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| # [[Book_-_Congenital_Cardiac_Disease_13|Coarctation of the Aorta]] | | # [[Book_-_Congenital_Cardiac_Disease_13|Coarctation of the Aorta]] |
| # [[Book_-_Congenital_Cardiac_Disease_14|Hypoplasia of the Aorta and its Branches]] | | # [[Book_-_Congenital_Cardiac_Disease_14|Hypoplasia of the Aorta and its Branches]] |
| | | # [[Book_-_Congenital_Cardiac_Disease_15|Diagnosis, Prognosis, and Treatment of Congenital Cardiac Diseases]] |
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| ==Diagnosis, Prognosis, and Treatment of Congenital Cardiac Diseases==
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| 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
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| 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 sj-mptoms 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
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| of atypical physical signs.
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| 1 Arch. filr. klin. Med., 1911, ccv, 122.
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| 2 Johns Hopkins Hosp. Bull., 1907, xviii, 136.
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| ^ Virchoivs Arch., 1908, cxciii, 252.
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| The diagnosis of the various defects from each other is a more difficult task. In some of the most complicated forms of congenital cardiac
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| disease both signs and sj^mptoms may be conspicuous by their absence.
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| And on the other hand, several anomalies are frequently combined in*
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| the same case, so that a bizarre picture is liable to be produced, even in
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| the presence of marked physical signs. Nevertheless, a careful study
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| of the literature, and the application of this at the bedside has convinced
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| the writer that in the great majority of cases auricular and ventricular
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| septal defects, abnormal communication between the aorta and pulmonary
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| artery, patent ductus arteriosus, subaortic stenosis, and coarctation of
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| the aorta, all of which conditions are characterized usually by slight or
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| absent cyanosis, can be distinguished from each other and from pulmonary
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| stenosis or atresia, and from those forms of biloculate and triloculate
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| heart, persistent truncus arteriosus and transposition of the arterial
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| trunks, in ivhich the cyanosis is a more conspicuous feature. This statement has the authority of Hochsinger, whose special work along the
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| line of congenital defects convinced him that the differentiation of
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| cardiac defects is largely a question of familiarity with the clinical
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| features.
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| Cyanosis is the rule (to which a few exceptions occur) in pulmonary
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| stenosis and atresia, in complete defects of the septa, as biloculate or
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| triloculate heart or persistent truncus arteriosus, and in transposition
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| of the arterial trunks. It is frequently absent, but may be present
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| and this especially as a terminal event, in patent foramen ovale and in
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| defects of the interauricular and interventricular septa. Cyanosis is
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| usually absent, except during dyspnoeic attacks, in patent ductus and in
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| coarctation and hypoplasia of the aorta.
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| Dysimoea, though always present to a certain degree when cyanosis
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| is advanced, does not appear to bear a definite relation to the degree
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| of deficient aeration but evidently depends on some other factor as well.
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| It is thus characteristic of many cases, such as patent ductus, patent
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| foramen ovale, or septal defects, in which no trace of cyanosis is seen,
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| shortness of breath and palpitation on exertion from early childhood,
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| being quite frequently complained of. The same is true of dyspnoeic
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| suffocative attacks with transient cyanosis which form an important diagnostic feature of such cases.
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| The distinctive character of the physical signs in those defects which
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| are of clinical significance have been discussed under the individual
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| lesions but may be briefly summarized. A harsh, systolic murmur and
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| thrill localized over the upper part of the precordium and of diminished
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| intensity or inaudible at the apex is characteristic of pulmonary stenosis
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| 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
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| pulmonary stenosis and in patency of the duct the murmur usually has
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| its maximum intensity high up over the second left interspace and may
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| be heard beneath the left clavicle. That of the auricular and ventricular
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| septal defects is heard over the third and fourth left interspaces.
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| Murmurs of congenital lesions, when heard in the back, are usually due
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| to patency of the duct or to septal defects. A precordial thrill with the
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| same localization as the murmur is present in about 15 per cent, of
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| congenital defects with physical signs, its presence as a rule corresponding
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| to the degree of harshness of the accompanying murmurs.
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| In defects of the inter auricular septum the murmur is often postdiastolic or presystolic, and in patent foramen ovale presystolic and
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| systolic murmurs may combine or alternate with each other, and may
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| vary with change of position, their inconstancy supplying a differential
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| point.
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| In patent ductus, a harsh rumbling machinery murmur, beginning
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| toward the close of systole and continuous throughout the cardiac cycle
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| is present in a certain number of cases, and when it occurs is pathognomonic; in others the murmur is systolic or (rarely) diastolic. The
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| pulmonary second sound is here usually accentuated and helps to differentiate patent ductus from pulmonary stenosis, in which the pulmonary
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| second is usually (not always) weak or absent. An abnormal area of
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| dulness above and distinct from the cardiac dulness, in the first and
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| second left interspaces (Gerhardt's sign), is also significant of the latter
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| stages of patent ductus, as indicating a dilated pulmonary artery. This
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| sign may be produced also by retraction of an atelectatic left lung
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| (Hochhaus), so is not positive unless confirmed by the a:-rays.
