries

Left atrium

LACV

Levoatriocardinal vein

LBCV

Left brachiocephalic vein

LICV

Left inferior cardinal vein

LOLs

Left-sided obstructive lesions

LSCV

Left superior cardinal vein

LSICV

Left superior intercostal vein

MDCT

Multidetector computed tomography

MRA

Magnetic resonance angiography

MRI

Magnetic resonance imaging

Oblique vein of the left atrium

Pulmonary atresia

PAPVD

Partial APVD

PCPV

Pericardiophrenic vein

PDA

Patent ductus arteriosus

PLSVC

Persistent left superior vena cava

Pulmonary stenosis

Right atrial structure

RAA

Right aortic arch

RICV

Right inferior cardinal vein

RSCV

Right superior cardinal vein

RSVC

Right superior vena cava

RVOTO

Right ventricular outflow tract obstruction

SCV

Subclavian vein

STVP

Superior transverse venous plexus

Sinus venosus;

SVC

Superior vena cava

Truncus arteriosus

TAPVD

Total APVD

TOF

Tetralogy of Fallot

VSD

Ventricular septal defect

Vertical vein

Key points

Persistent left superior vena cava (PLSVC) may lead to significant clinical symptoms and may affect surgical management.
PLSVC may accompany various congenital cardiac diseases as well as heterotaxy spectrum.
To be aware of the differential diagnoses of PLSVC is essential for correctly interpreting left-sided mediastinal vascular structures.

Background

Persistent left superior vena cava (PLSVC) is a rare vascular anomaly that begins at the junction of the left subclavian and internal jugular veins, passes through the left side of the mediastinum adjacent to the arcus aorta. It mostly drains into the right atrium via the coronary sinus (CS). Although PLSVC is infrequent among all vascular anomalies, it is the most common thoracic venous anomaly. Mostly, PLSVC is asymptomatic and detected incidentally in diagnostic and therapeutic examinations due to different reasons. However, it can be discovered as a component of the complex cardiac pathologies and may lead to significant problems such as arrhythmia [1‐4].

There are different modalities for evaluation of PLSVC, such as perinatal echocardiography, multidetector computed tomography (MDCT), magnetic resonance imaging (MRI), and invasive angiography. The advantages, disadvantages of these modalities, and optimal techniques for imaging of PLSVC are shown in Table 1 [5‐7].

Table 1

The advantages, disadvantages, and techniques of different modalities for evaluation of PLSVC

Imaging modality	Pros	Cons	Techniques
Echocardiography	✓ Cheap ✓ Widely available ✓ No ionizing radiation ✓ Not affected by cardiac rhythm ✓ Portable (bedside assessment) ✓ Real-time imaging ✓ Enables evaluation of flow direction	Difficult to interpret Operator-dependent Acoustic window dependent The spatial resolution could be limited.	* Coexistence of dilated coronary sinus without any evidence of right-sided congestion and positive “Bubble study” are diagnostic sonographic findings for PLSVC. * “Bubble study” is conducted with the injection of agitated saline from the left peripheral arm veins. If PLSVC is present, the agitated saline bubbles firstly are seen in the coronary sinus, before the right atrium. * In case of isolated PLSVC, positive “Bubble study” is observed after injection from right peripheral arm veins, as well. * Contrast-enhanced echocardiography and transesophageal echocardiography are other useful modalities for the detection of PLSVC.
Multidetector computed tomography	✓ Accessible ✓ Fast scanning speed ✓ The best spatial resolution ✓ Enables multiplanar imaging and reformatting	Radiation exposure (Recently developed dose reduction methods have partially reduced concerns about radiation exposure.) An iodinated contrast agent (allergy, nephrotoxicity) Cardiac rhythm changes may cause artifacts. Need for sedation in the pediatric age group	* “ECG-gated CCTA with thin slices and multiplanar reformation” provides a detailed assessment. * “Intravenous non-ionic iodinated contrast injection with a dose of 0,5-2 ml/kg at a rate of 1-2 ml/s” is recommended. *The identification of PLSVC is usually independent of the contrast injection route (right or left, upper or lower extremities). The optimal contrast opacification of PLSVC is mostly seen in the delayed venous phase images.
Magnetic resonance imaging	✓ Radiation free ✓ High spatial resolution ✓ Enables multiplanar image acquisition ✓ Enables assessment of flow direction ✓ Depiction of PLSVC even without the administration of contrast media ✓ Non-iodinated contrast	High cost Less accessible Slow scanning speed Contraindications such as the magnetic implant, claustrophobia. Cardiac rhythm changes may cause artifacts. Need for sedation in the pediatric age group	* Axial and coronal cine SSFP sequences are the best sequences for imaging of PLSVC. * The black blood TSE T2 images is also useful. * Contrast-enhanced MRA (± Dynamic imaging) and phase contrast angiography can be used as auxiliary modalities.
Invasive angiography	✓ Gold standard ✓ Excellent morphologic information ✓ Interventions can be made if necessary	Invasive Radiation exposure An iodinated contrast agent (Allergy, nephrotoxicity) Need for sedation in the pediatric age group	* The catheter angiography with water-soluble contrast agent is performed. Venograms are obtained after bolus contrast injection from the catheter. * Invasive angiography is not a routine imaging modality for evaluation PLSVC. * PLSVC can be detected incidentally during procedures like central venous catheter insertion or pacemaker implantation.

