Illustrated Encyclopedia of Human Anatomic Variation: Opus II: Cardiovascular System: Veins: Head, Neck, and Thorax
Ronald A. Bergman, PhD
Adel K. Afifi, MD, MS
Ryosuke Miyauchi, MD
Peer Review Status: Internally Peer Reviewed
Variations Due to Persistence of Left Common Cardinal
Vein.
The left subclavian may join the left internal jugular vein to form a
trunk that continues almost vertically downward over the arch of the
aorta in front of the root of the left lung and opens into the
coronary sinus of the heart. This variation is known as the
persistent left superior vena cava and results from the retention of
the embryonic arrangement. It occurs (all sources) in about 0.4% of
cases.
A persistent left superior vena cava may be associated with the presence of two azygos veins.
When a left superior vena cava is present, the accessory hemiazygos vein (the remnant of the anterior part of the left cardinal vein) may open into it by arching over the root of the left lung, in a way similar to that in which the azygos (the right cardinal vein) opens into the superior vena cava over the root of the right lung.
The usual left brachiocephalic vein may be absent, or quite small or even rudimentary.
The left innominate vein may receive anomalous pulmonary veins; the longest recorded living history of an individual with this "incompatible with life" variation is forty-two years.
The left superior vena cava may open into the left auricle (atrium) of the heart, coronary sinus, innominate vein, or right superior vena cava.
On occasion, a left superior vena cava communicates with a typical inferor vena cava by means of a channel that is a remnant of a persistent sinus venosus.
In another variation, a vein runs from the left brachiocephalic or from the left superior intercostal vein through the "vestigial fold" (of Marshall) of the pericardium to the vein of Marshall to the coronary sinus. When the coronary sinus is absent the blood flow is reversed.
Variations Due to Persistence of left and Suppression of Right
Common Cardinal Vein.
The right brachiocephalic vein may cross the arch of the aorta to
join a verticle left brachiocephalic vein, thus forming a left
superior vena cava. The right superior vena cava is absent. The
arrangement of the azygos veins under these conditions may be
reversed: a left azygos opens (over the root of the left lung) into
the left superior vena cava, and the now right hemiazygos and
accessory veins are organized in a manner similar to the typical
arrangement of the left hemiazygos veins. This arrangement of veins
may occur independently of any general transposition of the viscera.
There are other variations in the development of the great veins from
the common cardinals, primitive jugular, and anterior and posterior
cardinal veins.
The right superior vena cava is usually connected to the right atrium, but may be joined to the coronary sinus by a vein of substantial length and size.
Doubled superior vena cava has an incidence of 0.16% (a study of 3000 subjects).
Unusual tributaries of the superior vena cava include: the internal thoracic on the right side, the right supreme intercostal, and the thyroidea ima vein.
Ancel and Villemin (1908) (see Poynter, 1922) have classified the anomalies of the superior vena cava as follows:
a) Double superior vena cava without anastomoses. Persistence of the left duct of Cuvier and failure of development of the left innominate vein.b) Double superior vena cava with small anastomoses, persistence of the left duct of Cuvier, and a small innominate development.
c) Double superior vena cava with normal innominate vein.
d) Persistence of the left superior vena cava without atrophy of the right superior vena cava.
The embryological history of the vena cava is well-documented and found in most textbooks of embryology. Variations are associated with the embryologic pattern and consist of persistence of embryological connections alone or in conjunction with atrophy of normally developing channels (Poynter, 1922).
When an accessory vertebral vein is present, it arises from the venous plexus on the vertebral artery and descends through the seven transverse foramina to join the brachiocephalic vein.
Image 119, Image 170, Image 171, Image 178, Image 192, Image 193
Persistent Left Superior Vena Cava
See Image 119, Image 125, Image 170, Image 171, See Image 178, See Image 192, See Image 193,Image 200, Image 202, Image 203, Image 367, Image 398, Image 404, Image 503
Doubled
Image 172, Image 224, Image 375, Image 404
Left and Right Cardinal (Persistent)
Oblique (of Marshall)
References
Adachi, B. (1928) Anatomie der Japaner. Verlag der Kaiserlich-Japanischen Universität zu Kyoto, Maruzen Co., Kyoto.
