Book - Congenital Cardiac Disease 15

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

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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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Diagnosis, Prognosis, and Treatment of Congenital Cardiac Diseases

Differential Diagnosis

In the diagnosis of congenital cardiac disease two questions are to be considered: a congenital is to be distinguished from an acquired lesion, and the differentiation may be attempted of the particular defect. The first of these is the more important as well as the simpler problem. It is necessary both for a wise prognosis and for proper treatment to recognize the congenital nature of the lesion, and this can usually readily be done. The following conditions are significant of the presence of a defect: (a) The youth of the patient. (b) A history of symptoms originating in early childhood or in infancy, and of the absence of any event, as rheumatism or endocarditis, which could have led to an acquired lesion, (c) The cyanosis when this is present, and the symptom complex associated with it. (d) The presence of atypical physical signs.

1 Arch. filr. klin. Med., 1911, ccv, 122.

2 Johns Hopkins Hosp. Bull., 1907, xviii, 136. ^ Virchoivs Arch., 1908, cxciii, 252.


The diagnosis of the various defects from each other is a more difficult task. In some of the most complicated forms of congenital cardiac disease both signs and symptoms may be conspicuous by their absence. And on the other hand, several anomalies are frequently combined in the same case, so that a bizarre picture is liable to be produced, even in the presence of marked physical signs. Nevertheless, a careful study of the literature, and the application of this at the bedside has convinced the writer that in the great majority of cases auricular and ventricular septal defects, abnormal communication between the aorta and pulmonary artery, patent ductus arteriosus, subaortic stenosis, and coarctation of the aorta, all of which conditions are characterized usually by slight or absent cyanosis, can be distinguished from each other and from pulmonary stenosis or atresia, and from those forms of biloculate and triloculate heart, persistent truncus arteriosus and transposition of the arterial trunks, in ivhich the cyanosis is a more conspicuous feature. This statement has the authority of Hochsinger, whose special work along the line of congenital defects convinced him that the differentiation of cardiac defects is largely a question of familiarity with the clinical features.


Cyanosis is the rule (to which a few exceptions occur) in pulmonary stenosis and atresia, in complete defects of the septa, as biloculate or triloculate heart or persistent truncus arteriosus, and in transposition of the arterial trunks. It is frequently absent, but may be present and this especially as a terminal event, in patent foramen ovale and in defects of the interauricular and interventricular septa. Cyanosis is usually absent, except during dyspnoeic attacks, in patent ductus and in coarctation and hypoplasia of the aorta.


Dysimoea, though always present to a certain degree when cyanosis is advanced, does not appear to bear a definite relation to the degree of deficient aeration but evidently depends on some other factor as well. It is thus characteristic of many cases, such as patent ductus, patent foramen ovale, or septal defects, in which no trace of cyanosis is seen, shortness of breath and palpitation on exertion from early childhood, being quite frequently complained of. The same is true of dyspnoeic suffocative attacks with transient cyanosis which form an important diagnostic feature of such cases.


The distinctive character of the physical signs in those defects which are of clinical significance have been discussed under the individual lesions but may be briefly summarized. A harsh, systolic murmur and thrill localized over the upper part of the precordium and of diminished intensity or inaudible at the apex is characteristic of pulmonary stenosis and of septal defects. It may in a few cases be heard best at the apex, and it may vary in rhythm, particularly in septal defects. Both in pulmonary stenosis and in patency of the duct the murmur usually has its maximum intensity high up over the second left interspace and may be heard beneath the left clavicle. That of the auricular and ventricular septal defects is heard over the third and fourth left interspaces. Murmurs of congenital lesions, when heard in the back, are usually due to patency of the duct or to septal defects. A precordial thrill with the same localization as the murmur is present in about 15 per cent, of congenital defects with physical signs, its presence as a rule corresponding to the degree of harshness of the accompanying murmurs.


In defects of the inter auricular septum the murmur is often postdiastolic or presystolic, and in patent foramen ovale presystolic and systolic murmurs may combine or alternate with each other, and may vary with change of position, their inconstancy supplying a differential point.


In patent ductus, a harsh rumbling machinery murmur, beginning toward the close of systole and continuous throughout the cardiac cycle is present in a certain number of cases, and when it occurs is pathognomonic; in others the murmur is systolic or (rarely) diastolic. The pulmonary second sound is here usually accentuated and helps to differentiate patent ductus from pulmonary stenosis, in which the pulmonary second is usually (not always) weak or absent. An abnormal area of dulness above and distinct from the cardiac dulness, in the first and second left interspaces (Gerhardt's sign), is also significant of the latter stages of patent ductus, as indicating a dilated pulmonary artery. This sign may be produced also by retraction of an atelectatic left lung (Hochhaus), so is not positive unless confirmed by the x-rays.


