Persistent left superior vena cava

Presentation

In persistent left superior vena cava, the left brachiocephalic vein does not develop fully and the left upper limb and head and neck drain into the right atrium via the coronary sinus.

In isolation, the variation is considered benign, but is very frequently associated with cardiac abnormalities (e.g. ventricular septal defect, atrioventricular septal defect) that have a significant mortality and morbidity. It is more frequent in patients with congenital heart defects.

The (right) superior vena cava is almost always unaffected by the presence of persistent left superior vena cava.

Diagnosis

If an anomaly is detected during a routine ultrasound, a fetal echocardiogram is performed to determine whether a fetus has the condition. Otherwise, it is often unnoticed unless an extenuating circumstance warrants further examination of the heart, usually much later in life.

CT and MRI scans in a parasagittal section may show a "pipe" sign where the left superior vena cava occurs.

Treatment

If no other cardiac abnormalities are present, persistent left superior vena cava will not be treated, as it is usually asymptomatic and unharmful. If it drains into the left atrium, then deoxygenated blood enters the circulation to the body, and cyanosis may occur.

References

References

1. (May 2003). "Persistent left superior vena cava: an intensivist's experience and review of the literature". South. Med. J..
2. (September 2004). "Persistent left superior vena cava draining into the coronary sinus: report of 10 cases and literature review". Clin Cardiol.
3. (1984). "Angiography of Congenital Heart Disease". Macmillan Publishing.
4. (2018-01-01). "92 - Anomalies of Systemic Venous Return". Elsevier.
5. (2018-01-01). "2 - Surgical Anatomy for the Implanting Physician". Elsevier.
6. (March 2006). "Prenatal diagnosis of persistent left superior vena cava and its associated congenital anomalies". Ultrasound Obstet Gynecol.
7. (January 1980). "Persistent left superior vena cava. Incidence, associated congenital heart defects and frontal plane P-wave axis in a paediatric population with congenital heart disease". Acta Paediatr Scand.
8. Marelli, Ariane J.. (2012-01-01). "69 - Congenital Heart Disease in Adults". W.B. Saunders.