Book - Congenital Cardiac Disease (1915)
CONGENITAL CARDIAC DISEASE
BY
MAUDE E. ABBOTT, B.A., M.D.
MCGILL UNIVERSITY, MONTREAL, CANADA
REPRINTED FROM
OSLER & McCRAE'S MODERN MEDICINE
Vol. IV, 2d Edition, 1915
Lea & Febiger
Philadelphia and New York
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CHAPTER X.
CONGENITAL CARDIAC DISEASE.
By MAUDE E. ABBOTT, M.D.
Definition. - Congenital cardiac disease may be defined as that condition in which, through arrest of development or disease occurring in intra-uterine life, anomalies in the anatomical structure of the heart or great vessels exist, leading to irregularities in the circulation. It is frequently associated with congenital cyanosis and clubbing of the fingers, and constitutes in extreme cases the morbus ccBndeus of the older writers.
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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
Table of Contents
- The Development of the Heart
- Literature
- Etiology of Congenital Cardiac Disease
- Cyanosis
- Classification
- Anomalies of the Pericardium
- Displacements of the Heart
- Anomalies of the Heart as a Whole
- Anomalous Septa
- Defects of the Interauricular Septum
- Defects of the Interventricular Septum
- Complete Absence or Rudimentary Development of the Cardiac Septa
- Defects of the Aortic Septum
- Pulmonary Stenosis and Atresia
- Dilatation of the Pulmonary Artery
- Congenital Aortic Stenosis or Atresia
- Primary Patency and Anomalies of the Ductus Arteriosus Botalli
- Coarctation of the Aorta
Hypoplasia of the Aorta and its Branches
Hypoplasia of the aortic system may be described as that condition in which the lumen of the arterial vessels in the greater circulation remains abnormally small and the walls unnaturally thin and elastic. The heart may also be reduced in size or may undergo a compensatory dilatation and hypertrophy which involves especially the left ventricle, but may extend to the whole organ, and is usually succeeded by a marked degree of secondary dilatation. The subjects are, as a rule, pale individuals of delicate frame, who present signs of retarded development, such as a delayed advent of the signs of puberty. Anomalies of the sexual organs frequently occur. The general health is usually fair until early adolescence, when the condition generally manifests itself after some unusual physical strain has been endured, by the sudden appearance of failing compensation. The course is then progressively downward. In women, who are by natural conditions less exposed to undue muscular exertion than are men, this stage of cardiac insufficiency may not supervene, but the disease may run its course under the guise of a chlorosis. By some observers (Ortner, Hiller) the narrowing of the vessels is thought to predispose to the infectious fevers, and a special group of cases in which death has occurred from typhoid fever is described. It is also seen in young anemic subjects dying of pulmonary tuberculosis.
There has been some debate as to the pathological significance of the condition. Several authors have maintained that the greater elasticity of the walls of the vessels compensates for their smaller calibre, and so prevents undue strain upon the heart. A number of statistical contributions, have, however, demonstrated that hypoplasia of the aorta must be given a place in pathogenesis as one of the special causes of cardiac asystole. The etiology is obscure. In some few cases, such as the cachexias of wasting diseases, a true atrophy of the aorta occurs. In the majority some congenital defect, amounting in some instances to a congenital tendency to dwarfism, may be supposed. This view is supported by the frequent association of other anomalies, especially in the generative and circulatory systems.
Typical cases were described by Morgagni in 1761 and by Meckel in 1788. Rokitansky defined the condition in 1838 and commented upon its association in some instances with defects of the external genitalia. Bamberger, in 1843, noted the association of chlorosis with a small aorta. But in general the subject attracted little attention until Virchow, in 1872, published a series of cases illustrating the frequency of a small elastic aorta and a small heart in chlorosis, and suggested an etiological relation between the two conditions.- He explained the absence of compensatory hypertrophy of the heart in some cases and its presence in others, as depending upon the degree of diminution of the lumen of the vessels, the volume of the circulating blood, the elasticity of the vessel wall, and the amount of work done by the individual. Ortner, in 1891, dwelt chiefly on the medicolegal aspect of the subject, and emphasized as pathognomonic an absence of the jugular pulsation in the episternal notch in cases in which the upper border of the cardiac dulness is high. Spitzer (1897) attempted by a study of the recorded material to place the condition on a more definite clinical basis. He pointed out that while the cases usually terminate with failing compensation, this resembles the end stages of chronic valvular disease only in a general way, that the symptoms are in general those of a cardiac overstrain due to muscular fatigue, and have a progressive tendency to grow worse; that during the stage of broken compensation the cardiac dulness is usually much enlarged, and that the sounds are generally clear, with marked pulmonary accentuation, although occasionally accompanied by murmurs. Like Virchow, he noted as characteristic a remarkable pallor, but he ascribed it not to a diminution of the hemoglobin, which he found usually 90 to 100 per cent., but to the reduction in size of the vessels through which a smaller quantity of blood coursed beneath the skin.