PLSVC persistent left superior vena cava, ECG-gated CCTA electrocardiogram-gated coronary computed tomography angiography, SSFP steady-state free precession, TSE turbo spin echo, MRA magnetic resonance angiography

In this article, we will describe the epidemiology, embryology, and anatomic variations of PLSVC. Possible accompanying cardiac anomalies (CA) and heterotaxy spectrum will be reviewed with the help of MDCT images. The radiological pitfalls with their CT imaging features that may help make the differential diagnosis, and the clinical importance of PLSVC will be highlighted.

Epidemiology

The exact frequency of PLSVC is not known because PLSVC is often asymptomatic and is detected incidentally. There is no significant difference in its prevalence between males and females. The prevalence of PLSVC ranges from 0.2 to 3% in the general healthy population. In patients with congenital heart disease (CHD), its prevalence ranges between 1.3 and 11%. Additionally, the prevalence of PLSVC is thought to be higher in the prenatal period since the accompanying anatomic anomalies, including heart defects, may cause spontaneous abortions and premature deaths [1‐3, 8, 9].

Embryology

The primitive venous system consists of three paired veins: vitelline veins (VV), umbilical veins (UV), cardinal veins (CV). Superior and inferior CVs are essential structures that allow the blood to return from the cranial and caudal parts of the embryo to the primitive heart. They combine to form common CVs (or the duct of Cuvier) draining into the double horned sinus venosus [2, 3, 9]. The caudal part of the right superior CV, together with the common CV, forms the right superior vena cava (RSVC). 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, 8‐11]. The detailed schematic anatomy of the developmental stages of the primitive venous system is shown in Fig. 1.

Different hypotheses regarding the development of PLSVC have been proposed. One of these is “low left atrial pressure theory.” According to this theory, in the presence of anomalies, which may cause reduced left atrial pressure and insufficient development of the left atrium, such as atrioventricular septal defect (AVSD), the left atrium will be smaller than expected. Thus, it will not be able to compress the CS and left CVs adequately. As such, the left common CV and caudal part of the left superior CV will not regress, and PLSVC will develop. Some hypotheses suggest the vice versa. According to the “obstructive theory” hypothesis, the presence of PLSVC, which may cause an increase in CS size, could lead to the formation of a left-sided obstructive lesion because of the space restriction [1].

Drainage site and its impact on the anatomy

PLSVC is responsible for approximately 20% of the total venous blood return from the left arm, left half of the head and neck. The right atrial drainage is seen in 80–90% of cases, while the left atrial drainage accounts for the remaining 10–20%. Generally, it joins into the right atrium through the CS and mostly has no hemodynamic effect. However, CS ostial atresia may accompany PLSVC. In that case, PLSVC becomes the major retrograde drainage pathway for coronary veins unless collateral drainage pathways develop between the coronary sinus and the heart chambers. The left atrial drainage, which is rare, occurs directly via the left atrial appendage or indirectly through the left pulmonary veins or the CS. In some sources, the latter is defined as an unroofed CS or CS atrial septal defect. The association of the atrial septal defect (ASD) and PLSVC draining into the left atrium via unroofed CS is called as Raghib syndrome (Fig. 2a–f) [2‐4, 9, 12].

Left atrial drainage is a cause of right-to-left shunt and is mostly accompanied by CA. However, it was reported that this condition might also be observed without any cardiac defects [2, 3, 12].

It has been reported that the drainage of more than the expected venous blood volume into the right atrium leads to some changes in the heart anatomy. Of those, the most well-known is the enlargement of the CS, which is a helpful clue indicating PLSVC existence. This enlargement may rarely reach the aneurysmatic level (Fig. 2g, h). There are also other anatomical changes reported in the literature, and they are described in Table 2, together with possible underlying mechanisms [2, 3, 12].

Table 2

Anatomical changes and possible underlying mechanisms in the presence of PLSVC

Reported anatomical changes	Possible underlying mechanisms
The decrease in RSVC dimensions	Reduction of blood volume drained through RSVC
Decrease in mitral valve area	Compression to the left atrium via dilated CS
Atrophy of the valves of cardiac veins such as Vieussens, Thebesian	Increased blood volume draining into the CS
The presence of a common left pulmonary vein trunk	Limited space caused by the dilated CS
Increase in heart weight	–

PLSVC persistent left superior vena cava, RSVC right superior vena cava, CS coronary sinus

Presence of RSVC and bridging vein

In up to 90% of the cases, the right superior vena cava (RSVC) accompanies PLSVC, and this situation is known as double SVC (DSVC). If the caudal part of the right superior CV regresses in the intrauterine period, RSVC cannot develop, resulting in the presence of isolated PLSVC (IPLSVC). Mostly, IPLSVC is associated with CA and cardiac situs disorders. However, there are examples of IPLSVC without any accompanying apparent CA in the literature. In cases of DSVC, dimensions of RSVC may be larger or smaller than PLSVC (Fig. 3a–c) [2, 3, 9, 13].

In 65% of the cases, DSVC runs along each side of the mediastinum without interconnection. However, there could be the left brachiocephalic vein (LBCV) connecting them, which is also called the bridging vein (BV) (Fig. 3d, e) [3].

