Fact-sheet: Superior vena caval duplication - Left-sided superior veina cava


Updated on 01/23/2026 at 10:16 AM

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Definition

The presence of a persistent left superior vena cava results from failure of obliteration of the left anterior cardinal vein, and corresponds to a left superior vena cava that passes anterior to the left pulmonary hilum and lateral to the aortic arch before joining the systemic circulation via:

  • the coronary sinus (92%)
  • the left atrium (8%)

Two forms:

  • double superior vena cava. >80% of cases, of smaller caliber than the left SVC.
  • isolated left superior vena cava with a persistent left brachiocephalic vein
  • superior intercostal vein communicating with a left SVC and an accessory hemiazygos vein, forming a left azygos arch

Clinical features

Prevalence: 0.3 to 0.5% of the general population, and 5% of congenital heart disease

Most often: incidental finding

Associations:

  • Atrial septal defect
  • Single atrium
  • Ventricular septal defect
  • Tetralogy of Fallot
  • Aortic coarctation
  • Pulmonary stenosis
  • Anomalous pulmonary venous return
  • Cardiac arrhythmias

CT

Reference examination

The left superior vena cava drains the left internal jugular vein and the left subclavian vein, passes lateral to the aortic arch and anterior to the left pulmonary hilum, and drains into the coronary sinus or the left atrium.

Analysis of the course of the right superior vena cava

Search for associated congenital heart disease