Book - Congenital Cardiac Disease 13

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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

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

Coarctation of the Aorta

This term applies to a well-recognized group of cases in which there is a narrowing or stenosis, amounting sometimes to a complete obliteration, of the descending arch at, or immediately below, the so-called isthmus of the aorta, which is that part of this vessel lying between the left subclavian artery and the insertion of the ductus arteriosus. During the period of fetal circulation this segment is comparatively little used, and at birth is usually observed to be of slightly smaller lumen than the adjacent portions of the aorta, the difference soon disappearing under normal conditions. Theremin states, as a result of his measurements of the normal infant heart, that in SO per cent, a slight diminution in diameter exists in the isthmus during the first three months of postnatal life, after which a calibre uniform with the remainder of the arch is attained; and that in some 6 per cent, a slight difference remains throughout life which he does not consider abnormal unless it amounts to more than 2 mm. Bonnett classed as anomalous those cases in which the difference was about 3 mm.

Two distinct groups of cases are understood under the term, (a) A diffuse narrowing of the aorta at the isthmus (Bonnet's infantile type). In some of these cases in which the stenosis is marked, the circulation in the lower part of the body is maintained by a large patent ductus arteriosus through A^hich the descending aorta aypears to he a direct continuation of the jjvhnonary artery. Such cases, being essentially the same in origin as coarctation, may be included with it. (h) A more or less abrupt constriction of the aorta at or near the insertion of the ductus arteriosus (Bonnet's adult type). Here, where coarctation is marked and has lasted some time, the establishment of an extensive collateral circulation frequently completes the picture and lends distinctive features to what is otherwise an obscure lesion.

Relative Frequency

The figures in the literature are somewhat misleading, for curiously little account is taken of its occurrence by many workers, and therefore the lesser degrees of coarctation are probably often overlooked in the postmortem room, and cases with well-marked vascular changes may escape diagnosis at the bedside. On the other hand, this subject has been so carefully worked over and brought up to date by successive writers, that its statistics are clearer and more accessible than is the case perhaps in any other chapter of congenital cardiac disease. Very probably, therefore, the 212 cases enumerated here are not far from being the full number recorded, whereas the total number of pulmonary stenosis or of septal defect (which anomalies have not been subjected, at least of recent years, to such careful repeated statistical analysis) must be much higher than that given by any author. For this reason Vierordt's statement that coarctation ranks next in frequency to pulmonary stenosis is probably placing the incidence too high. A truer estimate may perhaps be gathered from the fact that among 205 cardiac anomalies recorded in the Transactions, there are 22 of stenosis or obliteration of the aorta at the isthmus and 2 of entire absence of the aortic trunk between the left subclavian and the ductus, against 91 of pulmonary stenosis and 165 defects of the interventricular septum.

The first case was reported by Paris in 1789. Craigie collected 10 from the literature in 1841, von Leeuwen 18 in 1850, Rokitansky 26 in 1852, and Peacock 46 cases in 1866. Barie,'^ in 1885, gave a review of 89 cases, in which he published the series of the above authors, with others from the literature. The fact that 6 of these are without autopsy findings reduces the number of Barie's cases for statistical purposes to 83. Schichhold, in 1897, added 30 to these, and Vierordt, in 1898, brought the number of recorded cases to 126. In 1903, Bonnet^ published an article analyzing Barie's findings, and adding to these a synopsis of 77 additional cases which include the series of Schichhold and Vierordt, make, together with the 83 cases collected by Barie, a total of 160, of which 55 are in infants and 105 in adults. In addition to Bonnet's 160, the writer^ has collected records of 52 cases not included by the above authors, 15 of which are in infants and 37 in adults, making a total of 212 cases, of which 70 are in the newly born, and 142 in patients over one year.

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1 Revue de Medecine, 1886, vi, 501. - Revue de Medecine, 1903.

^ Of these 52, 15 are from a series of 18 cases collected by Fawcett from Guy's Hospital Reports and published in 1902; 12 are from the Transactions reported by Chevers (vol. i, p. 55), Rees (vol. ii, p. 203), Peacock (vol. vii, p. 83), Lees (vol. xxi, p. 58), Wilkes (vol. xi, p. 57), Smith (vol. i, p. 52), Barlow (vol. xxvii, p. 41), of coarctation in infants, and by Peacock (vol. xii, p. 38), Finlay (vol. xxx, p. 262), King (vol. xxiii, p. 83), Habershon (vol. xxxix, p. 71), Mackenzie (vol. xxx, p. 66), in adults.