PLSVC and accompanying cardiac anomalies

To date, many CA associated with PLSVC have been identified and grouped in different ways [1, 14, 15]. Shunt lesions (Figs. 4 and 5), conotruncal malformations (CTMs) (Figs. 6 and 7), left-sided obstructive lesions (LOLs) (Fig. 8), right-sided lesions, and single ventricular anomalies (Fig. 9) constitute the main CA groups. Aortic arch anomalies are also associated with PLSVC (Figs. 10 and 11). The subgroups of these anomalies are listed in Table 3. Besides, a summary of the literature about PLSVC and accompanying CAs is compiled in Table 4. Additionally, heterotaxy forms another disease spectrum associated with PLSVC and will be discussed under a separate title.

Table 3

Main groups and subgroups of cardiac/aortic arch anomalies associated with PLSVC

Main groups	Subgroups
Shunt lesions	ASD, VSD, AVSD, PDA, APVD
Conotruncal malformations	TOF, PA with VSD, L/D-TGA, TA, DORV
Left-sided obstructive lesions	CoA, cor triatriatum, mitral stenosis, bicuspid aortic valve
Right-sided lesions	PS, PA, tricuspid atresia, bicuspid pulmonary valve, Ebstein anomaly
Single ventricular anomalies	None
Aortic arch anomalies	Cervical arch, RAA, ARSA, RAA + ALSA

PLSVC persistent left superior vena cava, ASD atrial septal defect, VSD ventricular septal defect, AVSD atrioventricular septal defect, PDA patent ductus arteriosus, APVD anomalous pulmonary venous drainage, TOF tetralogy of fallot, PA pulmonary atresia, L/D TGA-levo/dextro-transposition of the great arteries, TA truncus arteriosus, DORV double outlet right ventricle, CoA coarctation of the aorta, PS pulmonary stenosis, RAA right aortic arch, ARSA aberrant right subclavian artery, ALSA aberrant left subclavian artery

Table 4

Summary of literature about PLSVC and accompanying CA

Name of the author	Reported findings of PLSVC and associated CA
Perles et al. [1]	The most common groups of anomalies associated with PLSVC (Based on odds ratio)	AVSD, CTMs, LOLs
Nagasawa et al. [6]	The highest incidence group of cardiac anomalies according to PLSVC index	CoA and DORV
Lendzidan et al. [14]	The most common cardiac anomalies associated with PLSVC	Single ventricle, AVSD, TOF
Ari et al. [12]	The most common cyanotic heart diseases associated with PLSVC	DORV and TOF
Ari et al. [12]	The most common acyanotic heart diseases associated with PLSVC	ASD and PDA
Berg et al. [13]	The most common concomitant anomalies in patients with heterotaxy and PLSVC	AVSD, RVOTO, DORV
Berg et al. [13]	The most common concomitant anomalies in patients with PLSVC, without heterotaxy	VSD and CoA
Oztunc et al. [16]	The most common anomalies with PLSVC drained into the right atrium	TOF and PS
Oztunc et al. [16]	The most common anomalies with PLSVC drained into the left atrium	Tricuspid atresia, TGA, situs anomalies

PLSVC persistent left superior vena cava, CA cardiac anomaly, AVSD atrioventricular septal defect, CTMs conotruncal malformations, LOLs left-sided obstructive lesions, CoA coarctation of the aorta, DORV double outlet right ventricle, TOF tetralogy of fallot, ASD atrial septal defect, PDA patent ductus arteriosus, RVOTO right ventricular outflow tract obstruction, VSD ventricular septal defect, PS pulmonary stenosis, TGA transposition of great arteries

In the literature, there is a wide range of information about the frequency of cardiac anomalies accompanying PLSVC [1, 6, 14‐22]. According to Lendzidan et al., the most common cardiac anomalies accompanying PLSVC are single ventricle, atrioventricular septal defect (AVSD), and tetralogy of Fallot (TOF). Cha et al. reported that the most frequent concomitant anomaly is ASD, whereas, according to Eldin et al., complete atrioventricular septal defect comes the first [17‐19].

Moreover, attention has been drawn to the relationship of some specific cardiac anomalies with PLSVC in many publications. In addition to left-sided pathologies such as mitral atresia, cor triatriatum, and hypoplastic left heart, transposition of the great arteries (TGA) and tricuspid atresia are other rarer anomalies that have been reported to be closely related to PLSVC in the literature [6, 16, 20].

Different cardiac anomalies come to the fore in different situations such as type of accompanying cardiac anomaly (cyanotic or acyanotic), presence of heterotaxy, and drainage location of PLSVC [14, 15, 21]. Different parameters, such as odds ratio and PLSVC index, are calculated in the literature and used to determine the relationship between PLSVC and cardiac anomaly [1, 8]. In some publications, cardiac anomalies accompanying PLSVC were grouped and evaluated as in Table 3, and in others, they were examined separately [1, 17].

Association of PLSVC with aorta-related pathologies such as right-sided arcus aorta (RAA) and coarctation of the aorta (CoA) have also been emphasized in the literature. It was mentioned that the association of PLSVC with RAA is approximately 16% [14]. In another study, CoA was reported to be an independent and powerful factor for the existence of PLSVC [8]. Gustapane et al. underlined the coexistence of PLSVC with coarctation of the aorta (CoA) (21.3%) and suggested that fetuses with PLSVC that are detected in the antenatal period should be followed during pregnancy in terms of CoA development [22].