A powerful diastolic murmur and thrill with maximum intensity over the second and third interspaces, and an accentuated second pulmonary sound characterized several of the cases of abnormal communication between the lower part of the aorta and pulmonary artery (defect of the aortic septum) recorded.


Complete transposition of the arterial trunks h^as been diagnosed by the absence of physical signs in the presence of marked cyanosis, and an accentuated pulmonary second sound (Hochsinger).


Coarctation of the aorta is to be recognized by the evidences of the collateral circulation when this has been established, hypertrophy of the left heart, the frequent association of an acquired aortic insufficiency, and a reduction in the force of pulsations in the lower extremities as compared with the upper.


The above are a few of the indications by which typical cases may be distinguished from each other. But in this subject it should be remembered that hard and fast lines may not be drawn. Thus rhythm, maximum intensity, transmission of murmur or thrill, and all other manifestations of the defect will be found to vary with the associated cardiac conditions, valvular and myocardial, congenital and acquired, far more here, where so wide a range of combinations is possible than in the acquired forms of heart disease. For this reason we look with interest to the introduction of the newer methods of precision in this difficult and little studied field. Several positive findings have already been recognized and are indicated below. There is good reason to believe that their further application will supply us with a more exact knowedge upon the difi^erential diagnosis of congenital defects.


Graphic Methods

X-ray Examination and Orthodiagraphic Tracings. - Definite information upon the existence of hypertrophy of the ventricles and dilatation of the auricles is to be obtained from the skiagraph. Examination of the shadow at the base of the heart shows also a distinct widening in the presence of dilatation of one or other of the great trunks. When the pulmonary artery is the one dilated the widening appears as a distinct bulging on the left side just above the upper border of the heart, forming an "x-ray cap" in the position of the so-called Gerhardt's dulness. Dilatation of the aorta is indicated by an increased shadow to the right of the median line in the same situation. Conversely, hypoplasia of the pulmonary artery is indicated by a narrowing of the shadow to the left of the heart's base. These points and the typical shape which the ventricular portion of the heart assumes in the various valvular lesions are well shown in the heart-silhouette obtained by the orthodiagraphic tracing. Groedel^ has figured the outline obtained both in the various acquired valvular lesions and also in congenital pulmonary stenosis, patent ductus arteriosus, and coarctation of the aorta and points out that in patent foramen ovale and in ventricular septal defects there is no change observed at the site of the great vessels so that unless hypertrophy of the ventricles has occurred the silhouette is normal.


Fluoroscopic Findings

Deneke^ diagnosed a case of interventricular septal defect in transposition of the arterial trunks by the appearance on fluoroscopic examination. The heart showed a moderate degree of hypertrophy of the right ventricle, and this chamber formed the right border of the heart in place of the right auricle as normally occurs, so that the strong pumping movement of the ventricle could be seen on the right border as well as on the left, instead of the fluttering auricular movement normally seen in this situation, Deneke describes the appearances as follows: "In normal hearts the movement of the right border is seen on the fluoroscope as a sharp auricular twitching preceding the contraction of the left. Its character can be readily distinguished as auricular, i. e., a short fluttering contraction followed by long, passive dilatation. The movement on the left border is slow, lasting much longer than that of the auricular, and is a strong pumping motion followed by a short delay in the contracted state, then a gradual dilatation which is slower than the ventricular contraction, but much quicker than the dilatation of the auricles."


The Electrocardiographic Curve in congenital hearts has been studied by Lewis,^ Owen,^ Nicolai,^ Ratner,*^ Groedel and Monckeberg,^ and others. So far only a few positive points have been elicited, but these are sufficient to indicate the value of the cardiogram in this connection.


(1) A "negative initial Schwankimg" representing a deep exaggeration of the S wave, in lead I, has been observed in many congenital cases, and was described by Nicolai and Steriopulo as pathognomonic of cardiac defects. It is merely significant, however, of the extreme right-sided hypertrophy so common in these cases in which both right auricle and ventricle are involved, and is seen also in acquired mitral stenosis where the same condition of marked right-sided hypertrophy and dilatation occurs; as a corroborative sign it is often of use.

(2) An extreme amplitude of curves in several leads was observed by Lewis in congenital cyanosis and is described by him as a "valuable sign of congenital valve or septal defects."