Burke^ (1901) gave a historical review of the subject and a full account of all the cases on record. He divided the material into four groups:
(1) Hypoplasia of the aorta in the so-called blood diseases, as chlorosis, pernicious anemia, hemophilia; (2) hypoplasia in association with infectious diseases, considered as predisposing to these or tending to their fatal termination; (3) hj^Doplasia with general dystrophies, as acromegaly; (4) hypoplasia presenting the picture of a cardiac lesion, the mass of the cases belonging to this last group. Apelt^ collected 100 cases from the literature and added an account of two cases, both of which were diagnosed during life. The subjects were young men aged seventeen and twenty-one years, of slight build and medium size, who had been capable of the usual amount of physical exertion, and had presented no symptom of disease. Both passed through a period of unusual physical strain just before the sudden onset of symptoms, which took place a few weeks before death. The picture was that of an acute dilatation of the heart with slight terminal cyanosis, oedema, ascites, the cardiac area enormously increased, and the pulmonary second sound markedly accentuated. The heart sounds were pure except toward the close in one patient, in whom a systolic mitral murmur developed. Postmortem, in both cases, the arteries were throughout thin, delicate, elastic, and of diminished calibre, and there was moderate hypertrophy with great pathological dilatation of the heart, although its valves and chordae tendinese were delicate, and healthy. Microscopic examination revealed an entire absence of fatty degeneration of the myocardium.
Van Ritook analyzed 73 cases, 56 from the literature including the series of Burke and Apelt, and 17 from personal observation, and he enumerated the following points as of diagnostic value: (1) The youth of the patient. (2) Marked and obstinate anemia persisting in spite of all treatment. (3) The early development of fatigue in a young individual on slight physical exertion. (4) Subnormal temperature or only slight rise of temperature in febrile diseases. (5) Palpitation. (6) Hypertrophy of the left heart. (7) Acute cardiac insufficiency developing after comparatively slight physical strain. (8) Diminished resistance to infectious diseases.
^Deut. Arch.f. Uin. Med., 1901, No. 71, 187.
2 Deut. med. Woch., 1905, xxxi, 1186.
VOL. IV — 28
Anomalies of the Aortic Arch
Quite a wide variation of anomalous conditions of the aortic arch and its branches occur, the individual forms of which repeat themselves in different subjects with such similarity, that an underlying developmental error may be inferred. The units of the series may be summed up under the various headings of (1) double aortic arch, (2) right aortic arch, (3) origin of left subclavian artery, from (a) a patent ductus arteriosus, or (b) the pulmonary artery, (4) origin of the right subclavian artery from the descending thoracic aorta below the left subclavian artery, and (5) common brachiocephalic trunk. In all these the underlying defect is either a persistence of an embryonic arch which normally undergoes involution (double aortic arch, right aortic arch, left subclavian from patent ductus), or an arrested development of a portion of the embryonic arches that normally persists (right subclavian from thoracic aorta, common brachiocephalic trunk). In man the primitive aorta is at first double and of the six embryonic arches the first, second, and fifth disappear on both sides as w^ell as the left sixth and the distal part of the left fourth, while the third parts persist as the carotid arteries, the fourth left as the aorta, the proximal portion of the right fourth, as the subclavian, and the left sixth becomes the pulmonary artery with the ductus arteriosus.