PLSVC and heterotaxy

The term heterotaxy comprises situs inversus and situs ambiguus (right/left isomerism) (Fig. 12). DSVC or IPLSVC anomalies may be present in patients with heterotaxy. Meanwhile, in a study, “patients with both IPLSVC and situs inversus” were considered normal because of mirror image and excluded. In contrast, “patients with both isolated RSVC and situs inversus” were regarded as abnormal and accepted as SVC anomaly [1].

According to the literature, PLSVC-heterotaxy coexistence is frequently observed, and PLSVC is present in 50–70% of heterotaxy cases. 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].

According to a study, while right atrial isomerism in patients with PLSVC is about 7%, left atrial isomerism is about 9% [14]. In another study, those prevalences are nearly 15% and 30%, respectively. It was stated that the absence of inferior vena cava (IVC) is associated with left atrial isomerism, while the juxtaposition of IVC is observed in right atrial isomerism [15] (Fig. 13).

Complete atrioventricular septal defect, right ventricular outflow tract obstruction (RVOTO) (pulmonary stenosis and atresia), and double outlet right ventricle (DORV) are found as the most common accompanying anomalies of PLSVC in patients with heterotaxy [15] (Table 4, Fig. 14). The presence of concomitant heterotaxy and atrioventricular septal defect in patients with PLSVC during the antenatal period has been associated with poor prognosis.

Berg et al. reported that they never saw CS dilatation, which is a well-known sonographic finding supporting the presence of PLSVC, in the heterotaxy group. The absence of CS dilatation has been associated with unroofed CS, which is found in almost all heterotaxy cases. It should be kept in mind that the absence of CS dilatation does not exclude the presence of PLSVC in patients with heterotaxy during the antenatal period, and the possibility of concomitant unroofed CS anomaly is high [15].

Clinical importance

The clinical significance of PLSVC depends on the drainage site and the accompanying anomalies. PLSVC without CA is generally asymptomatic and is detected as an incidental finding. In the case of PLSVC with right atrial drainage, the CS often expands (Fig. 2g, h). This enlargement may cause compression of the atrioventricular node and His bundle. So, it can lead to cardiac arrhythmias, such as atrial/ventricular fibrillation. The compression of the left atrium and decreased cardiac output may occur due to this enlargement. Moreover, the presence of CS dilatation may complicate mitral valve surgery due to the close anatomic relationship [2, 9, 12, 19].

In a recent study by Yun Gi Kim et al. [24], it was demonstrated that PLSVC plays a considerable role in the induction and maintenance of atrial fibrillation (AF) in nearly half of the patients. So, pre-radiofrequency catheter ablation cardiac imaging in AF patients is useful and necessary for not only the evaluation of pulmonary venous anatomy but also for the detection of PLSVC existence. If PLSVC is detected as the trigger or driver of AF, it can be ablated (Fig. 2c).

It is crucial to know the PLSVC existence in advance in invasive procedures, such as central venous catheter (CVC) insertion (Fig. 2a, b), cardiac resynchronization therapy leads, or pacemaker implantation. It may complicate pacemaker implantation by causing fixation difficulties of the electrode due to the tortuous course. CVC insertion without fluoroscopy may cause angina, hypotension, and heart perforation. Furthermore, there may be constriction or atresia of the CS ostium. In this case, the catheterization will be challenging and may result in serious complications, such as dangerous arrhythmias, cardiogenic shock, and tamponade [2, 9, 12, 14].

The presence of CS ostial atresia is also critical in the operations that require PLSVC ligation. In this case, the CS still drains the blood from the coronary veins to the right atrium via the retrograde PLSVC-LBCV-RSVC pathway, instead of the atretic ostium. The ligation of PLSVC will be catastrophic due to the acute interruption of the cardiac venous drainage [12].

The left atrial drainage of PLSVC (Fig. 2d–f), sometimes, remains asymptomatic because it does not cause a right-to-left shunt at a significant level. In cases where the shunt is more pronounced, as a result of desaturation, the condition manifests itself with severe cyanosis, syncope, reduced exercise tolerance, and progressive fatigue. Thromboembolic events and even brain abscesses may develop in these patients. In this case, treatment can be done in two ways based on anatomy: PLSVC can be ligated if there is an adequate sized BV, and PLSVC can be re-anastomosed to the CS if the BV is not adequate in size or there is no RSVC [2, 9].

The knowledge of PLSVC is fundamental in some cardiac surgeries such as venous rerouting procedures, operations with cavo-pulmonary anastomosis (Glenn, Fontan), and heart transplantation. In heart transplantation surgery, if PLSVC without BV is present in the recipient’s heart, the bicaval anastomosis technique will be performed. It requires separation of the CS of the donor’s heart for the establishment of the recipient’s PLSVC anastomosis to the donor’s right atrium [1].

In the case of unknown PLSVC, retrograde cardioplegia, a common practice for cardiac surgeries for myocardial protection, will be ineffective. Clamping of PLSVC may be required for the prevention of retrograde flow. However, cardioplegia may fail even after clamping of PLSVC, due to the steal effect by the hemiazygos venous system linked to PLSVC [1, 3].

During cardiopulmonary bypass, not knowing PLSVC existence may result in both surplus blood return through the right atrium and insufficient venous return to the pump. This problem is mostly encountered in pathologies such as pulmonary atresia, tricuspid atresia, TOF, where increased systemic venous pressure gets over the level of left atrial pressure [19].

With the help of screening echocardiography, PLSVC can be detected as early as in the prenatal period. It can be used as a marker for cardiac or non-cardiac embryopathy. It may require extensive evaluation to exclude possible developmental anomalies. In cases with CHD, symptoms will be mainly due to these anomalies [1].

