Book - Congenital Cardiac Disease 8

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Abbott ME. Congenital Cardiac Disease (1915) Osler & Mccrae's Modern Medicine 6, 2nd Edition.

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Pages where the terms "Historic Textbook" and "Historic Embryology" appear on this site, and sections within pages where this disclaimer appears, indicate that the content and scientific understanding are specific to the time of publication. This means that while some scientific descriptions are still accurate, the terminology and interpretation of the developmental mechanisms reflect the understanding at the time of original publication and those of the preceding periods, these terms and interpretations may not reflect our current scientific understanding.     (More? Embryology History | Historic Embryology Papers)
1915 Congenital Cardiac: Congenital Cardiac Disease | Heart Development | Literature | Etiology | Cyanosis | Classification | Pericardium | Heart Displacement | Whole Heart | Anomalous Septa | Interauricular Septum | Interventricular Septum | Absence of Cardiac Septa | Aortic Septum | Pulmonary Stenosis and Atresia | Pulmonary Artery Dilatation | Aortic Stenosis or Atresia | Primary Patency and Ductus Arteriosus | Aorta Coarctation | Aorta Hypoplasia | Diagnosis Prognosis and Treatment | Figures | Embryology History | Historic Disclaimer

Complete Absence or Rudimentary Development of the Cardiac Septa

A rudimentary development of the cardiac septa, leading to a diminution in the number of the heart's cavities, should not be treated entirely apart from localized septal defects, being simply a more extreme degree of the same lesion. Yet the cases may be conveniently grouped together as indicating arrest at a very early stage of embryonic life (fourth week), frequently associated with anomalies elsewhere, and as forming an altogether different and more serious picture.

Cor Biloculare

Early cases were recorded by Wilson in 1798, Farre in 1814, Ramsbotham in 1846, and Forster in 1847, and there are six in the recent literature by Rudolf,[1] Ivonstantinowitsch,[2] Gierke,[3] Schroeder,[4] Jensen,[5] and Rivet and Girard.^ We have had an opportunity of examining the specimen recorded by Rudolf, which is in the Museum of the Toronto University. The patient was a girl of sixteen years, undeveloped and cyanotic, who suffered from marked dyspnoea and died of pulmonary tuberculosis. The auricular septum was absent and a large right was divided from a smaller left auricular portion, with corresponding auricular appendages, by a slight ridge on the posterior wall. A single large bicuspid auriculoventricular orifice opened into the left side of a single ventricle which gave off a large aorta and a smaller artery from its right side, in transposed relations, and separated from the left part of the cavity by a shallow muscular ridge, which lay in the posterior wall and marked the site of the absent interauricular septum. The pulmonary orifice was stenosed. The cases by Gierke and Konstantinowitsch were identical with this, except that the great vessels were not transposed and in the former the pulmonary artery was atresic, in the latter the aorta. In other cases the development of the aortic septum was completely arrested and a common arterial trunk replaced the two great vessels.


6 Deut. Arch. J. klin. Med., 1911, ccv, 122

7 Giessen Thesis, 1912.

8 Arch, des Mai. du Coeur., November 11, 1913.


An anomalous entrance of the pulmonary veins into the superior cava, innominate, hepatic, or other veins, instead of into the left auricle, was noted in Wilson's case, and in most of those recently reported. In that by Rivet and Girard, of a cyanotic infant aged twenty-five days with polycythemia, these veins formed a common trunk which ended in the lobus spigelii of the liver, and anastomosed here with the venae cavae and portse. It seems possible that this anomaly may be the primary one, at least as regards the non-development of the auricular septum, for comparative anatomy shows that differentiation of the auricles is evolved during the formation of a pulmonary circulation.

Abbott 29.jpg

Fig. 29 "Incomplete double heart," showing (A) the interventricular septum, defective in its upper half; B, a large, thick-walled aorta, arising from both ventricles above the defect; C, a single auriculoventricular cusp arising from both ventricles; D, stenosis of the conus of the pulmonary artery; R.A., the enlarged right auricle. The right auricle and left ventricle are much hypertrophied and dilated; the left auricle and sinus of the right ventricle rudimentary. The interauricular septum is defective in its lower half. (From a specimen in the McGill Pathological Museum, presented by Dr. Andrewes.)


Such cases of pure biloculate heart are extremely rare. A more common form is that in which the septa are partly developed, and an incomplete division has occurred into four cavities, the organ still remaining two-chambered in the exercise of its function (incomplete double heart). A good example is shown in a specimen in the McGill Museum, presented by Dr. F. W. Andrewes (Fig. 29). Here the auricles are incompletely divided into a large right and a small left chamber, by a narrow septum having a large defect above, multiple fenestrations, and a deeply, concave lower free border (persistent ostium primum) . A thick muscular septum, one inch high, with rounded free border, projects upward from the lower wall of the ventricle, partly dividing it into a small, thick-walled right, and a capacious left ventricle. A dilated aorta rides above this rudimentary septum and a narrow thin-walled bicuspid pulmonary arises from a rudimentary conus. There is a common auriculo ventricular orifice with five cusps, one of which is very strong and large and arises from the opposing wall of either ventricle, stretching across above the rudimentary septum and shielding the auriculoventricular orifice from the two arterial ostia.


A third variation of biloculate or triloculate heart is presented by cases of mitral or tricuspid atresia with absent or defective auricular septum. In tricuspid atresia the ventricular septum has been developed, but a defect remains at the base through which the blood passes from the large left ventricle into the pulmonary artery through the persistent bulbus, the sinus of the right ventricle having become obliterated. An excellent example of the former condition (tricuspid atresia) is published by Robertson^ under the title, cor biatriatrum triloculare. The auricular septum was defective below (persistent ostium primum) and an anomalous septum, evidently the persistent right valvula venosa, crossed the right auricle from the Eustachian valve, and was inserted into the base of the interauricular septum at the site of the tricuspid orifice, which was here obliterated, possibly as a result of the insertion of the afiomalous septum at this point. The great arterial trunks were transposed, the aorta arising from the persistent bulbus arteriosus of the obliterated right ventricle, which communicated with the cavity of the left ventricle by a defect at the base of the otherwise fully developed interventricular septum. Aplasia of the left chambers in mitral atresia is described by Bernstein^ and by Girauld and Tissier.[6] In the latter's case the left auricle was tiny, and was separated from the left ventricle by a rudimentary bicuspid valve, and a large orifice united it with the greatly dilated right auricle. The right ventricle was also enormous and gave off both the aorta and the pulmonary artery in normal relations but separated from each other by a thick muscular cushion (apparently marking the conus of the pulmonary artery).


Biloculate heart is frequently displaced to the right side of the body, in that type of dextrocardia that is apparently due to arrest of development. It is often associated with transposition of one or more viscera or other serious defects. The spleen was absent in three of the cases.

Cor Biatriatrum Triloculare

Absence of the ventricular with presence of the auricular septum constitutes a three-chambered heart with two auricles and the tricuspid and mitral orifices opening into a common ventricle, from which (if the aortic septum develops), two arterial trunks arise. Arnold[7] reports a case of complete absence of the ventricular septum, with auricular septum defective below (persistent ostium primum), pulmonary atresia, dextrocardia, and absence of the spleen, and adds a study of 30 case[8] of cor biatriatrum triloculare. As in cor biloculare, obliteration of the right ventricle in tricuspid atresia (with auricular septum present) is frequently reported as triloculate heart.


A special type of anomaly has been established, of what is functionally a cor biatriatrum triloculare, although four chambers exist, by a series of cases recorded in which an anomalous septum cuts off from a common ventricle a small chamber which lies at the base of the heart and gives off one or the other of the great arterial trunks. Such a case, reported and figured by Holmes,^ in 1824, the specimen from which is in the McGill Museum, is represented diagrammatically in Fig. 30. The auricles, which were enormously dilated, especially the right, emptied their contents through their respective ostia into a common ventricle, which gave off the aorta behind and somewhat to the left and communicated through a diamond shaped opening in an anomalous septum with a small cavity at its right upper angle of the common ventricle which gave off the dilated pulmonary artery in its normal relation to the aorta. In eight other similar cases reported by Young/ Peacock,[9] Rokitansky (2 cases), Chiari, Theremin (Obs. 43), and Marchand,^ and in a specimen which the writer has had the privilege of studying in the Museum of the Harvard Medical School, the aorta arose from the small chamber in front and to the right, and the pulmonary artery from the common ventricle behind it, the two vessels being in transposed relations. It has been suggested by Keith that in these cases the anomalous septum is the persistent lower orifice of the embryonic bulbus cordis, but study of both the McGill and Harvard specimens lead us to the conclusion, that in these specimens, at least, the strong muscular wall with a large defect at its upper border through which the small cavity communicates with the large common ventricle, is simply the malposed interventricular septum itself, which has failed to unite with the aortic septum, and has been carried around to the right in the further development of the heart.

Abbott 30.jpg

Fig. 30 Cor biatriatrum triloculare with malposed interventricular septum. Diagrammatic sketch by Prof. R. Tait Mackenzie, showing course of blood and relation of cavities in Dr. Andrew Holmes' case of displaced interventricular septum cutting off a small cavity which gives off the pulmonary artery. The pale line shows venous, the dark line arterial blood. (From the Medical Museum of McGill University.)

Cor Biventriculare Triloculare

When the auricular septum is absent and the ventricular septum present, a heart with two ventricles and a single auricle results. Such a case is reported by Williams.'^ All the blood entered the common auricle through the superior vena cava (the pulmonary veins having again an anomalous entrance).


Symptoms and Physical Signs

In biloculate heart cyanosis is usually present from birth, and becomes very marked. The cases of Forster,^ Ramsbotham,^ and Crisp^ were all typical morbus cseruleus, dying, respectively, at seventy-eight hours, ten days and ten weeks. On the other hand symptoms may be moderate, as in Turner's case, in which there was no cyanosis until just before death at the age of fifteen months. Cases of cor biatriatrum triloculare present perhaps the best illustrations we have that the adniixture of venous and arterial blood is compatible with long life and with only slight disturbances of the circulation. Young's patient, who died at thirty-nine years, cyanosis having developed only within the last three weeks of life, and Peacock's almost identical case, already noted, and that by Mann,^ dying at twenty-one years, are illustrations. Holmes' specimen (Fig. 30) was from a young man, aged twenty-four years, in whom there was only moderate cyanosis and a tendency to suffocative attacks. Physical signs may be prominent, but cannot be said to be characteristic.



  1. Virchows Arch., 1905, clxxxii, 168.
  2. Trans. Path. Soc. London, 1892, xliii, 34.
  3. Anat. Soc. of Gt. Brit, and Ire., Nov., 1899.
  4. Prag. Med. Woch., 1906, p. 657.
  5. Charite-Annalen, 1908, xxxii, 229.
  6. Lancet, 1911, 180, p. 872.
  7. Proc. Neiv York Path. Soc, 1906, N. S., vi, p. 29.
  8. Bull. Soc. d'Obstet. de Paris, 1910, xiii, 420. Virchows Arch., xlii.
  9. Edin. Med. Chir. Soc, 1824, republished by M. E. Abbott, Montreal Med. Jour., 1901.

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Pages where the terms "Historic Textbook" and "Historic Embryology" appear on this site, and sections within pages where this disclaimer appears, indicate that the content and scientific understanding are specific to the time of publication. This means that while some scientific descriptions are still accurate, the terminology and interpretation of the developmental mechanisms reflect the understanding at the time of original publication and those of the preceding periods, these terms and interpretations may not reflect our current scientific understanding.     (More? Embryology History | Historic Embryology Papers)


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العربية | català | 中文 | 中國傳統的 | français | Deutsche | עִברִית | हिंदी | bahasa Indonesia | italiano | 日本語 | 한국어 | မြန်မာ | Pilipino | Polskie | português | ਪੰਜਾਬੀ ਦੇ | Română | русский | Español | Swahili | Svensk | ไทย | Türkçe | اردو | ייִדיש | Tiếng Việt    These external translations are automated and may not be accurate. (More? About Translations)

Abbott ME. Congenital Cardiac Disease (1915) Osler & Mccrae's Modern Medicine 6, 2nd Edition.

Historic Disclaimer - information about historic embryology pages 
Mark Hill.jpg
Pages where the terms "Historic Textbook" and "Historic Embryology" appear on this site, and sections within pages where this disclaimer appears, indicate that the content and scientific understanding are specific to the time of publication. This means that while some scientific descriptions are still accurate, the terminology and interpretation of the developmental mechanisms reflect the understanding at the time of original publication and those of the preceding periods, these terms and interpretations may not reflect our current scientific understanding.     (More? Embryology History | Historic Embryology Papers)
1915 Congenital Cardiac: Congenital Cardiac Disease | Heart Development | Literature | Etiology | Cyanosis | Classification | Pericardium | Heart Displacement | Whole Heart | Anomalous Septa | Interauricular Septum | Interventricular Septum | Absence of Cardiac Septa | Aortic Septum | Pulmonary Stenosis and Atresia | Pulmonary Artery Dilatation | Aortic Stenosis or Atresia | Primary Patency and Ductus Arteriosus | Aorta Coarctation | Aorta Hypoplasia | Diagnosis Prognosis and Treatment | Figures | Embryology History | Historic Disclaimer


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Cite this page: Hill, M.A. (2019, May 19) Embryology Book - Congenital Cardiac Disease 8. Retrieved from https://embryology.med.unsw.edu.au/embryology/index.php/Book_-_Congenital_Cardiac_Disease_8

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