Double Aortic Arch. - In this anomaly, of which some 8 cases have been recorded, the aorta ascends to the right and turns backward and divides near the beginning of its transverse portion into two large trunks which lie parallel with each other and unite just beyond the insertion of the ductus to form the descending arch, enclosing between them an elliptical space in which the oesophagus and trachea lie embraced within the vascular circle thus produced. The posterior member of the pair, which is usually the larger, appearing as the true arch of the aorta, gives off the right carotid and subclavian, and lies behind the trachea. The smaller anterior limb lies below the other, appearing like a loop from it, and gives off the left carotid and subclavian, either as a simple trunk (left innominate) or as separate vessels. Examples are the cases recorded by Curnow^ in a woman, aged eighty-seven years, and by Hamdi,^ in a woman aged forty-five years. In the latter case the trachea and oesophagus were slightly compressed. Although no symptoms had been produced, the deformity of the trachea was sufficient to prove the possibility of a fatal obstruction.
Henle explains the posterior limb of the double aorta as a persistence of the fourth right arch. The anterior limb represents the fourth left arch, and the two unite at the point of insertion of the ductus (sixth arch) to form the descending aorta as in the embryo, and as is persistent in the amphibia.
Right Aortic Arch. - In this anomaly the aorta is normal at its origin, but curves over the root of the left instead of the right lung, so that its convexity lies to the left, and it passes down on the right side of the aorta, the right recurrent laryngeal nerve hooking round the arch in the same manner as does the left under normal conditions. The left carotid, or, in some cases, a left innominate artery, arises from the front of the aorta shortly after its origin and represents the persistent left aortic root. The right carotid rises next in about its normal situation, and then the right subclavian more posteriorly and to the right, while the left subclavian arises either (a) in its normal situation or (6) with the left carotid from the left innominate, or from a patent ductus or from the pulmonary artery.
1 Trans. Path. Soc, London, 1874, xxvi, 23.
2 Deut, wed. WocK, 1906, xxxii, 1410.
Fig. 41
Dr. Kaufmann's case of right aortic arch with ligamentum arteriosum encircling the trachea and oesophagus. Diagrammatic representation following Evans' diagram of the survival of the aortic arches, to show that in the present case the fourth right arch (represented by the arch and trunk of the descending thoracic aorta), the left proximal part of the fourth left arch (represented by the left innominate and subclavian), and the left sixth arch (represented by the hgamentum arteriosum), survive, and that the trachea is necessarily encircled by the passing over of the ductus to the arch of the opposite side. The oesophagus is here omitted for the sake of clearness: Car. Int. Sinistra, left internal carotid; Car. Ext. Sinistra, left external carotid; Art. Car. Comm. Sinistra, left common carotid; Car. Int. Dextra, right internal carotid; Car. Ext. Dextra, right external carotid; Art. Car. Comm. Dextra, right common carotid; Art. Subcl. Sinistra, left subclavian; Art. Subcl. Dextra, right subclavian; Arc. Aortic Dextra, right aortic arch; Art. Pulm. Sinistra and Dextra, left and right pulmonary arteries; D. A., funnel-sbaped patent aortic end of ductus; A. P., pulmonary arterj'. (Drawing by Prof. G. Adami, McGiU University.)
The curve of the aorta passing from the right to left and then back to the right side of the vertebral column becomes very sinuous in those cases in which the ligamentum arteriosus or a patent ductus remains attached to the right aortic arch at its usual site of insertion opposite or near the left subclavian. In these cases the ductus is forced to pass from its origin in the pulmonary artery, on the left side anteriorly, backward and to the right to meet the right arch which curves toward it beJiind the trachea and oesophagus which are thus again, as in double aortic arch, engaged in a complete vascular circle formed in this case by the aorta, ductus arteriosus (patent or obliterated), and the pulmonary artery. Four such cases are recorded, in two of which the ductus was obliterated; in the third, it was widely patent, and in the fourth case (a specimen in the McGill Museum presented by Dr. Kaufmann, which has been figured for me diagrammatically by Professor Adami, see Fig. 41), it was widely patent at its aortic end, but was closed beyond, the ligamentum arteriosum forming a long thick cord. In the latter case the aorta gave off a left innominate trunk and then curved backward soon after its origin and to the left, passing behind the trachea and oesophagus and gaining the right side of the vertebral column below. At the point where the convexity of the arch gains the left side of the trachea, it presents a deep triangular pouch, which represents the patent aortic end of the ductus, to the apex of which externally a cordlike structure of remarkable length and thickness, the ligamentum arteriosum, is attached. This ligament passed forward anteriorly to the trachea and oesophagus to its attachment in the left branch of the pulmonary artery, and encloses these viscera within the vascular circle formed by it with the aorta and pulmonary artery.