Pitfalls and differential diagnoses

In the presence of the vessel on the left side of the aorta in the mediastinum, other vascular structures apart from PLSVC should be considered in the differential diagnosis. They are vertical vein, levoatriocardinal vein, left superior intercostal vein, aberrant left brachiocephalic vein, pericardiophrenic vein, and vascular structures secondary to surgery.

To make the definitive diagnosis, features which should be taken into consideration are as follows: “origin site,” “drainage site,” “orientation of the route between the origin and drainage site according to mediastinal structures,” “the expected direction of the blood flow,” and “characteristics of accompanying cardiac and non-cardiac diseases.” According to the above-mentioned features, a comprehensive summary of the differential diagnoses of PLSVC is depicted in Fig. 15.

Some masses on the expected course of PLSVC could be confusing at first look due to their location. For making the differential diagnosis, it is essential to follow all of the slices carefully and see the beginning and end of the mass (Fig. 16) [25, 26].

Moreover, an interesting variant of PLSVC, which has an intra-atrial course within the left atrium, has been identified recently. If this pitfall variant is not known, it may be misunderstood as left atrial cystic mass, may cause patient anxiety, and may lead to unnecessary effort for further investigations [27].

Vertical vein

The vertical vein (VV) is the vessel that drains the blood from the pulmonary veins into the LBCV in the presence of supracardiac type total or partial APVD (TAPVD or PAPVD) (Fig. 17). It may be left- or right-sided. The left APVD accounts for approximately 18% of all PAPVD and left superior pulmonary veins are affected mostly. The left-sided VV is one of the differential diagnoses of PLSVC. The critical point in the distinction is the caudal continuity of the vessel with atrial chambers. If there is no continuity, it is compatible with the VV. However, PLSVC may have a direct connection with the left pulmonary veins. In this scenario, the pulmonary vein drains into the left atrium after joining PLSVC [12, 28].

There are also some auxiliary features to differentiate PLSVC and VV. The expected flow direction is craniocaudal in PLSVC, while it is caudocranial in the VV. In the case of PLSVC, there are two vessels in the anterior aspect of the left main bronchus: one of them is PLSVC, and the other one is the left superior pulmonary vein. Ordinarily, only the left superior pulmonary vein is expected to be at this location. However, in the case of the VV with PAPVD, no vessel is seen in the anterior aspect of the left main bronchus. The size of the LBCV can also be helpful in finding for differentiation. In the case of APVD, the LBCV and RSVC may be of large caliber because the VV transports blood via these venous structures. On the other hand, PLSVC, frequently, is associated with an absent or small-sized LBCV [12, 28].

Levoatriocardinal vein

The levoatriocardinal vein (LACV) is the interatrial connection that originates from the left atrium (68%) or pulmonary vein (32%). It drains into one of the systemic venous structures, mostly, into the LBCV (48%) (Figs. 18 and 19) [29, 30].

The differentiation of LACV from PLSVC with right atrial drainage is straightforward. Because this drainage site is unlikely for the levoatriocardinal vein. Similarly, in cases where PLSVC drains into the left atrium via the unroofed CS, unroofed CS and ASD facilitate the differential diagnosis in favor of PLSVC, since they are unusual for LACV [29‐31].

However, PLSVC may drain directly into the left atrium or pulmonary vein. In this situation, the expected origin and drainage site of those two vessels will be the same, and it is necessary to search other features for distinguishment. The anatomical feature that may help distinguish is their relative orientation according to the left pulmonary artery. PLSVC is seen in the anterior aspect of the left pulmonary artery, while the LACV is in the posterior aspect. The evaluation of the flow direction with echocardiography or velocity-encoded cine magnetic resonance imaging is another way to make differential diagnoses. The blood flows in the caudocranial direction in the LACV while it flows craniocaudal direction in the PLSVC. However, the bidirectional flow could be seen in the levoatriocardinal vein [29‐31].

Moreover, the caudocranial flow may be observed in PLSVC when there is atresia or stenosis of the CS ostium. Identification of accompanying CA may also help in the differential diagnosis. If LOLs without ASD are present, LACV should be considered in the differential diagnosis, firstly. It is hypothesized that, in the presence of in utero LOLs such as mitral stenosis, collaterals between pulmonary and systemic circulations cannot regress due to increased pressure in the left atrium and remain as LACV in the postnatal period. However, in the presence of complex CA, the diagnosis of PLSVC should be considered mainly [29‐31].

LACV could be isolated without any CA, like PLSVC. Nevertheless, the frequency of this probability is very low for LACV compared to PLSVC. Additionally, they may be seen together, and the LACV may drain into PLSVC [28, 30].

Pericardiophrenic vein

The pericardiophrenic veins (PCPV) are responsible for pericardial and diaphragmatic venous drainage. They lie along the lateral border of the heart and mediastinum, accompany pericardiophrenic arteries/phrenic nerve and drain into the internal thoracic, superior intercostal, or BCV. Due to the connection with inferior phrenic veins, dilated PCPVs could be observed as a collateral pathway in cases of SVC or IVC occlusion. Besides, they can serve as a collateral route via portosystemic shunting in portal hypertension (Fig. 20) [32‐34].

