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Anatomy Atlases: Illustrated Encyclopedia of Human Anatomic Variation: Opus II: Cardiovascular System: Veins: Head, Neck, and Thorax: Pulmonary Veins

Illustrated Encyclopedia of Human Anatomic Variation: Opus II: Cardiovascular System: Veins: Head, Neck, and Thorax

Pulmonary Veins

Ronald A. Bergman, PhD
Adel K. Afifi, MD, MS
Ryosuke Miyauchi, MD

Peer Review Status: Internally Peer Reviewed

Anomalous connections between the pulmonary and systemic venous system were first recognized by Winslow in 1737. He collected 106 cases (Winslow, J. Mem Acad. Roy. de Sci., p113, 1739).

Parsons, et al., reported 35 cases of anomalous pulmonary drainage without cardiac anomalies. In five cases, the pulmonary vein was connected to the superior vena cava, in five to the right auricle, in five to the left innominate (brachiocephalic) vein, in nine to the coronary sinus, in one to the inferior vena cava, in three to the portal vein, in five to a persistent left superior vena cava, and two to the ductus venosus. The pulmonary veins may also open into the azygos vein.

Pulmonary veins vary in number and size. There are reports of one or more pulmonary veins entering either the right atrium or the inferior or superior vena cava or azygos vein. In one case, a left pulmonary vein opened into the left brachiocephalic vein.

These unusual connections between the pulmonary veins and neighboring, many adversely inappropriate, veins are explicable only on the basis of very early embryonic stages in which the developing foregut, trachea, and lung buds are supplied by a common plexus of small channels that network in all directions through the loose mesenchyme and communicate with the primitive cardinal veins in many places. With the development of certain channels in this primitive vascular bed to form the pulmonary veins leading to the left atrium, the primitive connections with the cardinal veins usually disappear. The unusual development of one or more of them, and its retention by the adult derivative of the particular part of the cardinal system involved, are most probably responsible for the occurrence of these unusual pulmonary connections.

Steinberg and Finby classified the congenital anomalies of the pulmonary circulation as follows

A. Malformations of the Pulmonary Artery.
Primary dilitation (aneurysm)

Requires no treatment.

Valvular stenosis

Requires surgical treatment.

B. Malformation of a Branch of the Pulmonary Artery.
Absence of a main branch pulmonary artery

No treatment required, prognosis good.

Absence of a lung (agenesis)

Compatible with normal life.

C. Pulmonary Arteriovenous fistulas

Requires surgical treatment.

D. Alteration of Pulmonary Circulation.
Patent ductus arteriosus

Reversal of blood flow

Requires surgical treatment.

Anomalous insertions of pulmonary veins.

May be asymptomatic (with one lung involved). Requires surgical treatment.


Requires surgical treatment.

Anomalous aortic circulation to the lung

May require surgery.

From Steinberg, I. and N. Finby, 1956. See complete reference below.

Keith, et al. reported on 58 cases of anomalous pulmonary drainage as follows;


Drainage Site

% of Cases


Left superior vena cava (or left innominate vein)



Coronary sinus



Right auricle



Right superior vena cava



Portal vein



Ductus venosus



Inferior vena cava



Superior vena cava and right auricle


Anomalous drainage of the pulmonary veins occurs in about 0.7% of individuals (Specht and Brown).


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Dotter, C.T., Hardesty, N.M. and I Steinberg. (1949) Anomalous right pulmonary vein entering the inferior vena cava: Two cases diagnosed during life by angiocardiography and cardiac catheterization. Am. J. of the Medical Sciences. 218:31-36.

Douglass, R. (1948) Anomalous pulmonary vessels. J. Thoracic Surg. 17:712-716.

Edwards, J.E. (1953) Pathologic and developmental considerations in anomalous pulmonary venous connection. Staff Meetings of the Mayo Clinic 28(17):441-452.

Edwards, J.E. and J.W. DuShane (1950) Thoracic venous anomalies. Arch. Path. 49:517-537.

Edwards, J.E., DuShane, J.W., Alcott, D.L. and H.B. Burchell. (1951) Thoracic venous anomalies. Arch. Path. 51:446-460.

Findlay, Jr.,C.W. and H.C. Maier (1966) Anomalies of the pulmonary vessels and their surgical significance. Surgery 29:604-641.

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Grishman, A., Poppel, M.H., Simpson, R.S. and M.L. Sussman. (1949) The roentgenographic and angiographic aspects of (1) aberrant insertion of pulmonary veins associated with interatrial defect and (2) congenital arteriovenous aneurysm of the lung. Am. J. Roentgenol. 62:500-508.

Grishman, A., Brahms, S.A., Gordon, A. and F.H. King. (1951) Aberrant insertion of the pulmonic veins. J. Mount Sinai Hospital 17:336-343.

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. (1885) Einmündung der Vena pulmonalis dextra superior in die Vena cava superior. Arch. Pathol. Anat. Physiol. Klin. Med. 102:3-5.

Harris, H.A. and I. Lewis. (1940) Anomalies of the lungs, with special reference to the dangers of abnormal vessels in lobectomy. J. Thoracic Surg. 9:667-671.

Healey, Jr., J.E. (1952) An anatomic survey of anomalous pulmonary veins: Their clinical significance. J. Thoracic Surg. 23:433-444.

Hickman, -. (1869) Malformation of the heart.; 1) Transposition of auricles and of the aorta; absence of pulmonary artery; patent foramen ovale; communicating ventricles; with lateral transposition of principal viscera; cyanosis. 2) Transposition of viscera. Malformation of heart; pulmonary veins from right lung entering left auricle and from left lung entering right auricle. Trans. Pathol. Soc. Lond. 20:88-92, 92-98.

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Ingalls, N.W. (1932) Anomalous pulmonary vessels. Anat. Rec. 53:269-281.

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.

Keith, J.D., Rowe, R.D., Vlad, P. and J.H. O'Hanley. (1954) Complete anomalous pulmonary venous drainage. Am. J. Medicine 16:23-38.

Kolesnikow, N. (1932) Ein seltener Fall der Mündung der Vena pulmonalis in die Vena cava superior. Anat. Anz. 74:233-237.

Lachi, P. (1879) Di alcune varietà anatomiche. L'Imparziale 19 (N. 14):365-372.

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McManus, J.F.A. (1941) A case in which both pulmonary veins emptied into a persistent left superior vena cava. The Canadian Medical Association Journal 46:261-264.

Nabarro, D. (1903) Two hearts showing peculiarities of the great veins. J. Anat. Physiol. 37:382-391.

Neil, C.A. (1956)Development of the pulmonary veins with reference to the embryology of anomalies of pulmonary venous return. Pediatrics 18:880-887.

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Specht, H.D. and A.F. Brown. (1953) Drainage of pulmonary veins into ductus venosus with other anomalies. A.M.A. Arch. of Internal Medicine 92:148-151.

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