This VISUOG chapter focuses on Persistent Left Superior Vena Cava (PLSVC), the most common form of anomalous systemic venous return in adults. PLSVC results from the in-utero failure of the regression of the left anterior and common cardinal veins.

In 92% of cases, PLSVC joins the coronary sinus in the posterior left atrioventricular groove and drains into the right atrium. In the remaining cases, it drains into the left atrium when the coronary sinus is partially or completely unroofed.

PLSVC is commonly associated with cardiac malformations (60% of cases) and extracardiac malformations (40% of cases). Isolated PLSVC is associated with coarctation of the aorta in 21% of cases. It is also linked with conotruncal anomalies and ventricular septal defects.

This chapter details the pathogenesis, etiology, pathology, associated anomalies, and diagnosis of PLSVC, with a specific focus on fetal ultrasound. The detection of PLSVC can be achieved or suspected in different planes of the fetal chest:

  • In the four-chamber view, LSVC can be identified in cross-section at the left border of the left atrium; a dilated coronary sinus may also be noted.
  • In the three-vessel and trachea view, LSVC can be identified in cross-section as a fourth vessel located to the left of the pulmonary artery and left parasagittal plane of the thorax and neck.

The chapter further elaborates on the implications for sonographic diagnosis and screening. Antenatally detected PLSVC should prompt a detailed examination of the fetal anatomy to rule out associated malformations, especially CDH. It should be arranged to rule out other anomalies such as coarctation of the aorta and fetal karyotype, and discussion with parents CMA should be considered.

For more detailed information and to see high-quality ultrasound images with annotations, read the VISUOG chapter on Persistent Left Superior Vena Cava (PLSVC) for free throughout July.

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