In the case of catheters located at the left paramediastinal region, the left PCPV is one of the possible differential diagnoses. In posteroanterior chest X-ray, left PCPV has a lateral course along the left heart border, while PLSVC turns medially near the left atrium. Although they both are located in the middle mediastinum and connected with left brachiocephalic vein cranially, their caudal courses differ in CT imaging. While the caudal end of PLSVC is either the coronary sinus or the left atrium, the left pericardiophrenic vein moves toward the diaphragm lateral to the heart when it is followed from top to bottom [35, 36].

Left superior intercostal vein

The left superior intercostal vein (L-SICV) drains the blood from the second, third, and fourth left intercostal veins into RSVC through the hemiazygos/azygos venous systems. Ordinarily, it can be seen as a small aortic nipple (1.4–5%) on the chest radiograph and is indistinguishable in CT. If its diameter exceeds 4.5 mm, it should be considered as abnormal. In the case of occlusion of SVC at the distal level of the azygos vein, the connection between SVC and IVC becomes possible with the dilation of L-SICV and other collateral vessels (Fig. 21) [9, 37, 38].

Furthermore, L-SICV may dilate in congenital conditions such as hypoplasia of LBCV, and diseases leading to volume overload such as congestive heart failure. In such cases, L-SICV might be confused with PLSVC. However, knowing their courses and drainage sites will facilitate the diagnosis [9, 37, 38].

Aberrant left brachiocephalic vein

Aberrant left brachiocephalic vein (ALBV) is a rare anomaly (≈ 1%) and is often associated with CAs, such as TOF, septal defects, and right atrial isomerism. Ordinarily, the LBCV passes through the anterior of the arcus aorta and connects with the right BCV. In the presence of an aberrant course, the LBCV begins with the junction of the left subclavian and jugular veins, moves inferiorly along the left side of the mediastinum, and joins to the right BCV passing behind the ascending aorta or esophagus. Retroesophageal ALBV is a more rare variation (Fig. 22) [10, 11, 39].

Vascular structures secondary to surgery

Vascular structures located on the left side of the mediastinum in patients with the history of cardiac surgery performed for complex CA may also be included in the differential list of PLSVC. The differential diagnosis could be made by knowing the performed surgery and demonstrating the drainage site of the vessel. Bicaval Glenn shunt, the left-sided Blalock-Taussig (BT) shunt, and collateral vessels after Fontane surgery are possible differentials (Fig. 23).

Bicaval Glenn shunt is an anastomosis of both SVCs to pulmonary arteries in the presence of PLSVC. The Glenn shunt allows the direct drainage of venous blood into the pulmonary arteries via bypassing the right heart chambers [40, 41].

BT shunt is one of the surgical methods for complex CA. In this procedure, the connection of the subclavian artery and the pulmonary artery is enabled via the graft placement [40, 42].

In Fontan surgery, the SVC and IVC are anastomosed to the pulmonary artery. After surgery, collaterals, which may be seen as large vessels on the left side of the mediastinum, may develop and may be confused with PLSVC [43].

Conclusion

In conclusion, PLSVC is the most common thoracic venous anomaly known to be mostly asymptomatic. However, contrary to common misconception, it may cause a number of clinically significant symptoms, even in a heart with normal anatomy. Likewise, it may significantly affect the proper approaches to heart transplantations, effective surgical treatments for complex cardiac anomalies, and ablative procedures for cardiac arrhythmias. Thus, it should be recognized correctly and reported explicitly in radiological reports, even when it is an incidental finding. Besides, it is important to be aware of differential diagnoses of PLSVC and their radiological features to correctly interpret the vascular structures on the left side of the mediastinum.

Acknowledgements

A part of this paper was submitted as an educational exhibit to RSNA 2020.

Not applicable.

Competing interests

The authors declare that they have no competing interests.

Open AccessThis article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/.

Publisher’s Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Perles Z, Nir A, Gavri S et al (2013) Prevalence of persistent superior vena cava and association with congenital heart anomalies. Am J Cardiol 112(8):1214–1218. https://doi.org/10.1016/j.amjcard.2013.05.079

Tyrak KW, Holda J, Holda MK, Koziej M, Piatek K, Klimek-Piotrowska W (2017) Persistent left superior vena cava. Cardiovasc J Afr 28(3):e1–e4. https://doi.org/10.5830/CVJA-2016-084CrossRefPubMedPubMedCentral

Goyal SK, Punnam SR, Verma G, Ruberg FL (2008) Persistent left superior vena cava: a case report and review of literature. Cardiovasc Ultrasound 6:50. https://doi.org/10.1186/1476-7120-6-50CrossRefPubMedPubMedCentral

Hutyra M, Skala T, Sanak D, Novotny J, Köcher M, Taborsky M (2010) Persistent left superior vena cava connected through the left upper pulmonary vein to the left atrium: an unusual pathway for paradoxical embolization and a rare cause of recurrent transient ischaemic attack. Eur J Echocardiogr 11(9):E35–E35. https://doi.org/10.1093/ejechocard/jeq079

Ahmed S, Johnson PT, Fishman EK, Zimmerman SL (2013) Role of multidetector CT in assessment of repaired tetralogy of fallot. Radiographics 33(4):1023–1036. https://doi.org/10.1148/rg.334125114

Sheikh AS, Mazhar S (2014) Persistent left superior vena cava with absent right superior vena cava: review of the literature and clinical implications. Echocardiography 31(5):674–679. https://doi.org/10.1111/echo.12514CrossRefPubMed

