Paper - Complete situs inversus of the vena cava superior (1930)
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Complete Situs Inversus of the Vena Cava Superior
Béla Halpert and Francis D. Coman
(From the Department of Anatomy, Jokns Hopkins Unisersity, Baltimore, Ma., end the Department of Pathology, University of Chicago, Chicago, IU.)
Received for publication November 18, 1929.
During the routine anatomical investigation of the body of a 2 weeks-old, well nourished, male, negro infant, a vascular anomaly was found which may be termed a “complete situs inversus of the vena cava superior,” the arrangement and course of the dural sinuses, the large venous trunks of the neck, the vena cava superior and the vena azygos being a mirror image of the normal. The drawings by Mr. J. F. Didusch illustrate the condition.
At the confluens sinuum the sinus sagittalis superior appeared to continue into the left sinus transversus (Fig. 1), while the sinus rectus continued into the right sinus transversus. No difference was noted in the size of the internal jugular veins. On the right as well as on the left side, the vena jugularis interna joined the vena subClavia in the usual manner. The right vena anonyma, however, had the usual length of a left vena anonyma, measuring about 25 mm. in this case and appearing rather a continuation of the vena subclavia dextra than that of the vena jugularis interna dextra. The left vena anonyma had the usual length of a right vena anonyma, measuring about 9 mm., and appearing rather a continuation of the vena jugularis interna sinistra than of the vena subciavia sinistra (Fig. 2).
The junction of the right and left venae anonymae occurred in front of the arch of the aorta at about the level of the left sternoclavicular articulation. The vena cava superior, thus formed, joined the sinus coronarius after passing between the pulmonary artery and the left auricle on its right and the upper lobe of the left lung to the left. The size of the vena cava superior sinistra was about the same as that of a normal right vena cava superior. It measured from the anonyma angle to the point of entrance into the sinus coronarius about 30 mm.; the length of the greatly enlarged coronary sinus was about 25 mm. (Fig. 3). Before entering the pericardial cavity, a large venous trunk emptied into the vena cava superior sinistra exactly as would the vena azygos into the normal superior vena cava (Figs. 2 to 4). No trace or vestige of a right vena cava superior was found nor was there a vena azygos on this side (Fig. 4). No other deviation of significance was found in the remaining tributaries of the superior vena cava. The course of the inferior vena cava and its tributaries was normal.
The presence of a vena cava superior on the left side as well as on the right is the normal condition in certain animals. Such an arrangement of the great venous trunks in man is rather infrequent. If present, the anomaly may be looked upon as persistence of a condition existing only during the latter part of embryonic, and the beginning of fetal life}? Of this anomaly well over a hundred cases have been reported up to 1916* and undoubtedly many more observed.* The presence of a left vena cava superior without a right vena cava superior is a far more spectacular condition and one less likely to be overlooked. Yet in the available literature to date there appear to be records of only about seventeen cases.
According to Krause,® Cheselden * reported the first case. The complete report, however, printed in the Philosophical Transactions of the Royal Society of London in 1713 reads as follows: ‘‘A heart, with the vena azygos inserted into the right auricle; and the descending cava coming round the basis of the heart, above the aorta and pulmonary vessels, to enter the auricle at the lower part with the ascending cava.’’ The brevity of this description leaves some doubt as to the existence of an actual situs inversus of the superior vena cava with complete absence of the right vena cava superior.
The first detailed description of a case in which the superior vena cava showed a complete situs inversus was given by Halbertsma’ in 1862. His observations were made on an injected heart, apparently of an adult. The two innominate veins united in front of the arch of the aorta to form the superior vena cava which ran from above downward and to the left, thence continuing in front of the pulmonary artery, and between the pulmonary veins and left auricle it reached the coronary sulcus and passing to the right emptied into the right atrium below and to the left of the inferior vena cava. The vena azygos emptied into the left vena cava superior 4 cm. below the union of the innominate veins.
