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# [[Book_-_Congenital_Cardiac_Disease_8|Complete Absence or Rudimentary Development of the Cardiac Septa]]
# [[Book_-_Congenital_Cardiac_Disease_8|Complete Absence or Rudimentary Development of the Cardiac Septa]]
# [[Book_-_Congenital_Cardiac_Disease_9|Defects of the Aortic Septum]]
# [[Book_-_Congenital_Cardiac_Disease_9|Defects of the Aortic Septum]]
# [[Book_-_Congenital_Cardiac_Disease_10|Book_-_Congenital_Cardiac_Disease_10]]
# [[Book_-_Congenital_Cardiac_Disease_10|Book_-_Congenital_Cardiac_Disease_10|Pulmonary Stenosis and Atresia]]
 
# [[Book_-_Congenital_Cardiac_Disease_10|Dilatation of the Pulmonary Artery]]
==Pulmonary Stenosis and Atresia==
 
 
Pulmonary stenosis is the form of cardiac defect most familiar to the
practitioner. It is of much clinical importance on account of its comparative frequency, the relatively long duration of life, and the prominence
of the cyanosis nearly always associated. To the student also it is a subject of the highest interest, for in its symptomatology and pathogenesis
are focused the most difficult problems of congenital cardiac disease.
 
Owing to the wide variations in the conditions presented and the
differing aspects from which the subject must be approached, a classification of the different forms is as difiicult as it is necessary. Rauchfuss
points out that the simple anatomical findings furnish the best guide to
a useful grouping. Thus, the degree of narroicirig_is important, and a
simple stenosis is to be distinguished from a complete atresia; from the
stand-point of pathogenesis, the seat and character of the stenosis are
criteria of much value; and thirdly, the presence or absence of defects
of the mterventricidar seiDtum provides a dividing line of the greatest
importance. This last is important etiologically as indicating the stage
of embryonic or fetal life at which the stenosis took place, and clinically
in that the duration of life and symptomatology differ somewhat in the
two groups.
 
----
1 Jour. Anat. and Physiol., 1877, xi, 302.
 
 
 
Statistics. - Pulmonary stenosis is probably the commonest of all
oarcW^ r anomaH ls. The oases are scalfer'eJ'soTreerv'-TErougK the literature
that an exact statistical statement is impossible. Vierordt estimated at
least 300 in 1898, and placed coarctation of the aorta next in frequency
with 130 cases. Among 181 anomalies of the heart which he analyzed,
Peacock found 119 of pulmonary defect. In 1906 Keith^ examined 185
specimens of cardiac malformations in the hospital museums of London,
and found that in 135, or 70 per cent., there was an anomalous condition of the pulmonary tract, the deformity being in the conus of
the right ventricle in 133 cases and in the pulmonary valve in 22.
In his later communication,^ among 272 malformed hearts examined
in the various London Museums, Keith found 141 in which the defect
was due to an imperfect transformation of the bulbus cordis of the
embryo. Of these 141, in 19, there was incomplete fusion of the
infundibulum with the body of the right ventricle (conus a separate
chamber) ; in 44, partial arrest in development of the infundibulum ; in
37, complete arrest of the infundibulum; in 23 fusion of semilunar valves;
in 7, partial or complete absence of the body of the right ^'entricle with
development of the infundibulum; in 4 subaortic stenosis; and in 7,
congenital aortic stenosis.
 
Among the cases of anomalies analyzed here, there are 150 of congenital
pulmonary disease. The proportion of stenosis to atresia in recorded
cases analyzed is as follows:
 
Number
Stenosis. Atresia. analyzed.
 
Kussmaul 64 26 90
 
Rauchfuss 81 33 114
 
Peacock 90 29 119
 
Vierordt 83 24 107
 
Theremin 20 10 30
 
This series .116 34 150
 
The relatively high percentage of atresia in Theremin's cases is explained b}" the fact that his material was entirely among infants, in whom
the mortality from atresia is high.
 
The condition of the fetal passages was the subject of statistical study
by Meyer, Kussmaul, Taruffi, and other authors, of whose work a full
review is given by Vierordt. A defect of the interventricular septum
exists in the great majority. 'THe number of cases with closed septum
IS relatively larger in atresia than in stenosis; thus Rauchfuss finds
among 192 cases, 171 in which the interventricular septum is defective
and 21 in which it is closed. Of these 21, 10 are cases of atresia and 11 of
stenosis. Among Vierordt's 83 cases of stenosis are 71 with defective
and 12 with closed interventricular septum; among his 24 of atresia, in
14 the septum was defective and in 10 it w^as closed. When the interventricular septum is entire, the foramen ovale is usually widely patent,
but it also may in rare cases be closed.
 
----
 
1 Festschrift, Quater centenary Aberdeen University, July, 1906.
 
2 Hunterian Lectures, Lancet, 1909, ii, 359, 433, 519.
 
 
 
Among the 116 cases of stenosis analyzed here the interventricular
septum was defective in 95, and entire in 21 cases. In 12 of these 21
the foramen ovale was patent, but in 9 the auricular septum was also
closed, and no communication existed between the two sides of the
heart. Among the 34 cases of atresia the interventricular septum was
defective in 26 and entire in 8 cases. Of these 8, in one the foramen
ovale was also closed but there was a large patent ductus. The ductus
arteriosus is nearly always patent in atresia but is usually closed in
jtenosis. Among the 82 cases of pulmonary stenosis classed as the primary lesion in the chart, the ductus was patent in only 11; of the 24 cases
of atresia, it was patent in 15 cases. The condition of the cardiac septa
and ductus in these cases was as follows:
 
Stenosis. Atresia.
 