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| A powerful diastolic murmur and thrill with maximum intensity
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| 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
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| of the aortic septum) recorded.
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| Complete transposition of the arterial trunks h^as been diagnosed by
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| the absence of physical signs in the presence of marked cyanosis, and
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| an accentuated pulmonary second sound (Hochsinger).
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| Coarctation of the aorta is to be recognized by the evidences of the
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| collateral circulation when this has been established, hypertrophy of
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| the left heart, the frequent association of an acquired aortic insufficiency,
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| and a reduction in the force of pulsations in the lower extremities as
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| compared with the upper.
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| The above are a few of the indications by which typical cases may be
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| 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
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| conditions, valvular and myocardial, congenital and acquired, far more
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| here, where so wide a range of combinations is possibl^,^than in the
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| acquired forms of heart disease. For this reason we look with interest
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| to the introduction of the newer methods of precision in this difficult and little studied field. Several positive findings have already been
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| recognized and are indicated below. There is good reason to believe
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| that their further application will supply us with a more exact knowedge upon the difi^erential diagnosis of congenital defects.
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| Graphic Methods. - X-ray Examination and Orthodiagraphic Tracings. - Definite information upon the existence of hypertrophy of the
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| ventricles and dilatation of the auricles is to be obtained from the
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| skiagraph. Examination of the shadow at the base of the heart shows
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| also a distinct widening in the presence of dilatation of one or other
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| of the great trunks. When the pulmonary artery is the one dilated the
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| widening appears as a distinct bulging on the left side just above the
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| upper border of the heart, forming an ".r-ray cap" in the position of the
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| so-called Gerhardt's dulness. Dilatation of the aorta is indicated by
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| an increased shadow to the right of the median line in the same situation. Conversely, hypoplasia of the pulmonary artery is indicated by
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| a narrowing of the shadow to the left of the heart's base. These points
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| and the typical shape which the ventricular portion of the heart assumes
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| in the various valvular lesions are well shown in the heart-silhouette
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| obtained by the orthodiagraphic tracing. Groedel^ has figured the outline obtained both in the various acquired valvular lesions and also in
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| congenital pulmonary stenosis, patent ductus arteriosus, and coarctation of the aorta and points out that in patent foramen ovale and in
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| ventricular septal defects there is no change observed at the site of the
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| great vessels so that unless hypertrophy of the ventricles has occurred
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| the silhouette is normal.
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| Fluoroscopic Findings. - Deneke^ diagnosed a case of interventricular
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| septal defect in transposition of the arterial trunks by the appearance
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| on fluoroscopic examination. The heart showed a moderate degree
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| of hypertrophy of the right ventricle, and this chamber formed the
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| right border of the heart in place of the right auricle as normally occurs,
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| so that the strong pumping movement of the ventricle could be seen on
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| the right border as well as on the left, instead of the fluttering auricular
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| movement normally seen in this situation, Deneke describes the
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| appearances as follows: "In normal hearts the movement of the right
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| border is seen on the fluoroscope as a sharp auricular twitching preceding
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| the contraction of the left. Its character can be readily distinguished
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| as auricular, i. e., a short fluttering contraction followed by long, passive
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| dilatation. The movement on the left border is slow, lasting much
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| 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
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| which is slower than the ventricular contraction, but much quicker
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| than the dilatation of the auricles."
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| The Electrocardiographic Curve in congenital hearts has been studied
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| by Lewis,^ Owen,^ Nicolai,^ Ratner,*^ Groedel and Monckeberg,^ and
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| 1 Deut. Arch.f. klin. Med., 1911, ciii.
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| 2 Ibid., 1906, Ixxix, 38.
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| 3 Clinical Electrocardiogra'phy , 1913. * Heart, iii, 113.
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| 5 Berlin, klin. Wchnsch., 1911, xlviii, 51. ^ Berlin Thesis, 1912.
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| ^ Cent. f. Herz. u. Gef. Leid., January 1, 1913.
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| others. So far only a few positive points have been elicited, but these
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| are sufficient to indicate the value of the cardiogram in this connection.
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| (1) A "negative initial Schwankimg" representing a deep exaggeration
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| of the S wave, in lead I, has been observed in many congenital cases,
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| and was described by Nicolai and Steriopulo as pathognomonic of
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| cardiac defects. It is merely significant, however, of the extreme
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| right-sided hypertrophy so common in these cases in which both right
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| auricle and ventricle are involved, and is seen also in acquired mitral
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| stenosis where the same condition of marked right-sided hypertrophy
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| and dilatation occurs; as a corroborative sign it is often of use. (2) An
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| extreme amplitude of curves in several leads w^as observed by Lewis in
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| congenital cyanosis and is described by him as a "valuable sign of congenital valve or septal defects." (3) Finally, the electrocardiogram in
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| true (mirror-picture) dextrocardia when the heart is transposed upon
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| itself, supplies the most positive sign of this abnormality that we possess.