(3) Finally, the electrocardiogram in true (mirror-picture) dextrocardia when the heart is transposed upon itself, supplies the most positive sign of this abnormality that we possess. In this case Lead I is completely reversed upon itself, and Lead II takes the place of Lead III, exactly the same tracing being obtained as when the leads themselves are reversed in a normal individual. The transposed electrocardiogram decides clearly between this condition and a simple dextroversio cordis. For a comparison of the electrocardiograms in these two conditions (see Fig. 22).



1 Deut. Arch.f. klin. Med., 1911, ciii.

2 Ibid., 1906, Ixxix, 38.

3 Clinical Electrocardiography , 1913. * Heart, iii, 113.

5 Berlin, klin. Wchnsch., 1911, xlviii, 51.

^ Berlin Thesis, 1912.

^ Cent. f. Herz. u. Gef. Leid., January 1, 1913.


The polygraphic tracing may show a positive venous pulse of mitral insufficiency with communication between the two sides of the heart. In the absence of mitral stenosis (in which auricular fibrillation is so common and may give rise to this condition) and when other signs of tricuspid insufficiency are lacking this point may be of diagnostic value.

Estimation of the Oxygen Content of the Alveolar Air

An important diagnostic point between those defects due to abnormal communications between the right and left sides of the heart, and those due to pulmonary obstruction has been supplied by Plesch, ^ working in Ivraus' laboratory. In septal defects and patent ductus, there is usually an admixture of arterial blood with the venous current entering the lungs through the pulmonary artery, owing to the fact that under normal conditions the pressure in the aorta is greater, so that blood passes from left to right through the defects. Plesch estimated the amount of oxygen in the alveolar air expired from the lungs which he obtained by his method (described by Boothby and Peabody[1]), and found that in these conditions the venous blood passing to the lungs is reduced; that is to say, in terms of percentage of its oxygen content, the latter (O2) is raised. This was also demonstrated in two cases of Vaquez disease (polycythemia with splenomegaly) in which the oxygen content of the alveolar air was examined by Senator-Lowy and v. Bergmann. On the other hand the alveolar air in cases of acquired valvular diseases and in one of congenital pulmonary stenosis was examined and showed no deviation from the normal. These figures and those showing the amount of oxygen consumed are shown in the table taken from an article by Kraus.[2]


The differential diagnosis between the cyanosis and clubbing of congenital cardiac diseases and other forms is discussed under cyanosis.

Prognosis

The duration of life has been considered in detail in connection with those defects that are of clinical interest, but a few generalizations may be made. The prognosis varies with the lesion and includes a wide range of possibilities, but is in general grave; this is based upon the direct interference with the circulation by the defect itself, and upon the well-known tendency of certain anomalies to become the seat of a future malignant endocarditis.


Among the least harmful forms of congenital cardiac disease may be mentioned anomalous septa in the auricles, patent ductus arteriosus, and coarctation of the aorta with extensive collateral circulation, which may exist until past middle life without symptoms, frequently terminating then with a general failure of compensation under some undue strain. Localized defects of the interauricular and interventricular septa belong likewise to the more innocent lesions which may give rise to symptoms, or may be present indefinitely without producing any effect upon the circulation, becoming serious only upon the advent of some pulmonary complication raising the pressure in the right heart, or through the engrafting of a malignant endocarditis along the edges of the defects.


In the more complicated defects life is correspondingly shorter. Young's patient with cor biatriarum triloculare and anomalous septum attained the age of thirty-nine years, and Holmes' twenty-four years, but these are rare exceptions, the subjects of biloculate and triloculate heart usually dying in infancy. This is true also of persistent truncus arteriosus, although a patient reaching twelve years is recorded by Crisp. In pulmonary stenosis early adult life is not uncommonly attained, but is rarely passed, the patients dying as often of tuberculosis as of the direct effects of the lesion. Here again in exceptional cases life may be prolonged, Vulpian recording pulmonary stenosis, rechtslage of the aorta, and defect of the septum in a man who died at the age of fifty-two years. The average duration of life in pulmonary stenosis is fifteen years, and in atresia 2.25 years in our series.


Chart from Kraus' article, Berl. klin, Wchnschr,, 1910, Ixxiv, 230.


aorta, and defect of the septum in a man who died at the age of fifty-two year The average duration of life in pulmonary stenosis is fifteen years, and in atresia 2.25 years in our series.