Ancel, P. et F. Villemin. (1908) Sur la persistance de la veine cave supérieure gauche chez l'homme. J. de la Anat. et de la Physiol. Normales et Pathologiques 44:46-62.
Andreassi, G. (1941-42) über die Verbindung zwischen dem oberen und unteren Hohlvenensystem in Himblick auf die laterale und medial Wurzel der Vena azygos und der Vena hemiazygos. Anat. Anz. 92:240-245.
Atwell, W.J. and P. Zoltowski (1938) A case of a left superior vena cava without a corresponding vessel on the right side. Anat. Rec. 70:525- 532.
Baroni, B. (1925) Un caso di persistenza dell vena cava superiore sinistra in un cuore di uomo adulto. Monitore Zool. Ital. 36:66-68.
Barth, -. (1853) Oblitération de la veine cave supérieur. Bulletins et Mem. de la Société Anatomique de Paris XXVIII(1):2-6.
Bassiano, T. (1881) Otto vene polmonari nell'orecchietta sinistra del cuore. Annali Universali di Medicina e Chirurgia 6:403-408.
Basu, B.N. (1932) Persistent "left superior vena cava," "left duct of Cuvier" and left horn of the sinus venosus. J. Anat. 66:628-270.
Beattie, J. (1931) The importance of anomalies of the superior vena cava. in man. Canad. Med. Assoc. J. 25:281-284.
Bedart, -. (1892) Veine cave supérieure située à gauche. Bull. de la Soc. d'Anthrop. de Paris 1892:379.
Bergman, R. A., Thompson, S.A., Afifi, A.K. and F.A. Saadeh. (1988) Compendium of Human Anatomic Variation: Catalog, Atlas and World Literature. Urban & Schwarzenberg, Baltimore and Munich.
Beyerlein, K. (1914) Die persistierende Vena cava superior sinistra als Abflussrohr für das Coronarvenenblut. Frankfurter Zeitschrift für Pathologie 15:327-332.
Cameron, J. (1911) Persistence of the left posterior cardinal vein. J. Anat. Physiol. 45:416-419.
Cameron. J. (1915) A specimen showing complete remains of the left superior vena cava, with a description of a venule, which represents the "lost" extrapericardiac portion of the left duct of Cuvier. J. Anat. Physiol. 49:140-147.
Charles, J.J. (1889). Notes of a case of persistent left superior vena cava, the right superior vena cava being in great part a fibrous cord. J. Anat. Physiol. 23:649-650.
Cheselden, W. (1713) Some anatomical observations. Philosophical Transactions, London 28:282.
Chouke, K.S. (1939) A case of bilateral superior vena cava in an adult. Anat. Rec. 74:151-157.
Cooper, C.M. (1900) A pericardial sac in which the large azygos vein pierced the sac before opening into the superior vena cava. J. Anat. Physiol. 34:426.
Cords, E. (1921) Ein Fall von teilweiser Erhaltung der V. cava sup. sin. zusammen mit einer Anomalie der Vv. pulmonalis. Anat. Anz. 54:491-495.
Donadio, N. (1924-25) Ein Fall von Verdoppelung der Vena cava superior. Anat. Anz. 59:321-327.
FitzGerald, D.P. (1909) The study of developmental abnormalities as an aid to that of human embryology, based on observations on a persistent left superior vena cava. Dublin J. Med. Sci. 1909:14-18.
Fraser, R.S., Dvorkin, J., Rossall, R.E. and R. Eidem. (1961) Left superior vena cava. A review of associated congenital heart lesions, catherization data and roentgenologic findings. Am. J. Med. 31:711-716.
Gozuloff, G.I. (1910) (Left superior vena cava, the right being absent.) (In Russian) Universitatskiya Izviestiya, Kiyev 50:1-29.
Green field, W.S. (1876) Persistence of the left vena cava superior, with absence of right. Trans. Pathol. Soc. Lond. 27:120-124.
Gruber, W. (1864) Duplicität der Vena cava superior. Memoires de la Academie Imperiale des Sciences de St. Petersbourg, Series 7, 1864:45-82.
Gruber, W. (1865) Rudimentäre Vena cava superior sinistra bei einem Erwachsenen. Arch. Pathol. Anat. Klin Med. 32:114-117.