The remainder are reported by Preisz (jahrb. f. Kinderheilk., xxxiii, p. 140), Lawrence and Nabarro, Hektoen, Dick {Proc. Clin. Path. Society, May 9, 1904), Osier {Montreal Gen. Hosp. Rep., P. M. No. 252), Pansch, {Giessen Thesis, 1905) in infants, and by Pappenheimer {Proceedings New York Path. Soc, May, 1905, January, 1906, p. 177, October, 1906), Variot, Carmichael, one in St. Bartholomew's Hosp. Rep., vol. i, series I, one in the Museum of Toronto University, in adults, Monckeberg (2 cases) {Deut. Path. Gesell., 1907, xi, 267), Moon {Lancet, June 8, 1912), Rokitansky (3 cases) {Krank der Arterien, 1862, Obs. 21), Sella (2 cases) {Zieg. Beit., 1910, Lxix, 501), Strassner {Deut. Arch. f. klin. Med., 1909, hx, 349), Wadstein, 1897 (quoted by Sella), Oberndorfer {Verh. deut. Path. Gesell., January, 1910), MacCaUum.

Pathogenesis

The proximity of the stenosis to the insertion of the duct in the aorta suggests that the part which this vessel takes in the circulation, or the changes which go on in its form and tissues during its closure after birth, have an essential bearing on the production of coarctation. Rokitansky (1852) assumed in all cases, as the essential condition, a persistence of the isthmus and a consequent weakening of its walls so that they yielded, in a way the healthy aorta would not do, to the traction exerted upon them by the contraction of the duct in its obliteration. Skoda (1855), made the interesting suggestion that in those cases in which the isthmus was not obliterated at birth as a true anomaly brought about by an atrophy of the corresponding embryonic aortic arch, the tissue of the duct had extended into the wall of the aorta, which thus contracted as part of the same process by which the canal itself is obliterated, and Brunner (1888) supposed the transplantation of free portions of the ductus tissue into the adjacent wall of the aorta to occur, rather than its direct extension.

Bonnet gave the most satisfactory contribution to the subject. He divides the cases of coarctation into two types, according as these occur in the newly born or in adults, for each of which he claims a different etiology :

1. The form described by him as that usually seen in the newly born is a diffuse narrowing of the isthmus, and is assumed to be of developmental origin; it is frequently associated with grave anomalies; in it the ductus arteriosus is often patent. The cases in this type fall again into two classes as regards their etiology: (a) When, as in the majority of cases, the stenosis is moderate in degree, it is explained as a persistence of the isthmus at birth, an arrested fetal condition in which this segment fails to attain its normal calibre, and the cause of which is to be sought at or shortly before birth in a simple weakening of the vessel wall, the result probably of a lowered state of general nutrition. Thus Theremin observed that in the case of his so-called normal infant hearts in which the isthmus was abnormally narrowed at birth, there was a history of premature delivery or of general weakness, and, conversely, that in 50 per cent, of infants born before term or weakly, marked narrowing was present, (h) Those rare cases of the infantile type, on the other hand, with an extreme degree of diffuse stenosis, or in which the isthmus is reduced to an atrophic cord, are probably to be explained, as are also the few recorded cases in which there is a complete absence of the aorta between the left subclavian and the entrance of the ductus, as a failure of development in early embryonic life of that part of the fourth left branchial arch which corresponds to the isthmus of the aorta.

2. Bonnet places in a second class as the adult type those cases seen usually after infancy is passed, in which the coarctation consists of a more or less abrupt constriction of the aorta at or near, often a little below, the insertion of the ductus. This condition, which is never seen in the fetus, nor at birth before the closure of the ductus has begun, is he thinks, not of developmental origin, but is to be explained on Skoda's theory of an extension of the peculiar tissue of the duct into the adjacent wall of the aorta, which thus contracts after birth along with the contraction of the arterial canal. As the malposed tissue is scanty and tends to be of a width corresponding to that of the narrow ductus, its contraction will have the effect of a narrow ligature or cord. These cases differ from those of the infantile type not only in the character of the stenosis, but also in that an extensive collateral circulation, giving rise to marked physical signs, usually develops, while serious anomalies are generally absent, this last fact arguing in favor of its postnatal origin. The ductus arteriosus may remain patent, but is usually obliterated.