Number Number with Number Number with
analyzed. patent D. A. analyzed, patent D. A.
 
F. 0. and V. S. closed ... 7 1 1
 
F. O. patent, V. S. closed . . 11 1 5 4
 
F. O. closed, defect V. S. ... 40 5 7 3
 
F. O. patent, defect V. S. ... 24 5 11 7
 
Total 82 11 24 15
 
Rechtslage of the aorta is present in the majority of cases with septal
defect, and is especially frequent in atresia. Among the 64 cases of
pulmonary stenosis with septal defect, classified in the chart as the
primary lesion, it was present in 39 cases, in 29 of which the aorta arose
from both ventricles above the defect, and in 10 chiefly or entirely from
the right ventricle. Among the 18 cases of atresia with septal defect
the aorta arose from both ventricles above the defect in 5, entirely from
the right ventricle in 10.
 
Pathology. — Pulmonary Stenosis. — The narrowing may involve the
whole pulmonary tract, or be localized to the valve, artery, or conus.
Two distinct types may be recognized:
 
1. In a few cases the stenosis is valvular in character and is produced
by a thickening, shortening, or fusion of the pulmonary cusps. A thick
diaphragm with three raphe of fusion on its arterial surface is usually
formed, which protrudes into the pulmonary artery in a funnel-shaped
way and is perforated by a circular or triangular opening of varying size.
The pulmonary artery is frequently dilated above, may be normal, or
somewhat thin-walled. The conus below the valve shares in the hypertrophy of the right ventricle, but is otherwise normal; the interventricular
septum is usually closed. There is every evidence to show that the
stenosis has originated in an inflammatory process, in later fetal life after
the heart has been fully formed. ^'
 
2. In the second and larger group, the cusps may or may not be
thickened or fused, but the stenosis is due to a rudimentary condition,
hypoplasia, or deformity of some part of the pulmonary tract. In
these cases a defect of the interventricular septum is usually associated
 
"^and a deviation to the right of the aorta, so that this arises from both
ventricles above the defect, or chiefly from the right ventricle, communicating with the left through the defect. Such forms, which suggest a
'developmental origin, make up the great majority of cases of pulmonary
stenosis, and the combination of these three conditions, pulmonary stenosis, defect of the septum at the base, and rechtslage of the aorta,/
 
I is probably the commonest of all cardiac anomalies.
 
In the majority of cases - in Keith's estimate 90 per cent. - the conns
of the right ventricle is involved in the deformity. Two distinct types of
conus stenosis may be distinguished. The whole infundibulum may be
more or less constricted, its musculature thickened, and th^ endocardium
opaque. In a case of this kind, reported by Cautley,^ the pulmonary
cusps were delicate and healthy above the stenosis, but both they and the
artery were very small. Usually the valves are thickened, fused, or rudimentary, and they are often bicuspid. Sometimes a thin diaphragm
with delicate raphe showing no sign of inflammatory change, is stretched
across the pulmonary orifice, suggesting an incomplete division of the
endocardial cushions. In these cases a defect of the septiun is almost
invariably present.
 
A second group of conus deformities is that in which a cavity, described
by some of the older pathologists as a third ventricle, is cut off from
the sinus of the right ventricle by a definite septum perforated by a
small opening. There are 19 in this series, including one case of atresia.
Keith describes and figures an illustration of this anomaly. The infundibulum is enormously dilated and communicates with the sinus by a
small opening with thickened fibrous borders. The pulmonary cusps
are large and competent, the artery dilated above, and there is a small
defect of the interventricular septum. Another variation of this form
of conus deformity is figured by Andrewes.^ The conus is atrophied and
is represented by a small cavity with thick muscular walls. It communicates with the sinus of the ventricle by an opening admitting a
crow-quill one-quarter inch below the pulmonary cusps, which are
small, bicuspid, and not thickened.
 
1 Hypertrophy and dilatation of the right ventricle and auricle are
 
/constant in pulmonary 'stenosis, and in the cases associated with defectof the septum at the base and rechtslage of /the aorta. In these the
hypertrophy is sometimes most marked in the wall of the sinus of the
ventricle, indicating that it had been produced by the force needed to
send the blood into the aorta through the defect, rather than by the
obstruction in the pulmonary artery. The aorta is usually thick walled
and of large caliber. In the developmental cases the pulmonary artery
is usually narrow and thin-walled, resembling a vein in structure. When
the stenosis is confined to the valve, the artery may be dilated.
 
Pulmonary Atresia. — All that has been said of the seat and character of
the deformity in pulmonary stenosis applies equally to a complete atresia.
In a small series of cases the point of obliteration is at the valve, the
artery dilated above, the foramen ovale widely open, and the septum
entire. In Weiss'^ case the seat of atresia was in the conus, which admitted
only a pinhead or a fine straw, and was lined by thickened endocardium;
just above this were two fairly large pulmonary cusps, and the artery
itself was comparatively large. There was a small patent foramen ovale, a large defect of the septum at the base, and a large thick-walled aorta
arose frora the right ventricle above the defect.
 
----
 
1 Edin. