Book - Congenital Cardiac Disease 3

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

Historic Disclaimer - information about historic embryology pages 
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Pages where the terms "Historic" (textbooks, papers, people, recommendations) 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, interpretations and recommendations 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

Etiology of Congenital Cardiac Disease

Cardiac anomalies may be divided, according to etiology, into two main groups: those due to arrest of growth at an early stage, before the different parts of the heart have been entirely formed, and those produced in the more fully developed heart by fetal disease.

Arrest of Growth

From the earliest times search has been made for the underlying causes of the arrest of development manifest in cardiac malformations. Long before Darwin, Meckel, in 1812, pointed out the resemblance of certain defects to the hearts of those animals, which present in a stationary form the different stages through which the mammalian heart passes in its development, and explained them as reversions to a more primitive type.


In seeking the causes of the defect we may turn first to the study of associated anomalies. Do these occur in such frequency and constancy as to place their combination beyond the range of coincidence? And if so, may the causes leading to malformations elsewhere, such as disease and adhesions of the amnion, maternal disease, hereditary predisposition, etc., be assumed to act upon the fetal heart?


In Rokitansky's Defekie der Scheidewdnde des Herzens, among 24 complicated defects of the septum, all evidently of developmental origin, associated anomalies such as transposition of the viscera, cleft palate, etc., occurred in 8, that is, in one-third of the cases. Yierordt, in the 700 cases reviewed by him, found associated anomalies in 80 (11 per cent.). On the other hand Keith found among 23 malformed fetuses and infants showing anencephaly, hydrocephaly, spina bifida, umbilical hernia, atresia ani, cleft palate, harelip, and stricture of the oesophagus, in 14 a malformation of the heart.


Among the 631 cases studied here, anomalies elsewhere in the body, among which may be enumerated malformations of liver and lung, asymmetry of calvarium, partial or complete transposition of viscera, harelip and cleft palate, encephalocele, gastro- and rachischisis, absence of spleen or kidney, diverticula, h^^pospadias, hernia, etc., occurred in 92 cases, that is, in 14 per cent. Defect of the interventricular septum was associated in Chaffey's case with imperforate anus, in Moore's with a supernumerary thumb, in Morestin's with syndactylism and absence of femur, fibula, and genitalia. A widely patent foramen ovale was combined in Berthel's case with rudimentary genitalia, and in Tylecote's with a congenital perforation of the nasal septum. Kingsley reports patent ductus with macroglossia and absence of the left kidney, and Dick a case of pulmonary artery forming the descending aorta, with the uterus bipartite, and the kidneys fused. Mental deficiency or derangement of the higher nerve centres is not infrequent. Thus idiocy was reported by Simmons, Carpenter and Rheiner, in cases of patent foramen ovale, patent ductus, and septal defect respectively, and in a biloculate heart reported by Dublizhaza, idiocy was combined with strabismus and pes varus.


Further illustrations might be multiplied, but the above suffice to show that the association of grave anomalies with cardiac defects is too frequent to be considered accidental. That the cause of both is to be sought, not so much in a hereditary predisposition, as in a diseased condition of the fetal envelopes or of the maternal tissues is evident from the facts yielded by the family history of these cases. For a history of congenital disease in the ancestry is much less common, than is one of cardiac defect or other anomaly in other members of the same generation, and evidence of infective processes or depressing influences acting within the parental organism is still more frequently supplied. In this series there was a history of congenital defect in a brother or sister of the patient in 11 cases, of rheumatism or heart-disease in the parents in 13, and of small-pox or tuberculosis in six. Congenital syphilis in the father was recorded by Jacobi, in a case of ectopia cordis, and lues whether congenital or acquired is certainly a frequent cause. Baneful influences acting upon the mother during the early weeks of pregnancy have been described, such as great trouble, ill-treatment and fright. Severe inflammation of the bladder of the mother in the third month was blamed by Habershon for the development of pulmonary atresia with septal defect, and an operation on the mother for appendicitis was noted by Royer and Wilson, in their case of incomplete heterotaxy. In not a few instances the parents had both reached advanced middle life, and in some the child was the last of a series of many pregnancies.


Difficult delivery occurred in the cases of patent ductus by Luys, Roeder, and others. Laine reported a case of aortic stenosis with septal defect from a mother aged forty-eight years, who had had four other children, of whom three were feeble-minded.


The predominating cause of the defect is thus clearly to be sought in the immediate environment of the developing embryo. It must be recognized that the early death in most cases of congenital cardiac disease prevents direct transmission of cardiac defects, which might otherwise occur, and this lessens the apparent frequency of heredity. That heredity is a factor in a certain proportion of cases is evidenced by numerous facts. The association of symmetrical polydactylism is significant when one considers the well known familial tendency of this anomaly. Of much interest also is a specimen reported by J. McCrae[1] in which transposition of the viscera and atresia of the pulmonary artery were found in the fifteenth child of a forty-six-year-old mother, who was herself of poor intelligence and had a harelip.

Fetal Disease

Acute endocarditis was formerly claimed to be a widely acting cause of congenital cardiac disease, not only in those instances in which in the fully formed heart the traces of its presence are incontestable, but in the earlier cases of arrest of growth which were explained as due to its action upon the half-developed embryo. With the increasing knowledge of development, the trend of modern opinion is to explain the majority of cardiac defects as arrest of growth, dependent upon a variety of causes as enumerated above, and to class with these many cases of pulmonary stenosis and atresia, formerly thought to be inflammatory in origin. There remains a certain proportion due to fetal endocarditis, but it is impossible to state the exact degree of its influence, and it is therefore safest to divide cardiac defects into two classes, viz., those in which an arrest of development from any cause has taken place, I and those which show, by thickening and cicatricial contraction, that they have been produced by fetal disease after the heart has been fully formed.


The presence of thickening of the endocardium does not prove that a defect is originally due to an inflammatory process, for cardiac defects, giving rise so readily to abnormal currents and to undue strain upon the valves, are particularly liable to be the seat of future disease.



  1. 1 Jour. Anat. and Physiol., 1905, xl, 48.

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Pages where the terms "Historic" (textbooks, papers, people, recommendations) 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, interpretations and recommendations 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" (textbooks, papers, people, recommendations) 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, interpretations and recommendations 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. (2024, March 28) Embryology Book - Congenital Cardiac Disease 3. Retrieved from https://embryology.med.unsw.edu.au/embryology/index.php/Book_-_Congenital_Cardiac_Disease_3

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