In 1876 Greenfield * reported to the Pathological Society of London a case of a male, aged 39 years, in which due to an abnormal mode of development of the great venous trunks the left duct of Cuvier instead of the right, persisted as the main channel for the venous blood from the head and upper limbs to the right atrium. In this case apparently the left trunk alone persisted; ‘‘the right, if present, which is doubtful, being represented by only a very small vein: the usual condition being thus reversed, and the whole of the blood from the upper extremities and head entering the coronary sinus. . . . The heart was unfortunately removed before the relation of the great vessels was traced.”
In 1880 Gruber ® described the transposition of the normal relationship of the innominate veins in an adult male, +. ¢., the vena anonyma dextra was the longer of the two and ran obliquely downward to the left. The innominate veins united to form a left vena cava superior. There was no trace of a right vena cava superior. A vena intercostalis suprema, a vena hemiazygos superior, and a vena hemiazygos inferior were found on the right side; a vena intercostalis suprema and a vena azygos were noted on the left side.
The persistence of a vena cava superior sinistra and the absence of the normal right vena cava superior in a man 26 years old, described by Weigert '* in 1881, was attributed to an early synostosis of the sutura mastoidea, aplasia of the right transverse sinus and of the right internal jugular vein. The superior sagittal sinus continued into the left transverse sinus. The jugular foramen on the right was only one-third the size of that on the left.
The next undoubted case of complete situs inversus of the vena cava superior was presented in 1892 by Bédart " before the Society of Anthropology of Paris. No remnant of a right vena cava superior was present. The author gives only a meager description and apologizes for presenting the case which according to him represented a condition frequently reported previously. No references, however, are given.
In 1893 Boyd” demonstrated before the Anatomical Society of Great Britain and Ireland a complete situs inversus of the superior vena cava in a fetus. No trace of a right vena cava superior or duct of Cuvier could be found. There were two azygos veins of equal size: the right emptied into the right innominate vein, the left into the left vena cava superior. 194 HALPERT AND COMAN
In 1899 Miausert * described a complete situs inversus of the vena cava superior in a 4 year-old girl, dead from diphtheria. The right vena cava superior was entirely absent and the left vena cava superior emptied into the greatly enlarged coronary sinus which in turn opened into the right atrium, a little more to the left than is usual. The heart was otherwise normal. There were two azygos veins: a larger left emptied into the vena cava superior just before it entered the pericardial sac, a narrow right emptied into the right innominate vein.
In 1910 Gozuloff described a complete situs inversus of the vena cava superior in a 30 year-old male typhus victim. The anomaly was noted six years previously by V. P. Oven in the course of routine dissection in the Department of Anatomy, University of Kiev. No trace of a right vena cava superior was present. The vena azygos emptied into the left superior vena cava 5.5 cm. below the junction of the innominate veins. The vena hemiazygos emptied into the vena azygos.
Dietrich 15 in 1913 described a complete situs inversus of the superior vena cava in a 29 year-old woman who died of fibroxanthosarcomatosis. There was no vena azygos on the right side. The left vena azygos entered the superior vena cava just before it pierced the pericardium.
Schiitz '* in 1913 reported a clear-cut complete situs inversus of the vena cava superior in a male, aged 61 years. “Vena cava superior in latere sinistro descendens (ductus Cuvieri sinister persistens). Vena azygos sinistra, vena hemiazygos dextra.” This case had been recorded previously by Genersich in the Hospital Annals of the City of Budapest in 1910. Schiitz mentions an exactly similar case noted in the First Pathological Institute of the University of Budapest.
Niitzel "” in 1914 described a case, in a 25 year-old male, in which the only vena cava superior present was the left. This opened into the coronary sinus at the atrioventricular border line. The coronary sinus was large and aJthough emptying into the left atrium communicated also with the right. At the junction of the superior vena cava and the coronary sinus emptied the vena cordis magna ‘“‘linke Kranzvene.”’ The vena azygos opened into the vena cava superior sinistra.