Gruber, W. (1876) Ein Fall von Einmundung der Vena pulmonalis dextra superior in die Vena cava superior. Arch. Pathol. Anat. Physiol. Klin. Med. 68:284.
Gruber, W. (1880) Vorkommen einer Vena Cava superior sinistra (bei Abwesenheit der V. cava superior der norm). Arch. Pathol. Anat. Physiol. Klin Med. 81:458-462.
Gruber, W. (1880) Duplicität der Vena cava superior, mit Vorkommen zweir Nenae azygae und einer sufficienten Valvula an der Mündung der Vena azygos sinistra. Arch. Pathol. Anat. Physiol. Klin. Med. 81:462-465.
Gruber, W. (1881) Duplicität der Vena cava superior mit Vorkommen zweier transversaler Communicationsäste und zweier Venae azygae. Arch. Pathol. Anat. Physiol. Klin. Med. 86:38-41.
Gruber, W. (1885) Duplicität der Vena cava superior (11. Fall eigener Beobachtung) bei Verschluss der Mindüng der Vena cava superior sinistra in das Atrium dextrum und deren Auftreten als Abfuhrungskanal der Herzvenen in die Vena anonyma sinistra (sicherer 1. Fall). Arch. Pathol. Anat. Physiol. Klin. Med. 99:492-497.
Gruber, W. (1885) Einmundung der Vena pulmonalis dextra superior in die Vena cava superior. Arch. Pathol. Anat. Physiol. Klin. Med. 102:3-5.
Halbertsma, H.J. (1862) Ontleedkundige Aanteekeningen. Nederlandsch Tijdschrift voor Geneeskunde 6:609-612.
Halpert, B. (1927) Complete situs inversus of the vena cava superior. Anat. Rec. 35:38.
Halpert, B. and F. Coman. (1930) Complete situs inversus of the vena cava superior. Am. J. Pathol. 6:191-197.
Howden, R. (1887) Case of double superior vena cava with left -sided arrangement of the azygos vein. J. Anat. Physiol. 21:72-75.
Huffmire, A.P. and G.C. Bower. (1919-20) A case of persistence of the left superior vena cava in an aged adult. Anat. Rec. 17:127-129.
Hurley, L.E. and A.E. Coates (1927) A case of right-sided aortic arch and persistent left superior vena cava. J. Anat. 61:333-339.
Huu, N. (1956) Veine cave supërieure gauche et pathologic cardiovasculaire moderne. Assoc. Anatomistes, Comptes Rendus 43:619-635.
Johnston, T.B. (1915) A rare vascular anomaly - opening of the upper left pulmonary vein into a persistent left superior vena cava. J. Anat. Physiol. 69:182-186.
Kadyi, H. (1881) Ueber einige Abnormitäten des Gefässsystemes. Medizinische Jahrbücher 1881:37-44.
Keyes, D.C. and H.C. Keyes (1925) A case of persistent left superior vena cava with reversed azygos system. Anat. Rec. 31:23-26.
Kodama, K. (1930) Fortbestand der V. supracardinalis dextra und sinistra bei einem Japaner. Okajima's Folia Anat. Jpn. 9:72-76.
Kodama, K. (1932) Persistenz des Ductus Cuvieri sinister bei einem Japaner. Okajima's Folia Anat. Jpn. 9:365-369
Kolesnikow, N. (1932) Ein seltener Fall der Mindüng der Vena pulmonalis in die Vena cava superior. Anat. Anz. 74:233-237.
Krizan, Z., Herman, O. and V. Dzidrov. (1958) Teilweiser Fortbestand des Supracardinalsystems neben der normalen Vena cava inferior beim Menschen. Acta Anat. 34:312-325.
Kuhlenbeck, H. (1930) Teilweise erhaltene linke Vena supracardinalis, Fensterbildung im Beriche der rechten Vena iliaca communis und akzessoriche Nierengefässe bei einem erwachsenen Menschen. Anat. Anz. &0:117-122.
Little, T.E. (1880) Abnormality of the great cardiac vessels; Absence of the superior vena cava. Dublin J. Med. Sci. 60:443-445.
Lucien, M. (1954) Considérations sur le developpment de la veine cava supérieure chez l'homme. Assoc. Anatomistes, Comptes Rendus. 41:977-981.