Batouty NM, Sobh DM, Gadelhak B et al (2020) Left superior vena cava: cross-sectional imaging overview. Radiol Med 125(3):237–246. https://doi.org/10.1007/s11547-019-01114-9

Nagasawa H, Kuwabara N, Goto H et al (2017) Incidence of Persistent Left Superior Vena Cava in the Normal Population and in Patients with Congenital Heart Diseases Detected Using Echocardiography. Pediatr Cardiol 39(3):484–490. https://doi.org/10.1007/s00246-017-1778-3CrossRefPubMed

Sonavane SK, Milner DM, Singh SP, Abdel Aal AK, Shahir KS, Chaturvedi A (2015) Comprehensive imaging review of the superior vena cava. Radiographics 35(7):1873–1892. https://doi.org/10.1148/rg.2015150056

10.

Goel AN, Reyes C, Mclaughlin S, Wittry M, Fiore AC (2016) Retroesophageal left brachiocephalic vein in an infant without cardiac anomalies. Prenatal Cardiol 6(1):87–89. https://doi.org/10.1515/pcard-2016-0012CrossRef

11.

Kobayashi M, Ichikawa T, Koizumi J et al (2018) Aberrant left brachiocephalic vein versus persistent left superior vena cava without bridging vein in adults: evaluation on computed tomography. Ann Vasc Dis 11(4):535–541. https://doi.org/10.3400/avd.oa.18-00098CrossRefPubMedPubMedCentral

12.

Demos TC, Posniak HV, Pierce KL, Olson MC, Muscato M (2004) Venous anomalies of the thorax. AJR Am J Roentgenol 182(5):1139–1150. https://doi.org/10.2214/ajr.182.5.1821139

13.

Albay S, Cankal F, Kocabiyik N, Yalcin B, Ozan H (2006) Double superior vena cava. Morphologie 90(288):39–42. https://doi.org/10.1016/s1286-0115(06)74317-xCrossRefPubMed

14.

Ari ME, Doğan V, Özgür S et al (2017) Persistent left superior vena cava accompanying congenital heart disease in children: Experience of a tertiary care center. Echocardiography 34(3):436–440. https://doi.org/10.1111/echo.13447CrossRefPubMed

15.

Berg C, Knüppel M, Geipel A et al (2006) Prenatal diagnosis of persistent left superior vena cava and its associated congenital anomalies. Ultrasound Obstet Gynecol 27(3):274–280. https://doi.org/10.1002/uog.2704CrossRefPubMed

16.

Nsah EN, Moore GW, Hutchins GM (1991) Pathogenesis of persistent left superior vena cava with a coronary sinus connection. Pediatr Pathol 11(2), 261–269. https://doi.org/10.3109/15513819109064763

17.

Lendzian T, Vogt J, Krasemann T (2007) Are anomalies of the caval veins more common in complex congenital heart disease? Herz 32(8):657–664 doi.org/10.1007/s00059-007-2935-xCrossRef

18.

Cha EM, Khoury GH (1972) Persistent Left Superior Vena Cava. Radiologic and clinical significance. Radiology 103(2):375–381. https://doi.org/10.1148/103.2.375CrossRefPubMed

19.

Eldin GS, El-Segaier M, Galal MO (2013) High prevalence rate of left superior vena cava determined by echocardiography in patients with congenital heart disease in Saudi Arabia. Libyan J Med 8(1):21679. https://doi.org/10.3402/ljm.v8i0.21679CrossRefPubMed

20.

Bezante GP, Deferrari L, Molinari G, Valbusa A, Rosa G, Barsotti A (2002) Cor triatriatum sinistrum and persistent left superior vena cava: an original association. Eur J Echocardiogr 3(2):162–165. https://doi.org/10.1053/euje.2002.0142

21.

Oztunc F, Pac A, Ozme S et al (1994) Persistan Sol süperior vena kava (145 Olgu Nedeniyle) LEFT PERSİSTENT VENA CAVA SUPERIOR. Turkiye Klinikleri J Cardiol 7(3):159–162

22.

Gustapane S, Leombroni M, Khalil A et al (2016) Systematic review and meta-analysis of persistent left superior vena cava on prenatal ultrasound: associated anomalies, diagnostic accuracy and postnatal outcome. Ultrasound Obstet Gynecol 48(6):701–708. https://doi.org/10.1002/uog.15914CrossRefPubMed

23.

Buirski G, Jordan SC, Joffe HS, Wilde P (1986) Superior vena caval abnormalities: their occurrence rate, associated cardiac abnormalities and angiographic classification in a paediatric population with congenital heart disease. Clin Radiol 37(2):131–138. https://doi.org/10.1016/s0009-9260(86)80382-8CrossRefPubMed

24.

Kim YG, Han S, Choi JI et al (2019) Impact of persistent left superior vena cava on radiofrequency catheter ablation in patients with atrial fibrillation. EP. Europace 254. https://doi.org/10.1093/europace/euz254

25.

Padhani AR, Hale HL (1998) Mediastinal venous anomalies: potential pitfalls in cancer diagnosis. Br J Radiol 71(847):792–798. https://doi.org/10.1259/bjr.71.847.9771393CrossRefPubMed

26.

Abbara S, Imbesi SG, Walker TG (2013) Diagnostic imaging cardiovascular. Salt Lake City, United States

27.