Such cases form a link between simple right aortic arch, in which the aorta lies entirely on the right side of the trachea, and double aortic arch in which trachea and oesophagus are completely embraced by a vascular ring. They throw light on the development of the latter, at first sight inexplicable, phenomenon. For, since a right aortic arch must pass behind the trachea to unite with the persistent sixth left arch which is represented by the ductus, it must do the same to unite with persistent left fourth arch which is represented by the anterior limb of the double pair. The whole situation is explained by the reflection that in the embryo these viscera occupy a position, not behind, but on the left posterior aspect of the primitive heart, and that the pairs of embryonic arches pass on either side of them to their destination in the dorsal aorta, so that if arches on opposite sides unite as they have done in the anomalies under consideration, trachea and oesophagus are bound to be encircled.
Left Subclavian from Ductus Arteriosus or Pulmonary Artery. - While the left subclavian is given off from the fourth right aortic arch, it is practically a continuation of the distal part of the sixth left arch represented by the ductus arteriosus to which its origin bears a constant relation.
Right Subclavian from Descending Thoracic Aorta. - In this anomaly the aortic arch has its normal course to the left, but the right subclavian is given off from a point in the thoracic aorta just below the insertion of the ductus arteriosus and passes up to its normal distribution. Here an arrest of the proximal part of the fourth right arch which normally forms the right subclavian has occurred, and the obliteration of the distal portion which in the embryo unites the fourth arch with the aortic trunk has not taken place.
Common Brachiocephalic Trunk. - All four great vessels may arise by common origin, recalling the embryonic stage in which all the arches emerged together from the third carotid arch. A case is cited by Freyberger.^
Clinical Aspects. - The evidences presented during life and the clinical significance of all the above conditions are slight. In right aortic arch and common brachiocephalic trunk the aorta is apt to rise higher toward the neck than is normal, leading to violent pulsation in the episternal notch, which may lead to a mistaken diagnosis of aneurism. In the case of origin of the left subclavian from the pulmonary artery or patent ductus the left arm usually remains entirely free from cyanosis, which shows how far the system can accommodate itself under favorable conditions to an admixture of venous with arterial blood.
Anomalies of the Coronary Arteries
Anomalous Origin from the Pulmonary Artery. - A vessel may arise from a sinus of Valsalva of the pulmonary artery, and, meeting the branches from the aortic coronaries, produce a remarkable anastomosis of a cirsoid character. In Brook's first case, a vessel the size of a crowquill sprang from the right anterior sinus of Valsalva of the pulmonary and passed down over the infundibulum of the right ventricle, there anastomosing with the aortic coronaries. In his second case a large anomalous artery arose from the same situation. It gave no branches to the heart but passed to the left and upward to enter a complicated mass of thin-walled arteries, which lay around the main pulmonary trunk and passed up along the trachea and behind the aortic arch. This mass received three other large vessels, one from the left subclavian, one from the right aortic coronary, and one from the posterior aspect of the transverse aortic arch. Krause's case is similar.
The McGill specimen (see Fig. 42), was from a woman aged sixty years, v/ho died accidentally. The right coronary arose in its normal situation from the anterior sinus of Valsalva of the aorta by a much dilated orifice, and expanded directly after its origin into a huge thickwalled loop the size of a crab-apple, which projected upward some 2.5 cm. above the subepicardial fat, and gave off the descending branches from the loop. Both these and the main trunk of the vessel were wide, thick-walled, tortuous channels. No coronary arose behind the left posterior aortic cusp in the normal situation of the left coronary, but instead a large patulous opening lay in the floor of the dilated posterior sinus of Valsalva of the pulmonary artery. From this sprang a large thin-walled trunk of venous character, which divided about 1 cm. beyond its origin into two large branches, one of which ran to the left in the auriculoventricular groove in the course normally followed by the transverse circumflex branch of the left coronary artery, while the other ran downward along the front of the interventricular septum in the position of its descending branch, and was here expanded into a large triangularly shaped venous sinus, 2 cm. in its widest diameter, and diminishing in size toward the apex. In the floor of this sinus were several thick-walled septa behind which large vessels opened into it from the myocardium.
1 Trans. Path. Soc, London, 1898, xliv, 44.
2 Jour. Anat. and Physiol., 1902-03, xxvii, 387.
Fig. 42
Aneurismal dilatation (arteriovenous aneurism) of branches of coronary arteries in a case of anomalous
origin of the left coronary from the pulmonary artery. (From a specimen in the Medical Museum of
McGill University, Montreal.)