Marchessaux, L. (1838) Transposition compléte des organes: Division du tronc brachio-céphalique gauche immédiatement après sa naissance, simulant deux troncs isolés; existence de deux veinescaves supérieures. Archives Générales de Médecine 1:346-348.
Marshall, J. (1850) On the development of the great anterior veins in man and mammalia; including an account of certain remnants of foetal structures found in the adult, comparative view of these great veins in different mammalia, and an analysis of their occasional pecularities in the human subject. Phil. Trans. Roy. Soc. (Lond.) 140:133-170.
McCotter, R.E. (1914-1915) Demonstration of three human hearts showing double superior venae cavae. Michigan University Clinical Society Transactions 6:120-122.
McCotter, R.E. (1915-16) Three cases of the persistence of the left superior vena cava. Anat. Rec. 10:371-383.
McCotter, R.E. (1915) Demonstration of three human hearts showing double superior vena cavae. Michigan State Medical Society, Journal 14:479-481.
Michaelsohn, A. (1920) Einmündung aller Lungenvenen in die persisterende Vena cava superior sinistra und Cor biloculare bei einem 21jährigen Manne. Frankfurter Z. Pathol. 23:222-246.
Mönckeberg, J.G. (1921) Das Verhalten des Sinusknotens bei Fehlen der Vena cava superior dextra und Persistenz der Vena cava superior sinistra. Beitr. Pathol. Anat. Allg. Pathol. 69:537-548.
Nandy, K. and C.B. Blair, Jr. (1965) Double superior vena cavae with completely paired azygos veins. Anat. Rec. 15:1-9.
Odgers, P.N.B. (1928) A case of bilateral superior vena cava in the adult. J. Anat. 62:221-223.
Papez, J.W. (1937-38) Two cases of persistent left superior vena cava in man. Anat. Rec. 70:191-198.
Papilian, V. and R. Rubin. (1923-24) Der Zusammenhang des Sinus venosus mit der Vena cava sinistra. Anat. Anz. 57:276-277.
Peele, T.L. (1932) A case of closed coronary sinus and left superior vena cava. Anat. Rec. 54:83-86.
Petersson, O. (1868) Menniskohjerta med vena cava superior sinistra. Upsala Lakareforening Forhandlingar 3:697-705.
Poynter, C.W.M. (1922) Congenital anomalies of the arteries and veins of the human body with bibliography. The University Studies of the University of Nebraska, Lincoln 22:1-106.
Prows, M.S. (1942) Two cases of bilateral superior vena cavae, one draining a closed coronary sinus. Anat. Rec. 87:99-106.
Reed, A.F. (1938-39) A left superior vena cava draining the blood from a closed coronary sinus. J. Anat. 73:195-197.
Rex, H. (1882) über einen Fall von Duplicität der Vena cava superior. Prager Medicinische Wochenschrift 7 (Nr. 35):341-342.
Ring, -. (1805) A singular case of malformation of the heart. London Medical and Physical Journal 13:120-121.
Rozel, A. (1966) über die Anomalie der Arteria subclavia dextra und die Persistenz der Vena cava superior sinsistra. Anat. Anz. 118:348-367.
Sahinoglu, K. Cassell, M.D., Miyauchi, R. and R.A. Bergman. (1994) Human persistent left superior vena cava with doubled coronary sinus. Annals of Anatomy 176:451-454.
Sanders, J.M. (1946) Bilateral superior vena cavae. Anat. Rec. 94:657-662.
Schütz, H. (1914) Einige Fälle von Entwicklungsanomalie der Vena cava superior (Persistenz des linken Ductus Cuvieri). Arch. Anat. Physiol. Klin. Med. 216:35-45.
Schütz, J. (1913) A vena cava superior (fejödési rendellennességének néhány esete). Orvosi Hetilap 57:845-849.
Shattock, S.G. (1885) Specimen of double vena cava (persistent left duct of Cuvier) the vessel of the left side being larger. Trans Pathol. Soc. Lond. 35:130-131.
Singh, B. and V.K. Ahuja. (1963) Double superior vena cava with double vena azygos. J. Anat. Soc. India 12:33-35.
Smith, J.H. (1919-20) Description of a case of persistent left duct of Cuvier in man. Anat. Rec. 17:131-133.
