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CONGENITAL CARDIAC DISEASE
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| [[File:Mark_Hill.jpg|90px|left]] This historic 1915 paper by Abbott describes abnormal human heart development.


BY
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[[File:Abbott_1915.jpg|thumb|300px]]
=Congenital Cardiac Disease=
 
By




MAUDE E. ABBOTT, B.A., M.D.  
Maude E. Abbott, B.A., M.D.  


MCGILL UNIVERSITY, MONTREAL, CANADA
Mcgill University, Montreal, Canada




REPRINTED FROM
Reprinted From


OSLER & McCRAE'S MODERN MEDICINE
Osler & Mccrae's Modern Medicine


Vol. IV, 2d Edition, 1915  
Vol. IV, 2d Edition, 1915  
Line 21: Line 38:
Philadelphia and New York  
Philadelphia and New York  


{{Historic Disclaimer}}


CHAPTER X.  CONGENITAL CARDIAC DISEASE.


CHAPTER X.  
By MAUDE E. ABBOTT, M.D.  
CONGENITAL CARDIAC DISEASE.


By MAUDE E. ABBOTT, M.D.


Definition. - Congenital cardiac disease may be defined as that condition  
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  
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  
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.  
associated with congenital cyanosis and clubbing of the fingers, and constitutes in extreme cases the ''morbus cordis'' of the older writers.  




{{Abbott1915}}
==Table of Contents==
[[File:Maude_Abbott.jpg|thumb|Maude Abbott (1869 – 1940)]]


==Table of Contents==
# [[Book_-_Congenital_Cardiac_Disease_1|The Development of the Heart]]
# [[Book_-_Congenital_Cardiac_Disease_1|The Development of the Heart]]
# [[Book_-_Congenital_Cardiac_Disease_2|Literature]]
# [[Book_-_Congenital_Cardiac_Disease_2|Literature]]
Line 56: Line 71:
# [[Book_-_Congenital_Cardiac_Disease_12|Primary Patency and Anomalies of the Ductus Arteriosus Botalli]]
# [[Book_-_Congenital_Cardiac_Disease_12|Primary Patency and Anomalies of the Ductus Arteriosus Botalli]]
# [[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_15|Diagnosis, Prognosis, and Treatment of Congenital Cardiac Diseases]]
==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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This historic 1915 paper by Abbott describes abnormal human heart development.


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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


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 cordis of the older writers.


Table of Contents

Maude Abbott (1869 – 1940)
  1. The Development of the Heart
  2. Literature
  3. Etiology of Congenital Cardiac Disease
  4. Cyanosis
  5. Classification
  6. Anomalies of the Pericardium
  7. Displacements of the Heart
  8. Anomalies of the Heart as a Whole
  9. Anomalous Septa
  10. Defects of the Interauricular Septum
  11. Defects of the Interventricular Septum
  12. Complete Absence or Rudimentary Development of the Cardiac Septa
  13. Defects of the Aortic Septum
  14. Pulmonary Stenosis and Atresia
  15. Dilatation of the Pulmonary Artery
  16. Congenital Aortic Stenosis or Atresia
  17. Primary Patency and Anomalies of the Ductus Arteriosus Botalli
  18. Coarctation of the Aorta
  19. Hypoplasia of the Aorta and its Branches
  20. Diagnosis, Prognosis, and Treatment of Congenital Cardiac Diseases



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

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Pages where the terms "Historic" (textbooks, papers, people, recommendations) appear on this site, and sections within pages where this disclaimer appears, indicate that the content and scientific understanding are specific to the time of publication. This means that while some scientific descriptions are still accurate, the terminology and interpretation of the developmental mechanisms reflect the understanding at the time of original publication and those of the preceding periods, these terms, interpretations and recommendations may not reflect our current scientific understanding.     (More? Embryology History | Historic Embryology Papers)
1915 Congenital Cardiac: Congenital Cardiac Disease | Heart Development | Literature | Etiology | Cyanosis | Classification | Pericardium | Heart Displacement | Whole Heart | Anomalous Septa | Interauricular Septum | Interventricular Septum | Absence of Cardiac Septa | Aortic Septum | Pulmonary Stenosis and Atresia | Pulmonary Artery Dilatation | Aortic Stenosis or Atresia | Primary Patency and Ductus Arteriosus | Aorta Coarctation | Aorta Hypoplasia | Diagnosis Prognosis and Treatment | Figures | Embryology History | Historic Disclaimer


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