Smith !* in 1916 described a complete situs inversus of the vena cava superior in a middle-aged male. There was no trace of a right vena cava superior. The vena cava superior sinistra emptied into the coronary sinus which joined the inferior vena cava at its entrance into the right atrium.
Monckeberg '* (1924) found in the collection of the Pathological Institute of Tiibingen, the heart of an adult which apart from the persistence of the left vena cava superior and absence of the right, presented no other developmental or pathological changes.
Benda” (1924) mentions a complete situs inversus of the vena cava superior in an adult male, and illustrates the case with diagrams.
Villa * (1926) working apparently on an adult body noticed the presence of a left vena cava superior which continued into the sinus coronarius. He found no trace of a right vena cava superior; the vena azygos emptied into the left vena cava superior.
In terms of embryology the vena cava superior consists: (a) of a proximal portion which is identical with the duct of Cuvier, and (6) of a distal portion formed by the terminal part of the vena cardinalis superior. The cardinal portion of the superior vena cava begins at the junction of the innominate veins, its transition into the Cuvierian portion is marked, of course, by the entrance of the vena azygos which represents the cranial half of the vena cardinalis inferior. In our case the right superior vena cava either has not developed or else has disappeared entirely, while the left remained taking functionally the place of the right. Whether the entrance of the sinus sagittalis superior into the left sinus transversus had anything to do with this transposition or whether it is only a part of it is open to discussion.” It may be recalled that the course of the sinus sagittalis superior is mentioned only by Weigert.’* In his case, as in ours, the sinus sagittalis superior continued into the left sinus transversus. Weigert attributed the presence of the left vena cava superior to an early synostosis of the sutura mastoidea, aplasia of the right sinus transversus and of the right vena jugularis interna.
A rare vascular anomaly in a 2 weeks-old negro infant* is described. The arrangement and the course of the dural sinuses, the large venous trunks of the neck, the vena cava superior and the vena azygos presented a mirror image of the normal. There was no trace of a right vena cava superior.
Previous reports of this anomaly are reviewed.
1. Marshall, John. On the development of the great anterior veins in man and mammalia, etc. Philos. Tr., 1850, 140, 133. :
2. Felix, Walther. Georg Ruge’s Priparieriibungen. Wilhelm Engelmann, Leipzig, 1921.
3. McCotter,R.E. Three cases of the persistence of the left superior vena cava. Anat. Record, 1916, 10, 371.
4. Locchi, R. Veia cava superior esquerda no adulto. An. da. Fac. de med. Sdo Paulo, 1927, 2, 103.
s. Krause, W. Varietiten der Kérpervenen. Henle’s Handbuch der systematischen Anatomie des Menschen. Braunschweig, 1876, 3.
6. Cheselden, W. Some anatomical observations. Philos. Tr., 1713, 28, 281.
7. Halbertsma, H. J. Abnormitat der Vena cava superior. Nederl. Tijdschr. ov. Geneesk., 1862, 6, 610; Schmidt’s Jahrb. d. ges. Med., 1863, 118, 163.
8. Greenfield, W. S. Persistence of left vena cava superior with absence of right. Tr. Path. Soc. London, 1876, 27, 120.
9. Gruber, W. Vorkommen einer Vena cava superior sinistra (bei Abwesenheit der Vena cava superior der Norm). Dritte der im Verlaufe von 167 Jahren zur Kenntniss gekommenen Falle. Virchows Arch. f. path. Anat., 1880, 81, 458.
10. Weigert, C. Ueber einen Fall von links verlaufender Vena cava superior muthmaasslich bedingt durch frithzeitige Synostose der Sutura mastoidea dextra. Virchows Arch. f. path. Anat., 1881, 84, 184.