Smith, W.C. (1916) A case of left superior vena cava without a corresponding vessel on the right side. Anat. Rec. 11:191-198.
Steinberg, I., Dubilier, Jr., W. and D.S. Lukas. (1953) Persistence of the left superior vena cava. Dis. Chest 24:479-488.
Thompson, I.M. (1929) Venae cavae superiores dextra et sinistra of equal size in an adult. J. Anat. 63:496-497.
Tichomiroff, M. (1895) Ein Fall von congenitalem Mangel der linken Lunge mit Persistenz der linken oberen Hohlvene bei einem erwachsenen Menschen. Internat. Monatsschrift für Anat. und Physiol. 12:37-45.
Tichomiroff, M. (1898) Verdoppelung der unteren Hohlvenen bei dem Menschen. Internatl. Monatsschrift für Anatomie und Physiol. 15:143-144.
Toldt, C. (1928) An Atlas of Human Anatomy for Students and Physicians. The Macmillen Co, New York.
Troyer, J.R. (1961) A multiple anomaly of the human heart and great veins. Anat. Rec. 139:509-513.
Vaket, L., Poppelier, G. and P. Vermeire. (1958) Sur un cas d'anomalie combinée de la veine cave supérieure et du système azygos. Acta Anat. 32:235-239.
Valleix, -. (1835) Transposition irrégulière des organs, de droite a gauche; absence de la closion inter-auriculaire du coeur; ventricule pulmonaire rudimentaire, et ne communiquant pas avec les oreilletes; closion inter-ventriculaires incomplète deux veines cave supérieures... Arch. Gén. Méd. 8:78-91.
Van Cleave, C.D. (1931) A multiple anomaly of the great veins and intratrial septum in a human heart. Anat. Rec. 50:45-51.
Versari, R. (1906) Rare anomalie de la valvule d'Eustache chez un homme adulte avec double veine cave supérieure. Arch. Ital. Biol. 46:298.
Villemin, G.., Dufour, R., Rigaud, A. und A. Gouaze. (1952) A propos d'une disposition peu fréquente de persistence de la veine cave supérieure gauche chez l'homme. Assoc. Anatomistes, Comptes Rendus. 39:178-182.
Voyer, -. et -.,Guibert. (1888) Cas rare de malformation congénitale du coeur. Gazette Médicale de Nantes 6:53-54.
Wallraff, J. (1938-39) Ein Fall von linker Vena cava superior ohne entsprechende Vene auf der rechten Seite. Anat. Anz. 87:305-311.
Walsham, W.J. (1880) A left superior vena cava. St. Bartholomews Hospital Reports 16:93-97.
Weigert, C. (1881) über einen Fall von links verlaufender Vena cava superior, mutmasslich bedingt durch frühzeitige Synostosis der Sutura mastoidea dextra. Arch. Pathol. Anat. Physiol. Klin. Med. 84:184-188.
Wennerstrand, J.R. (1963) A case with a vena cava superior on both sides. Anat. Anz. 112:338-343.
Zaaijer, T. (1869) Vena cava superior dextra et sinistra. Afwijking in de samenstelling der vena cava inferior. Nederlandsch Tijdschrift voor Geneeskunde 13:157-160.
Zaaijer, T. (1872) Observations Anatomiques. Veines caves supérieures droite et gauche. Archives Neerlandaises des Sciences Exactes et Naturelles 7:449-459.
Zoltowsky, P. and W. Atwell (1938) A case of a left superior vena cava without a corresponding vessel on the right side. Anat. Rec. 70:525-532.
Section Top | Title Page
Please send us comments by filling out our Comment Form.
All contents copyright © 1995-2024 the Author(s) and Michael P. D'Alessandro, M.D. All rights reserved.
"Anatomy Atlases", the Anatomy Atlases logo, and "A digital library of anatomy information" are all Trademarks of Michael P. D'Alessandro, M.D.
Anatomy Atlases is funded in whole by Michael P. D'Alessandro, M.D. Advertising is not accepted.
Your personal information remains confidential and is not sold, leased, or given to any third party be they reliable or not.
The information contained in Anatomy Atlases is not a substitute for the medical care and advice of your physician. There may be variations in treatment that your physician may recommend based on individual facts and circumstances.
URL: http://www.anatomyatlases.org/