Associated Anomalies

The distinction drawn by Bonnet between two types of cases offers a new and significant suggestion. A statistical analysis, on the basis of this division, of the 212 cases available gives interesting conJfirmation of this statement, and points to a radical difference in the etiology of the two groups. The following figures include as minor anomalies occurring chiefly in the adult type, anomalous semilunar cusps, irregular origin of the vessels from the arch, patency of the foramen ovale or duct, persistent left superior cava; and as grave anomalies, septal defects, transposition of the great trunlis, congenital stenosis, etc.

Associated Anomalies in Coarctation in the Newly Born (70 Cases).

Series. Absent. Minor. Grave.

Barie 3 3

Bonnet 13 11 25

New cases 3 12

Total 13 17 40

In Cases 0\^r One Year ("Adult Type") (142 Cases).

Series. Absent. IMinor. Grave.

Barie 57 19 1

Bonnet 15 11 2

New cases 8 16 8

Total 80 46 11

Thus among the 70 cases of stenosis in the newly born (dying under one year), in only 13 instances was there no other defect associated; minor defects were present in 17 and in 40 cases grave anomalies coexisted. That is to say, there is frequently associated with the graver cardiac anomalies that form of coarctation which may reasonably be ascribed to a simple arrest of development in later fetal life, and which is due probably to the depressing influences that led to the associated defects, or possibly in some instances to the disturbed circulation that results from the combined anomaly.

On the other hand, among the 142 cases in patients over one year (adult type), other anomalies were absent in 80 instances, minor defects were present in 46, and grave anomalies were associated in only 11 cases; moreover, 7 of these 11 had not the characteristic sharp constriction seen in the great majority of these cases, but were apparently a persistence of the infantile type; for in 3 (Cbiari, Houel, MacKenzie) the pulmonary formed the descending aorta through a large patent duct, and the 4 others were in children of two to five years in whom the isthmus was simply diffusely narrowed. Transposition occurred in only 1 (Fawcett), a child aged two years and nine months, with a stenosis apparently of the infantile type. Persistent left superior cava was noted only once (Bonnet).

Equally significant with this rarity of grave anomalies in the adult type of coarctation, suggestive, too, of some etiological factor as yet unknown, is the frequent association in this type of a certain set of minor defects in the structures connected with the aortic arch, namely, irregularities in the origin of the great vessels, absence of the ductus (3 cases), double ductus (Hammernjk), and especially anomalies of the aortic cusps, which last are relatively so common as to seem to place their combination beyond the range of coincidence. Thus the aortic valve was bicuspid (in itself a rare anomaly) in 1 5 instances; its segments were increased to four with fusion of two of these in one (Fawcett); in one instance (Babington) a small supernumerary cusp had formed on the aortic wall above the others; in two there was subaortic stenosis, in the form of a membranous band below the cusps, and in one there was sub-aortic stenosis and a band of fibrosis with contraction above the aortic cusps.

In the infantile type, on the other hand, amid so many grave anomalies, bicuspid aortic valves occurred only twice.

Pathology

1. The diffuse stenosis of the isthmus usually observed in infancy and always present during the period of the fetal circulation (Klotz), is seen occasionally, but rarely, in later life. It is usually limited below by the ductus, and may begin above as a gradual diminution of the arch, or abruptly at the origin of the left subclavian artery, or, in a few instances, in which the isthnms itself appears to be placed higher up than usual, at the innominate or left carotid artery. The ascending aorta may be dilated or of normal calibre, and below the stenosis the vessel may remain smaller than usual, may return to its full size, or in cases where its descending portion is supplied by a patent duct, be much dilated. The lining of the stenosed area is usually smooth and healthy. In degree it may vary from a mere shade below the normal to a lumen of 1 to 2 mm. in diameter, or be represented in rare instances by a fibrous obliterated cord. Among the total 212 cases, the pulmonary artery formed the descending aorta through a large patent duct in 15 instances, in all of which marked coarctation of the infantile type existed.

