Persistent left superior vena cava

In anatomy, a persistent left superior vena cava is the most common variation of the thoracic venous system.[1][2] It is present in between 0.3% and 0.5% of the population,[3][4][5] and is an embryologic remnant that results from a failure to involute.

Persistent left superior vena cava
The (right) superior vena cava (on left) and the left brachiocephalic vein (here labeled as left innominate vein). The left superior vena cava is not shown in this image.

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.[5]

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.[4][6] It is more frequent in patients with congenital heart defects.[7]

The (right) superior vena cava is almost always unaffected by the presence of persistent left superior vena cava.[4][5]

Diagnosis

If an anomaly is detected during a routine ultrasound, a fetal echocardiogram is performed to determine whether a fetus has the condition.[8] 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.[4]

Treatment

If no other cardiac abnormalities are present, persistent left superior vena cava will not be treated, as it is usually asymptomatic and unharmful.[4] If it drains into the left atrium, then deoxygenated blood enters the circulation to the body, and cyanosis may occur.[8]

References
  1. Pahwa R, Kumar A (May 2003). "Persistent left superior vena cava: an intensivist's experience and review of the literature". South. Med. J. 96 (5): 528–9. doi:10.1097/01.smj.0000060885.27846.91. PMID 12911199. S2CID 37083684.
  2. Gonzalez-Juanatey C, Testa A, Vidan J, et al. (September 2004). "Persistent left superior vena cava draining into the coronary sinus: report of 10 cases and literature review". Clin Cardiol. 27 (9): 515–8. doi:10.1002/clc.4960270909. PMC 6654321. PMID 15471164.
  3. Freedom RM, Culham JAG, Moes CAF (1984). Angiography of Congenital Heart Disease. New York: Macmillan Publishing.
  4. Crispi, Fatima; Martinez, Josep M. (2018-01-01), Copel, Joshua A.; D'Alton, Mary E.; Feltovich, Helen; Gratacós, Eduard (eds.), "92 - Anomalies of Systemic Venous Return", Obstetric Imaging: Fetal Diagnosis and Care (Second Edition), Elsevier, pp. 411–414.e1, doi:10.1016/b978-0-323-44548-1.00092-9, ISBN 978-0-323-44548-1, retrieved 2020-11-16
  5. Fligner, Corinne L.; Clark, John I.; Clark, Judy M.; Larson, Lyle W.; Poole, Jeanne E. (2018-01-01), Poole, Jeanne E.; Larson, Lyle W. (eds.), "2 - Surgical Anatomy for the Implanting Physician", Surgical Implantation of Cardiac Rhythm Devices, Elsevier, pp. 13–58, doi:10.1016/b978-0-323-40126-5.00002-1, ISBN 978-0-323-40126-5, retrieved 2020-11-16
  6. Berg C, Knüppel M, Geipel A, et al. (March 2006). "Prenatal diagnosis of persistent left superior vena cava and its associated congenital anomalies". Ultrasound Obstet Gynecol. 27 (3): 274–80. doi:10.1002/uog.2704. PMID 16456841. S2CID 26364072.
  7. Bjerregaard P, Laursen HB (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. 69 (1): 105–8. doi:10.1111/j.1651-2227.1980.tb07039.x. PMID 7368902. S2CID 22657811.
  8. Marelli, Ariane J. (2012-01-01), Goldman, Lee; Schafer, Andrew I. (eds.), "69 - Congenital Heart Disease in Adults", Goldman's Cecil Medicine (Twenty Fourth Edition), Philadelphia: W.B. Saunders, pp. 397–409, doi:10.1016/b978-1-4377-1604-7.00069-5, ISBN 978-1-4377-1604-7, retrieved 2020-11-16
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