The question of the circulation in the anastomosing vessels, in which blood from the systemic and pulmonary circulations must have mingled is of interest. Brooks suggests that the direction of the current must have been toward the cirsoid aneurism in the coronaries arising from the aorta, and toward the right ventricle in the coronary that arose from the pulmonary artery, which would thus drain the mass and would also send some arterial blood to the lungs.
In the McGill specimen the peculiar septa in the floor of the large venous sinus formed by the descending branch of the anomalous vessel, strongly suggested that the course of the blood was toivard the pulmonary artery. This case is additionally interesting from the fact that the anomalous vessel was here clearlv the left coronarv, which was absent from its normal situation and arose from the pulmona^3^ Both this and Brooks' second case were in elderly subjects, and the condition had not produced any manifestations during life.
Miscellaneous Anomalies. — Accessory coronaries may be present or both vessels may arise behind a single aortic cusp, or there may be a complete absence of one. A case has been recorded of an anomalous coronary sent to the lungs in pulmonary atresia.
Anomalies of the Pulmonary Arteries
Accessory Pulmonary Artery. - A series of 10 cases has been collected from the literature by McCotter,i [^ which an anomalous artery had arisen from the aorta or its branches, and had supnlied the lower lobe or the accessory lobe of one or other lung. In the case reported by himself, in a man aged sixty-five years, this artery was 7 mm. in diameter, and was given off from the front of the thoracic aorta on a level with the tenth dorsal vertebra, and passed up to the right between the folds of the ligamentum latum pulmonis to the lower margin of the right lung, where it ramified. The lung pleura and mediastinum were otherwise normal. In 8 of the cases collected the accessory branch was from the thoracic aorta, in one from the abdominal aorta, and in one from the seventh intercostal artery. In 5 cases the accessory branches supplied an accessory lobe and in 5 the lung was normal.
Pathogenesis. - The final explanation must be deferred until the origin of the pulmonary circulation is better understood. McCotter gives an interesting discussion. Accessory pulmonary arteries have been described in amphibia and reptiles, and are said to be normal in the latter. Thoma and Evans found that the blood \ascular system in the embryo arises as a capillary plexus spreading in all directions. Such a capillary plexus forms caudally from the pulmonary arches and envelops the primitive lung anlage with a rich capillary plexus. In the case of the accessory pulmonary branch, this plexus must have formed laterally from a primitive thoracic aorta and joined the pulmonary plexus just as a capillary network extends to the limb-bud. The explanation of this anomaly is thus either (1) that this plexus always occurs but has failed to atrophy in the present case; or (2) that the plexus is only occasionally laid down, i. e., is in itself an anomaly, and when present results in an accessory pulmonary branch. The condition has not shown itself to be of any clinical significance.
i^naL i?ec., August, 1910, 291.
Anomalies of the Systemic Veins entering the Heart and of the Pulmonary Veins
Systemic Veins. - Persistent left superior vena cava is the commonest of these anomalies. It is not infrequent in conjunction with other cardiac defects, and occurred 26 times in our series. It is of little clinical importance but is of great interest in cases where the congenital origin of the associated condition is questioned, as indicating the developmental nature of the latter. The cases may be divided into two groui3s : those in w^hich the right superior cava is also present, and those in which it is absent, and the blood from the upper portion of the body enters the right auricle through the persistent left cava. A series of 4 cases of persistent left cava is published by Schutz.^ In three of these the right cava was also present, in one it was equal in size to the left; in a second it was small and the left cava communicated with the left auricle by a valvular opening in its wall before entering the right auricle at the coronary sinus. In this case the apex of the heart was bifid; and the patient was a woman of thirty-eight of whom no other history was obtainable. In the third case the left superior cava was persistent but rudimentary. In Schutz's fourth case the right superior cava was absent. The left innominate veins emptied into the left innominate at the level of the left common carotid to form a left superior cava thicker than a man's thumb which widened into a bulbar swelling 4.5 cm. across, which entered the coronary sulcus and opened into the right auricle above the inferior vena cava. Habershon- reported a case of absence of the superior cava in a man aged thirty-seven years. The usual opening of the superior cava in the right auricle was marked by a smooth, white area of endocardium, like a closed foramen ovale, and there was extensive development of collateral circulation through the vena azygos major. The persistent left cava was formed by a union of the left jugular and subclavian and right innominate veins and emptied into the dilated coronary sinus. The recent literature on complete absence of the right superior cava with persistent left is given by Dietrich.^
A very interesting case is published by Beyerlein,^ in a boy, aged one and a quarter years, of double superior vena cava, in which the orifice of the coronary sinus in the right auricle was obliterated by the overgrowth of an extensive network of Chiari. The persistent left cava received all the blood from the coronary veins and the heart, and emerged from the coronary sulcus at the normal situation, emptying into the right superior cava through the transverse branch. Two cases practically identical with this very rare anomaly are reported by Gruber^ and LeCat (quoted by Gruber). Nabarro^ describes a case of double superior cava in an infant of three months where the persistent left duct, smaller than the right, was joined by the left hepatic vein, which emptied with it into the coronary sinus. Here the left horn of the embryonic sinus venosus had evidently escaped obliteration.