Irwin RB, Greaves M, Schmitt M (2012) Left superior vena cava: revisited. Eur Heart J Cardiovasc Imaging 13(4):284–291. https://doi.org/10.1093/ehjci/jes017CrossRefPubMed

28.

Lyen S, Wijesuriya S, Ngan-Soo E et al (2017) Anomalous pulmonary venous drainage: a pictorial essay with a CT focus. J Congenital Cardiol 1(1). https://doi.org/10.1186/s40949-017-0008-4

29.

Agarwal PP, Mahani MG, Lu JC, Dorfman AL (2015) Levoatriocardinal vein and mimics: spectrum of imaging findings. AJR Am J Roentgenol 205(2):W162–W171. https://doi.org/10.2214/ajr.15.14365CrossRefPubMed

30.

Bernstein HS, Moore P, Stanger P, Silverman NH (1995) The levoatriocardinal vein: Morphology and echocardiographic identification of the pulmonary—systemic connection. J Am Coll Cardiol 26(4):995–1001. https://doi.org/10.1016/0735-1097(95)00283-xCrossRefPubMed

31.

Odemis E, Saygili O, Akdeniz C, Karaci A (2011) Levoatriocardinal vein with normal intracardiac anatomy and pulmonary venous return. Ann Pediatr Cardiol 4(2):183. https://doi.org/10.4103/0974-2069.84667

32.

Ozawa Y, Suzuki R, Hara M, Shibamoto Y (2018) Identification of the pericardiacophrenic vein on CT. Cancer Imaging 18(1). https://doi.org/10.1186/s40644-017-0134-4

33.

Broderick LS, Brooks GN, Kuhlman JE (2005) Anatomic Pitfalls of the Heart and Pericardium. Radiographics 25(2):441–453. https://doi.org/10.1148/rg.252045075CrossRefPubMed

34.

Sharma M, Rameshbabu CS (2012) Collateral pathways in portal hypertension. J Clin Exp Hepatol 2(4):338–352. https://doi.org/10.1016/j.jceh.2012.08.001CrossRefPubMedPubMedCentral

35.

Krishnan A, Cacciarelli A, Gibson D (2004) Unusual complication of peripherally inserted central venous catheter placement: the left pericardiophrenic vein. Pediatr Radiol 34(2):180–181. https://doi.org/10.1007/s00247-003-1078-3CrossRefPubMed

36.

Verniquet A, Kakel R (2012) Cannulation of a persistent left superior vena cava or a pericardiophrenic vein? Can J Anesth 59(2):232–233. https://doi.org/10.1007/s12630-011-9631-2CrossRefPubMed

37.

Berk RN (1964) Dilatation of the left superior intercostal vein in the plain-film diagnosis of chronic superior vena caval obstruction. Radiology 83(3):419–423. https://doi.org/10.1148/83.3.419CrossRefPubMed

38.

Padovan R, Paar M, Aurer I (2011) (Mis)placed central venous catheter in the left superior intercostal vein. Radiol Oncol 45(1). https://doi.org/10.2478/v10019-010-0043-7

39.

Chen SJ, Liu KL, Chen HY et al (2005) Anomalous brachiocephalic vein: CT, embryology, and clinical implications. AJR Am J Roentgenol 184(4):1235–1240. https://doi.org/10.2214/ajr.184.4.01841235CrossRefPubMed

40.

Tomasian A, Malik S, Shamsa K, Krishnam MS (2009) Congenital heart diseases: post-operative appearance on multidetector CT-a pictorial essay. Eur Radiol 19(12):2941–2949. https://doi.org/10.1007/s00330-009-1474-7CrossRefPubMedPubMedCentral

41.

Nguyen TT, Le NT, Doan QH (2018) Superior cavopulmonary anastomosis in patients with bilateral superior caval veins: use of a rolled pericardial graft to create a single caval vein. World J Pediatr Congenital Heart Surg 9(4):446–450. https://doi.org/10.1177/2150135118765888CrossRef

42.

Kiran U, Aggarwal S, Choudhary A, Uma B, Kapoor PM (2017) The blalock and taussig shunt revisited. Ann Card Anaesth 20(3):323–330. https://doi.org/10.4103/aca.ACA_80_17CrossRefPubMedPubMedCentral

43.

Lluri G, Levi DS, Aboulhosn J (2015) Systemic to pulmonary venous collaterals in adults with single ventricle physiology after cavopulmonary palliation. Int J Cardiol 189:159–163. https://doi.org/10.1016/j.ijcard.2015.04.065CrossRefPubMed

Titel: Persistent left superior vena cava: clinical importance and differential diagnoses
verfasst von: Aynur Azizova
Omer Onder
Sevtap Arslan
Selin Ardali
Tuncay Hazirolan
Publikationsdatum: 01.12.2020
Verlag: Springer International Publishing
Erschienen in: Insights into Imaging / Ausgabe 1/2020
Elektronische ISSN: 1869-4101
DOI: https://doi.org/10.1186/s13244-020-00906-2

Update Radiologie

Bestellen Sie unseren Fach-Newsletter und bleiben Sie gut informiert.

Newsletter bestellen

Springer Medizin

Persistent left superior vena cava: clinical importance and differential diagnoses

Abstract

Publisher’s Note

Key points

Background

Epidemiology

Embryology

Drainage site and its impact on the anatomy

Presence of RSVC and bridging vein

PLSVC and accompanying cardiac anomalies

PLSVC and heterotaxy

Clinical importance