11. Bédart. Veine cave supérieure située a gauche. Bull. et mém. Soc. d’anthrop. de Paris, 1892, S. 4, 30, 379 12. Boyd, S. Case of left superior cava without transposition of viscera. Proc. Anat. Soc. Great Britain & Ireland, 1893, p. xx.
13. Mausert, A. Zur Kasuistik der Vena cava superior sinistra und der einen Spitzenlappen der rechten Lunge abschniirenden Anomalie der Vena azygos. Inaug. Diss., Giessen, 1899.
A brief description of this case appeared in the issue of the Anatomical Record * which contains the abstracts of papers presented at the forty-third session of the American Association of Anatomists. SITUS INVERSUS OF THE VENA CAVA SUPERIOR 197
14. Gozuloff, G. I. Left superior vena cava, the right being absent. Universitetskia Isvestia Kitev., 1910, 50, 1 (No. 7).
15. Dietrich, A. Ueber ein Fibroxanthosarkom mit eigenartiger Ausbreitung und iiber eine Vena cava superior sinistra bei dem gleichen Fall. Virchows Arck. f. path. Anat., 1913, 212, 119.
16. Schiitz, J. A vena cava superior fejlodési rendellenességének néhAny esete. Orevosi Hetil., 1913, 57, 845 Schiitz, H. Einige Falle von Entwicklungsanomalie der Vena cava superior. (Persistenz des linken Ductus Cuvieri.) Virchows. Arch. f. patk. Axat., 1914, 216, 35.
17. Niitzel, Heinrich. Beitrag zur Kenntnis der Missbildungen im Bereiche der oberen Hohlvene. Frankfurt. Zischr.f. Path. 1914, 15, 1.
18 Smith, W. C. A case of a left superior vena cava without a corresponding vessel on the right side. Asat. Record, 1916, 11, 191.
19. Ménckeberg, J. G. Herz, Henke und Lubarsch’s Handbuch d. speziellen pathologischen Anatomie und Histologie. Julius Springer, Berlin, 1924.
20. Benda, C. Venen, Henke und Lubarsch’s Handbuch der speziellen pathologischen Anatomie und Histologie. Julius Springer, Berlin, 1924.
a1. de la Villa, D. 4 Un caso de vena cava superior izquierda. Siglo méd., 1926, 78, 429
22. Streeter, G. L. The developmental alterations in the vascular system of the brain of the human embryo. Conérib. Embryol., 1918, 8, 7.
23. Halpert, Béla. Complete situs inversus of the vena cava superior. Axat. Record, 1927, 35, 38DESCRIPTION OF PLATES
Fic. 1. View of the confluens sinuum. The sinus sagittalis superior enters the left sinus transversus.
Fic. 2. Arrangement of the large vessels of the neck and thorax. The arrangement and course of the large venous trunks of the neck, the vena cava superior and the vena azygos present a mirror image of the normal. The right vena anonyma has the usual length of a left vena anonyma, while the left vena anonyma has the usual length of a right vena anonyma. By the junction of the right and left venae anonymae a left superior vena cava is formed which passing in front of the aorta and arteria pulmonalis joins the sinus coronarius. The vena azygos enters the vena cava superior sinistra as would the normal azygos vein the normal superior vena cava. AMERICAN JOURNAL OF PatHoLocGy. Vot. VI PLATE 41 Sinus sagittalis superior Sinus transversus sinister
Sinus transversus dexter I
V. jugularis interna sinistra V.anonyma dextra V.anonyma sinistra
V jugularis in terna dextra A.carotis com. dextra
\/ ¥. cava Superia f erior sirustra
V. jegularis externa
V. subclavia dextra
Aorta ascendens A. pulmonalis
2 Halpert and Coman Situs Inversus of the Vena Cava Superior PLATE 42
Fic. 3. Entrance of the vena cava superior sinistra into the sinus coronarius.
Fic. 4. View of the right atrium. The asterisk marks the site where the vena cava superior normally enters the right atrium.
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