A series of cases in which a displacement to the left of the superior cava has taken place so that its orifice comes to lie directly above the interauricular septum, and looks into both auricles, which has been described by Ingalls and others, and a similar condition of the inferior cava by Rokitansky, are described under auricular septal defects.
1 Virchows Arch., 1914, ccxvi, 35.
2 Trans. Path. Soc, Lond., 1876, xxvii, 79. ^ Virchows Arch., 1913, ccxii.
4 Frank. Zeit. f. Path., 1914, xv, 327.
^ Virchows Arch., 1885, xcix, 492.
^ Jour. Ajiat. and Path., 1902, xxxvii, 387.
Pulmonary Veins. - An anomalous distribution of the pulmonary veins
is much more common than is generally supposed, and quite serious
deviations from the normal have been attended with surprisingly little
results. Nevertheless, their displacement occurs in many complicated
anomalies, and their repeated combination with these grave defects
suggests a primary error in development in the pulmonary veins anlage.
Quite a large series of cases of biloculate heart are reported in which
the pulmonary veins were deflected from their entrance to the left
"auricle and were received by one or other of the great veins. Schroeder^
gives a full discussion of the various anomalies of the pulmonary and
systemic veins and traces their developmental origin, with especial
reference to those cases, like his own, in which a complete defect of the
interauricular septum was associated. Nabarro describes the pulmonary
veins opening into the coronary sinus in an infant aged five and a half
months, in whom all the blood from the systemic circulation must have
passed through the patent foramen ovale.
In the cases reported by Ingalls,- Chiari and others, of defects at the upper part of the interauricular septum, the right pulmonary veins either entered the right auricle or the superior vena cava. In those reported by Borst and Stoeber^ of an anomalous septum in the left auricle, the pulmonary veins entered the smaller upper chamber in the left auricle to the right of the anomalous septum, which evidently represented the septum primum, deflected to the left by the entrance of the pulmonary veins too far to the right side. In all, the primary defect is apparently the deflection of the pulmonary veins.
Ramsbotham describes a case in which the left pulmonary entered the left subclavian, and the right pulmonary the portal vein, and in three others (Arnold, Bochdalek, Geipel) the right and left pulmonaries entered the portal vein together as a common trunk. The pulmonary veins of both sides may enter the left auricle as a single or as two trunks. Here the original single vein has not been taken up in the wall of the auricle as occurs in normal development.
The clinical significance of these conditions depends less upon the defect itself than upon the associated developmental conditions.
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 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 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 sj^mptoms 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 a:-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 possibl^,^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 ".r-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
1 Deut. Arch.f. klin. Med., 1911, ciii.
2 Ibid., 1906, Ixxix, 38.
3 Clinical Electrocardiogra'phy , 1913. * Heart, iii, 113.
5 Berlin, klin. Wchnsch., 1911, xlviii, 51. ^ Berlin Thesis, 1912.
^ Cent. f. Herz. u. Gef. Leid., January 1, 1913.
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 w^as 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).
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^), 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.^
1 Berlin, klin. Wchnschr., 1909, xlvi, 392.
2 Arch. Iv,L Med., 1914, xiii, 502.
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
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
years. 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 oxA^genation 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^ 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 Ann. Surg., 1907, xlvi, 335.
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Abbott ME. Congenital Cardiac Disease (1915) Osler & Mccrae's Modern Medicine 6, 2nd Edition.
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