2. Adult Type. - A very different anatomical character and a much wider variation are presented. In typical cases the aorta is abruptly constricted at the level of, or a little above, or, most frequently, directly below the insertion of the ductus, as though by a tight ligature or cord, the groove thus formed being usually deepest on the convex side of the arch, which appears deeply indented as though cut through in V-shaped manner (Fig. 39). The aorta on either side usually diminishes rapidly toward the stenosis in an hour-glass or funnel-shaped manner, or it may be dilated on either side, giving a sausage-like effect (Bradley). Viewed from within, the inner surface of the constriction usually presents a projecting ridge or fold corresponding to the zone of constriction without. This may be so marked as to form a distinct septum bridging across the lumen, sometimes obliterating it entirely or leaving a small central circular or triangular lumen, the constriction involving all the coats of the vessel or only its inner ones, the adventitia passing outside of it like a bridge. In other cases the stenosis may occupy a wide area and appear from without like an annular band. Kriejk describes it in his case as a sort of resistant ring, enclosing the aorta like a cuff parallel to the axis of the vessel, and Mannaberg as a solid segment 0.5 cm. long just below the insertion of the duct. The lumen varies through all grades of stenosis down to one just admitting a bristle. In 25. cases of the 142 it was entirely obliterated, in some instances by a septum or diaphragm formed within, but more frequently by the elongated annular form of constriction.

Fig. 39 Coarctation of the aorta in a woman aged twenty-seven years. Stenosis beginning just beyond the origin of the innominate artery; sharp constriction immediately below the insertion of the obliterated ductus {D. A.). (Reproduced from Bonnet's article, Revue de Medecine, 1903.)

The aorta may be of normal calibre above and below the stenosis, or it may be slightly narrowed at its origin and dilated for a short distance up. The diminution in calibre not infrequently begins at the innominate or left subclavian artery, and in a certain percentage of cases is followed by a dilatation, below which again the characteristic tight constriction near the duct takes place; the effect being that of a double stenosis. The aorta immediately below the stenosis is often widely dilated at the seat of origin of the intercostals. Hypoplasia of the vessel in its whole length existed in the cases of Hale White, Riegel and Monckeberg (2 cases). In other cases the aortic walls, otherwise healthy, are noted as abnormally thin. The aorta may be smooth and healthy in its whole course, as in the cases reported by Brunner (complete obliteration), Cruveilhier, Almagro, Purser, and in the original one by Paris, or there may be extensive atheroma with calcification at the seat of stenosis, above or below it, or throughout the whole aorta. This was present in 39 of the 142 cases, in 9 of which it was definitely stated to be at the seat of stenosis, in 9 localized in the ascending aorta, in 4 localized below the stenosis, and in 7 diffuse throughout the aorta.

Fig. 40 Diagrammatic representation of collateral circulation in a case of coarctation of the aorta: a, pulmonary artery; b, arterial duct; c, arch of aorta; d, coarctation of aorta; e, descending aorta; /, innominate artery; g, internal mammary artery; h, epigastric artery; i, i, i, i, deep-seated arteries of neck and intercostals, forming, together with the internal mammary and epigastric arteries, a collateral circulation with the thoracic and abdominal aorta and internal iliacs. (From Diseases of the Heart and Great Vessels, by W. H, Walshe, London, 1862.)

Rupture of the aorta occurred in 14 cases, in 9 cases at the ascending portion, and in 8 at the seat of stenosis. Aneurism of the arch occurred in 11 instances, and in 8 of these it was of the dissecting form. Sella has made a study of 12 cases of rupture of the aorta, and ascribes its frequent occurrence to the abnormal thinning of the ascending arch, which occurs in many cases of coarctation and which renders the coats more liable to yield under the increased strain.

In most well-marked cases of coarctation of the adult type the blood supply of the lower part of the body is maintained by the development of an extensive collateral circulation. The great vessels of the arch are often enlarged to twice their calibre, and the smaller branches involved are converted into thick, tortuous, dilated trunks. The principal anastomoses are carried on by the superior intercostals, the internal mammaries, and the posterior scapular branches of the transversalis colli above, with the first four aortic intercostals, the phrenic and superficial and deep epigastrics beloiv the stenosis.