As graver conditions proving almost inevitably fatal during the first weeks or months of life may be enumerated: complete transposition of the arterial trunks without defect of the interventricular septum, pulmonary atresia with closed interventricular septum, tricuspid atresia, and aortic atresia, which is, indeed, the most serious of all, nearly all the cases recorded dying in the first two weeks of life, and many within a few hours of birth. The same is true of most forms of ectopia cordis. Finally, it is to be remembered that of the more complicated anomalies many must perish in the early stages of embryonic development, as only those in whom compensatory conditions arise survive until birth.


The prognosis depends largely upon the effects of the lesion upon the circulation, that is, upon the amount of deficient aeration produced, and upon the compensatory powers. For this reason symptoms will frequently prove a better guide to the immediate future than physical signs. Such conditions as septal defect, for instance, may give marked murmurs and thrill, yet lead to no hampering of the heart's action and to little interference with oxygenation until some additional factor, such as obstruction in the pulmonary circulation supervenes. Persistent cyanosis, a continued low temperature, a marked increase in the number of red blood cells (above 5,500,000), and dilatation of the heart, all point to a grave disturbance of the circulation and to a rapidly fatal issue. On the other hand, the entire absence of cyanosis and its attendant phenomena does not always argue a favorable prognosis, for in such cases sudden death may occur without any warning, either quietly, or in a paroxysm of cyanosis with dyspnoea. The embarrassment to the circulation which the lesion itself entails is not the only source of danger. Grave danger lies also in the frequent intercurrence of a malignant endocarditis, and in the fact that infections or bronchopneumonia are apt to prove rapidly fatal. The liability of patients with pulmonary stenosis to tuberculosis, and the frequent termination by sudden cerebral complications, are other unfavorable factors. These considerations indicate the extreme gravity of the more pronounced cases, and the fact that even in the more innocent forms of congenital cardiac disease the prognosis must be framed wdth reserve and caution. Among the better class, where good hygiene prevails and the most suitable conditions of living can be sought, the outlook is of course better than among the children of the very poor.


Treatment

This may be said to begin with the care of the mother during her pregnancy, for a study of the etiology clearly shows that to some unhealthy condition in the environment of the embryo or in the parental organism, rather than to an ancestral tendency toward anomalous growth, the majority of cardiac anomalies owe their origin.

The treatment of a patient suffering from congenital cardiac disease must be largely symptomatic or palliative, or directed to the preventing of complications. The indications here are to do all that is possible to facilitate the oxygenation of the blood, to avoid additional taxation of


the burdened circulation, and to shield the patient from accidents or illnesses which increase the pulmonary or systemic obstruction, remembering always that in the majority cyanosis first develops on the addition of some such factor to the pathological conditions produced by the lesion itself. A carefully regulated life, a plentiful supply of light, fresh air, and warmth, the maintenance of an equable bodily temperature, the avoidance of mental agitation and of undue physical exertion, rest, and quiet forms of exercise, where this last is permitted by the condition of the patient, are all essential. The diet should be carefully ordered, light and nutritious, and the often capricious appetite watched. Free action of the excretory organs, especially of the skin, should be promoted and the child kept clothed with flannel. Sudden changes in the external temperature must be avoided and, when possible, resort should be had to a warmer winter climate. Exposure to cold or wet, or to any of the causes of rheumatism, should be avoided on account of the great liability to acute endocarditis. When adult life is attained, choice of light employment which does not call for sudden or great physical exertion is important. In women child-bearing is fraught with danger.


Operative interference in patent ductus arteriosus in the form of ligation of the duct, was suggested by Munro[3] on the ground that a probable diagnosis is now possible and that the vessel lies in an accessible situation. The fact that distinctive signs occur only after pulmonary dilatation has taken place and a certain adjustment of the vessels to the new order of the circulation has set in, would make one hesitate to resort to so radical a measure, which might introduce a new factor of disturbance.


When cyanosis has developed, the administration of oxygen has been suggested as likely to be useful in relieving dyspnoea. Gibson and others report a negative result from its use in several cases. For the relief of the dyspnoeic attacks diffusible stimulants are of benefit and should be kept at hand; and in infants the hot mustard bath is useful. The frequent syncope may best be relieved by strychnine. When failing compensation sets in, the usual treatment of rest and cardiac tonics is to be employed, and here strychnine is said to give better results than digitalis.


Thus, in a very few words, a careful hygiene and an expectant and preventive treatment may be summed up as the only available assistance that can be given. The condition does not admit of cure, but permits of amelioration and of arrest of the downward trend of the disease.



  1. Berlin, klin. Wchnschr., 1909, xlvi, 392.
  2. Arch. Iv,L Med., 1914, xiii, 502.
  3. Ann. Surg., 1907, xlvi, 335.

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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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