Left superior vena cava

Left superior vena cava (LSVC) is the most common congenital malformation of the thoracic venous return and is present in 0.3 to 0.5% of individuals in the general population with a normal heart, and 4.5% in individuals with congenital heart diseases. A LSVC co-occurs with the right superior vena cava in 80 to 90% of cases, 2 and may also be accompanied by other heart abnormalities, such as anomalous connections of the pulmonary veins, aortic coarctation, tetralogy of Fallot, transposition of the great vessels as well as dextroversion. Moreover, cardiac rhythm disturbances concerning impulse formation and conduction have been observed.

 

Left superior vena cava

The LSVC usually drains into the right atrium (in 80–92%) through a dilated coronary sinus (CS),5,6 but in approximately 10 to 20% of cases, it is associated with left atrial (LA) drainage. The LSVC may drain directly through the left atrium or via the unroofed CS, which is a cause of right-to-left cardiac shunt. The majority of patients with LSVC are asymptomatic. In general, only patients with unusual drainage and right-to-left shunting are of clinical significance. Anomalous venous return via the LSVC may be the cause of cardiac arrhythmias, decreased exercise tolerance, progressive fatigue, chest discomfort, palpitations, syncope or cyanosis.

 

The left superior vena cava drains into the coronary sinus in this heart specimen. CS, coronary sinus; GCV, great cardiac vein; PLSVC, persistent left superior vena cava.

The implications of existing LSVC could be important for clinicians who are involved in placement of central venous-access devices.

📖 Netter’s Cardiology 2th Edition  

 Access to the right side of the heart or pulmonary vasculature through the left subclavian vein is much more difficult in patients with LSVC. Placement of a central line or cardiac resynchronisation therapy leads and pacemaker implantation in undiagnosed cases with LSVC can result in incorrect positioning. In those cases, access to the right heart and coronary sinus should be performed via the right subclavian vein, allowing for an easier route. Also the presence of LSVC is a relative contraindication to the administration of retrograde cardioplegia during cardiac surgery.

 

View of the internal surface of the right atrium. CSO, coronary sinus ostium; EuchV, Eustachian valve; IVC, inferior vena cava.

The right LSVC is usually present as well but may be absent. The two venae cavae may be equal in size, or one (generally the left) may be smaller than its counterpart. The left innominate vein, if present, is smaller than normal or may be more or less plexiform.

The coronary sinus ostium (coronary os) is very large because of the increased blood flow through it, detected easily by cardiac ultrasound. Occasionally, a defect is present in the wall between the sinus and left atrium (unroofed coronary sinus). Generally, such a defect results in a left-to-right shunt; that is, left atrial blood enters the coronary sinus and is carried to the right atrium. Hemodynamically, therefore, the anomaly resembles an atrial septal defect. If the defect is extremely large, particularly if the coronary os is small or atretic, the left SVC is said to “enter the left atrium.”

 Patients with persistent left SVC present a typical clinical picture consisting of moderate central cyanosis without other symptoms. There is no murmur, and the heart is normal in size. The electrocardiogram (ECG) generally shows signs of left ventricular hypertrophy. Similar but more pronounced findings have been described in the rare cases of isolated drainage of the inferior vena cava into the left atrium.

 CT, especially with contrast, is able to elegantly demonstrate the anomalous vessel coursing inferiorly to the left of the arch of the aorta and anterior to the left hilum. It is in direct continuation of the confluence of the left internal jugular vein and the left subclavian vein. Communication between the normal right SVC (which is present in 82-90% of cases) may also be seen.

Depending on the timing of the scan and the side of the injection variable amounts of contrast may be seen within the vessel.

CT is also able, especially with the benefit of reformats, to delineate the site of drainage (usually coronary sinus).

 Transthoracic echocardiography may be enhanced by the use of agitated saline if there is clinical suspicion of a left-sided SVC. Placement of a peripheral intravenous cannula in the left arm, agitation of saline, and subsequent injection should coincide with visualization of the right ventricular outflow tract and coronary sinus in the parasternal long-axis view; the latter (coronary sinus) will fill before the former (right ventricular outflow tract) in the presence of a left-sided SVC.

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