Some evidence of collateral circulation was present in 66 of the 142 cases of the adult type. The particular branches involved and the degree of dilatation vary greatly even in cases of extreme constriction. In 3 instances out of the 142 (Barie, Pic and Bonnamour, and Dubreuil) it was expressly stated to be absent; in most of the remainder the collateral circulation was not mentioned, but this does not imply that it was always absent entirely, as minor alterations in the peripheral vessels are easily overlooked.

The ductus arteriosus was patent in 13 of the 142 adult cases. In some instances the ligamentum arteriosum is described as "solidified" or much thickened as though by inflammatory action. Among the 70 cases in infants under one year the ductus was patent 51 times.

Marked hypertrophy with dilatation of the heart is rare in infants, but occurs in the majority of the older cases, being noted in 87 out of the 142. It is stated by most authors to be the direct result of the obstruction in the course of the descending arch, but the relation of the two conditions is rendered uncertain by the frequent association of chronic valvular disease, which must be a factor in the hypertrophy. Moreover, a few cases are recorded {e. g., Reynaud's aged ninety-two,) in which, with marked constriction or, even, as in Brunner's case, s0 obliteration at the insertion of the duct, the heart has remained norrhal throughout life. This w^as stated to be the case in 10 of the 142 cases. Although this is a small percentage, it proves that new channels provided for the blood by the dilated collaterals may be sufficient to carry on the circulation without increasing the work of the heart. In Dumontpallier's patient, aged thirty-nine years, in whom the stenosis was produced by a septum with triangular central opening, 13 mm. in diameter, and "the heart was not hypertrophied, the collateral circulation was carried on chiefly by the aortic intercostals and the vessels from the subclavian, the anastomosis between the internal mammary and the epigastrics being little developed.

On the other hand, an analysis of the 87 cases with hypertrophy shows that while 50 were complicated with chronic valvular lesions or other cardiac defects, in the remaining 38 no cause was present except the coarctation itself. Of the latter, there were 38 cases of hypertrophy without any assignable cause except the coarctation, hypertrophy was confined to the left ventricle in 9, and involved the whole heart in 30, of which latter the left ventricle especially w^as enlarged in 9. An interesting point is that in 20 of the 38, the collateral circulation was either stated to be absent or was not mentioned, and in 6 more it was not much developed, the onFy sign noted being a dilatation of the great vessels of the arch. In the 12 remaining cases a collateral circulation had developed, but in 4 of these the cardiac hypertrophy was only slight.

These facts argue that even in extreme degrees of constriction the heart may remain normal in the presence of an adequate collateral circulation, but that when this becomes insuflBcient, cardiac hypertrophy and dilatation supervene.

Age and Sex

A remarkable predominance of the adult type of the anomaly in the male sex is noted by all writers. Among 133 of the 142 cases over one year, 92 were in males, and 41 in females.

That the stenosis does not necessarily interfere with the duration of life is proved by the fact that 9 patients died in the sixth and 9 in the seventh decade, while one (Reynaud's) lived to the age of 92. More than half of the remainder, however (63 cases), died between the ages of twenty and forty years; indicating that, in 'the anomalous conditions of the circulation that prevail, the system is not, as a rule, equal to the full demands of the stress of normal existence.

In the 142 cases over one year death occurred as follows :

Cases.