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| In this case Lead I is completely reversed upon itself, and Lead II takes
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| the place of Lead III, exactly the same tracing being obtained as when
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| the leads themselves are reversed in a normal individual. The transposed electrocardiogram decides clearly between this condition and a
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| simple dextroversio cordis. For a comparison of the electrocardiograms
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| in these two conditions (see Fig. 22).
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| The polygraphic tracing may show a positive venous pulse of mitral
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| insufficiency with communication between the two sides of the heart.
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| In the absence of mitral stenosis (in which auricular fibrillation is so
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| common and may give rise to this condition) and when other signs
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| of tricuspid insufficiency are lacking this point may be of diagnostic
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| value.
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| Estimation of the Oxygen Content of the Alveolar Air. - An important
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| diagnostic point between those defects due to abnormal communications
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| between the right and left sides of the heart, and those due to pulmonary
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| obstruction has been supplied by Plesch,^ working in Ivraus' laboratory.
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| In septal defects and patent ductus, there is usually an admixture of
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| arterial blood with the venous current entering the lungs through the
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| pulmonary artery, owing to the fact that under normal conditions the
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| pressure in the aorta is greater, so that blood passes from left to right
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| through the defects. Plesch estimated the amount of oxygen in the
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| alveolar air expired from the lungs which he obtained by his method
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| (described by Boothby and Peabody^), and found that in these conditions the venous blood passing to the lungs is reduced; that is to say,
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| in terms of percentage of its oxygen content, the latter (O2) is raised.
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| 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
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| hand the alveolar air in cases of acquired valvular diseases and in
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| one of congenital pulmonary stenosis was examined and showed no
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| deviation from the normal. These figures and those showing the amount of oxygen consumed are shown in the table taken from an article
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| by Kraus.^
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| 1 Berlin, klin. Wchnschr., 1909, xlvi, 392.
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| 2 Arch. Iv,L Med., 1914, xiii, 502.
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| The differential diagnosis between the cyanosis and clubbing of congenital cardiac diseases and other forms is discussed under cyanosis.
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| Prognosis. - The duration of life has been considered in detail in
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| connection with those defects that are of clinical interest, but a few
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| generalizations may be made. The prognosis varies with the lesion and
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| includes a wide range of possibilities, but is in general grave; this is
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| based upon the direct interference with the circulation by the defect
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| itself, and upon the well-known tendency of certain anomalies to become
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| the seat of a future malignant endocarditis.
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| Among the least harmful forms of congenital cardiac disease may be
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| mentioned anomalous septa in the auricles, patent ductus arteriosus,
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| and coarctation of the aorta with extensive collateral circulation, which
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| may exist until past middle life without symptoms, frequently terminating then with a general failure of compensation under some undue strain.
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| Localized defects of the interauricular and interventricular septa belong
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| likewise to the more innocent lesions which may give rise to symptoms,
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| or may be present indefinitely without producing any effect upon the
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| circulation, becoming serious only upon the advent of some pulmonary
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| complication raising the pressure in the right heart, or through the
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| engrafting of a malignant endocarditis along the edges of the defects.
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| In the more complicated defects life is correspondingly shorter.
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| Young's patient with cor biatriarum triloculare and anomalous septum
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| attained the age of thirty-nine years, and Holmes' twenty-four years,
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| but these are rare exceptions, the subjects of biloculate and triloculate
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| heart usually dying in infancy. This is true also of persistent truncus
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| arteriosus, although a patient reaching twelve years is recorded by
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| Crisp. In pulmonary stenosis early adult life is not uncommonly attained,
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| but is rarely passed, the patients dying as often of tuberculosis as of
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| the direct effects of the lesion. Here again in exceptional cases life may
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| be prolonged, Vulpian recording pulmonary stenosis, rechtslage of the
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| Chart from Kraus' article, Berl. klin, Wchnschr,, 1910, Ixxiv, 230.
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| aorta, and defect of the septum in a man who died at the age of fifty-two
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| years. The average duration of life in pulmonary stenosis is fifteen
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| years, and in atresia 2.25 years in our series.
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| As graver conditions proving almost inevitably fatal during the first
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| weeks or months of life may be enumerated: complete transposition of
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| the arterial trunks without defect of the interventricular septum, pulmonary atresia with closed interventricular septum, tricuspid atresia,
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| and aortic atresia, which is, indeed, the most serious of all, nearly all
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| the cases recorded dying in the first two weeks of life, and many within
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| a few hours of birth. The same is true of most forms of ectopia cordis.