1 to 5 years 7

5 to 10 " 4

10 to 15 " 4

15 to 20 " 17

20 to 30 " 37

30 to 40 " 26

40 to 50 " 16

50 to 60 " 9

60 to 70 " 9

Over 70 " 1

Adults (exact age not mentioned) 12

Symptoms and Course

Coarctation in infants is of little clinical significance, except in so far as it may complicate other grave anomalies. In the adult type it is a condition of the greatest interest and importance. Symptoms when present may be distinguished as those associated with the lesion and those of the cardiac insufficiency which frequently supervenes. As characteristic of the overtaxing of the altered circulation, in which the blood supply to the head and upper extremities is freer than that to the lower part of the body, may be mentioned; violent pulsations (Flaherty's case), plethora with sleeplessness and continuous buzzing in the ears (Legrand), violent headaches (Hammernjk), lividity of the face (Che vers. Purser, Kjellberg), suffusion of the head and neck (Moore), epistaxis and hemoptysis (Flint); in Dubreuil's case, a vascular surcharge of the head and chest contrasted with an atony of the subdiaphragmatic viscera; in that of Redenbacher, a boy aged seven years, with a stenosis at the isthmus admitting a crow-quill, and extensive collateral circulation, the development of the head and upper extremities was in advance of that of the lower part of the body. Severe thoracic, epigastric, or abdominal pain and vomiting of long standing (Roemer's case), or pains in the back or lower extremities (Lebert) occur, and may perhaps be due to the local effect of the constipation. Of significance is Muriel's report of a man aged twenty-five years, who was always weakly, and who developed severe pains in the back and symptoms of aneurism of one of the large vessels of the chest; postmortem a dense mass of enlarged glands the size of a hen's egg was found adherent to the aorta at the point of its constriction; it had eroded the dorsal vertebree. Precordial pain and oppression, dyspnoea, and severe palpitation indicate the cardiac strain. Cyanosis is extremely rare except as a terminal event; in only 2 uncomplicated cases in the whole series (Almagro and Carmichael), in both of which the ductus was patent, was a true congenital cyanosis present throughout life. liack of development was noted three times, delayed menses once. Many end with a stage of failing compensation, which, in those not complicated by chronic valvular disease, is usually identical with that of mitral incompetence.

Symptoms are (a) absent, (b) late in developing, or (c) present throughout life, (a) In some of the most w^ell-marked cases of constriction or even obliteration at the isthmus, symptoms are absent throughout life. The condition may be quite latent, and constitute, in Barie's words, a "surprise d'aviphitheatre" at the autopsy, death occurring from some intercurrent, independent disease. Thus Crisp describes a chance finding of a stenosis admitting a goose-quill in a soldier aged fort^'-eight years, who had been in excellent health and had died accidentally, and Scheiber complete obliteration of the descending aorta in a man aged forty-one years, dying of pneumonia, who showed no signs of heart disease. In these latent cases sudden death may occur. In most instances a rupture of the heart or aorta is found (Liittich, Barker, Wise, Legg, and others). Death took place without previous warning or symptoms in 16 of the 142 cases, constituting an event of sufficient frequency to have an important medicolegal bearing.

(b) A large proportion of the cases are in able-bodied, vigorous men, in whom the lesion long remains latent, symptoms developing as the altered circulation becomes overtaxed, or on the intercurrence of some complicating condition, especially endocarditis. Not infrequently, symptoms developing late in life are entirely cardiac in character, cases otherwise latent terminating with a stage of failing compensation which may be due to the lesion itself, or to the chronic valvular disease so often associated.

(c) In a few instances only, symptoms of some obstruction in the cardiovascular system are present throughout life. Quinquaud's patient, a youth aged nineteen years, sufl'ered from infancy with intense palpitation and violent dyspnoea, so that he could not join in play, and oedema of the extremities developed shortly before death, which occurred suddenly. Erman's patient was weakly and had always sufi^ered from dyspnoea. Death took place at 19, after seven and a half months of failing compensation. Lebert's patient, aged twenty-two years, had long had epistaxis and dyspnoea, and developed cardiac symptoms in the last two years.

Physical Signs

These bear no constant relation to the symptoms, but may be present where these are quite lacking. Nor, on the other hand, do they correspond to the degree of the constriction, nor to the extent of the collateral circulation, both of which may be developed to an extreme degree without yielding any evidence of their presence.

The most marked signs appear to be produced in association with chronic valvular disease or bicuspid aortic vaKes, or with the relative mitral incompetency of the later stages of the cardiac dilatation that frequently supervenes, in which cases the murmurs formed in the heart may be propagated along the vessels. The signs peculiar to the lesion may best be studied in uncomplicated cases. They are both vascular and cardiac, and are present in varying degrees and combinations in the majority of cases.

Vascular

These depend chiefly upon the inequality of the circulation in the upper and lower halves of the body, and upon the unusual appearances presented by the dilated collaterals. In well-marked cases the vessels of the upper half of the trunk may be seen pulsating, the subclavians, as a rule, more markedly than the carotids; and pulsation may be traced in many cases along the abnormally dilated and tortuous vessels occupying the course of the internal mammaries on either side of the sternum, or the posterior intercostal or scapular arteries behind. In Libman's patient there was a varicose mass beneath the skin of the abdomen; in Flint's, both supraspinous fossse were occupied by a network of tortuous pulsating vessels; in Leudet's, small arterial dilatations extended over the middle of the thorax both in front and behind, and were most marked at the posterior border of the left axilla and in the left supraspinous fossa, where they formed tortuous, thickened vessels, pulsating synchronously with the radials. Along the whole course of these a murmur, usually postsystolic in rhythm, but sometimes systolic or double, may be heard, and a slight thrill may be felt.