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| Finally, it is to be remembered that of the more complicated anomalies
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| many must perish in the early stages of embryonic development, as
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| only those in whom compensatory conditions arise survive until birth.
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| The prognosis depends largely upon the effects of the lesion upon the
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| circulation, that is, upon the amount of deficient aeration produced,
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| and upon the compensatory powers. For this reason symptoms will
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| frequently prove a better guide to the immediate future than physical
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| signs. Such conditions as septal defect, for instance, may give marked
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| murmurs and thrill, yet lead to no hampering of the heart's action and
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| to little interference with oxygenation until some additional factor, such
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| as obstruction in the pulmonary circulation supervenes. Persistent
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| cyanosis, a continued low temperature, a marked increase in the number
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| of red blood cells (above 5,500,000), and dilatation of the heart, all
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| point to a grave disturbance of the circulation and to a rapidly fatal
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| issue. On the other hand, the entire absence of cyanosis and its
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| attendant phenomena does not always argue a favorable prognosis,
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| for in such cases sudden death may occur without any warning, either
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| quietly, or in a paroxysm of cyanosis with dyspnoea. The embarrassment to the circulation which the lesion itself entails is not the only
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| source of danger. Grave danger lies also in the frequent intercurrence
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| of a malignant endocarditis, and in the fact that infections or bronchopneumonia are apt to prove rapidly fatal. The liability of patients
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| with pulmonary stenosis to tuberculosis, and the frequent termination
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| by sudden cerebral complications, are other unfavorable factors. These
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| considerations indicate the extreme gravity of the more pronounced
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| cases, and the fact that even in the more innocent forms of congenital
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| cardiac disease the prognosis must be framed wdth reserve and caution.
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| Among the better class, where good hygiene prevails and the most suitable conditions of living can be sought, the outlook is of course better
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| than among the children of the very poor.
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| Treatment. — This may be said to begin with the care of the mother
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| during her pregnancy, for a study of the etiology clearly shows that to
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| some unhealthy condition in the environment of the embryo or in the
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| parental organism, rather than to an ancestral tendency toward anomalous growth, the majority of cardiac anomalies owe their origin.
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| The treatment of a patient suffering from congenital cardiac disease
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| must be largely symptomatic or palliative, or directed to the preventing
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| of complications. The indications here are to do all that is possible to
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| facilitate the oxA^genation of the blood, to avoid additional taxation of
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| the burdened circulation, and to shield the patient from accidents or
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| illnesses which increase the pulmonary or systemic obstruction, remembering always that in the majority cyanosis first develops on the addition
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| of some such factor to the pathological conditions produced by the lesion
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| itself. A carefully regulated life, a plentiful supply of light, fresh air,
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| and warmth, the maintenance of an equable bodily temperature, the
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| avoidance of mental agitation and of undue physical exertion, rest, and
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| quiet forms of exercise, where this last is permitted by the condition of
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| the patient, are all essential. The diet should be carefully ordered,
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| light and nutritious, and the often capricious appetite watched. Free
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| action of the excretory organs, especially of the skin, should be promoted
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| and the child kept clothed with flannel. Sudden changes in the external
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| temperature must be avoided and, when possible, resort should be had
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| to a warmer winter climate. Exposure to cold or wet, or to any of the
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| causes of rheumatism, should be avoided on account of the great liability
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| to acute endocarditis. When adult life is attained, choice of light
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| employment which does not call for sudden or great physical exertion is
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| important. In women child-bearing is fraught with danger.
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| Operative interference in patent ductus arteriosus in the form of
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| ligation of the duct, was suggested by Munro^ on the ground that a
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| probable diagnosis is now possible and that the vessel lies in an accessible
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| situation. The fact that distinctive signs occur only after pulmonary
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| dilatation has taken place and a certain adjustment of the vessels to the
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| new order of the circulation has set in, would make one hesitate to
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| resort to so radical a measure, which might introduce a new factor of
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| disturbance.
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| When cyanosis has developed, the administration of oxygen has
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| been suggested as likely to be useful in relieving dyspnoea. Gibson and
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| others report a negative result from its use in several cases. For the
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| relief of the dyspnoeic attacks diffusible stimulants are of benefit and
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| 'should be kept at hand; and in infants the hot mustard bath is useful.
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| The frequent syncope may best be relieved by strychnine. When failing
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| compensation sets in, the usual treatment of rest and cardiac tonics is
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| to be employed, and here strychnine is said to give better results than
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| digitalis.
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| Thus, in a very few words, a careful hygiene and an expectant and
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| preventive treatment may be summed up as the only available assistance
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| that can be given. The condition does not admit of cure, but permits
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| of amelioration and of arrest of the downward trend of the disease.
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| 1 Ann. Surg., 1907, xlvi, 335.
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