The radial pulse is frequently hard and full, and may be unequal on the two sides. The lower extremities may contrast strangely with the upper half of the body in the absence of all visible pulsations. On examination the pulse in the abdominal aorta and femorals is either very weak or absent, while the murmur usually audible on pressing over the femoral with the stethoscope cannot be heard. In Bonnet's case, diagnosed before death, no pulse could be felt in the abdominal aorta or femorals, and an artery pulsating visibly and as large as the radial, over which an intense systolic murmur could be heard, ran downward between the vertebral column and the inner border of the left scapula. On the right side of the column a similar but less strong pulsation could be felt, but no murmur was heard.

Hornung's patient, a man aged twenty-seven years, is an example of an extreme stenosis not producing any symptoms, but with marked physical signs, in whom death occurred suddenly from rupture of the aorta. There was energetic pulsation and a systolic murmur over the carotids and subclavians. At the inner border of the scapular region were sinuous pulsating vessels. The radial pulse was hard and resistant, and there was no pulsation in the abdominal aorta, popliteral, posterior tibial, or pedal arteries. As long ago as 1839, Mercier diagnosed a case in which there were visible pulsations in the intercostals, a marked bruit at the lower angle of the left scapula, and a very weak pulse in the lower extremities, with epistaxis and symptoms of failing compensation for three months before death.

Cardiac

The heart's action may be tumultuous, with a heaving impulse, and the organ may present evidences of enlargement, particularly of the left ventricle. A precordial thrill was present in only 3 of the cases. The heart sounds may be quite pure, or accompanied by loud murmurs, usually systolic or postsystolic in rhythm. In Hornung's patient a rough murmur was heard at the aortic area, most marked between the left clavicle and the third rib. In Decker's, a woman aged nineteen years, with complete obliteration at the isthmus and no complicating valvular disease, a rasping murmur filling the whole systole was heard at the apex, and could also be traced along the thickened, tortuous, and dilated arteries, among which the superior epigastric, the long thoracic, and the dorsalis scapulae formed pulsating cords; the heart was hypertrophied.

Diagnosis

When such symptoms and signs as the above occur together a very distinctive clinical picture may be formed. It must be remembered that they may be entirely absent, or present only in a fragmentary way, such as may awaken suspicion of the reality, yet render a positive diagnosis impossible. The fact that physical signs as well as symptoms usually do not remain stationary, but progress to a more definite development, furnishes the clue by which the presence of the anomaly may best be traced. A pulsation at an abnormal area, or a superficial murmur of unusual site, noted and watched, may lead to a second examination, at which the full development of the condition may be revealed.

Even where symptoms are present, the diagnosis may be very difficult between a constriction of the descending aorta at the isthmus and obstruction of this vessel or its branches by aneurism, or by the pressure of a mediastinal tumor. The absence of any considerable area of dulness, the transmission of the murmur for long distances along the branches of the ascending arch, the remarkable extent to which the collateral circulation is sometimes developed, above all, the results of a-ray examination, contribute differential points in favor of coarctation. In perhaps no other pathological condition are more extensive changes compatible with fewer evidences during life. The later stage of cases in which vascular phenomena are lacking and failing compensation develops, may be impossible to distinguish from that of organic insufficiency of the mitral valve.

Termination

The cases may be divided into three groups: (1) The condition may be latent throughout life and not interfere with its duration in any way. (2) Both in latent cases and in those presenting symptoms during life death may occur suddenly, by asystole, from rupture of the heart or aorta, or from causes unknown. (3) Death may follow a stage of broken compensation, which may be preceded by symptoms characteristic of the lesion, or may develop suddenly in an apparently healthy subject.

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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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Cite this page: Hill, M.A. (2024, April 16) Embryology Book - Congenital Cardiac Disease 13. Retrieved from https://embryology.med.unsw.edu.au/embryology/index.php/Book_-_Congenital_Cardiac_Disease_13

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© Dr Mark Hill 2024, UNSW Embryology ISBN: 978 0 7334 2609 4 - UNSW CRICOS Provider Code No. 00098G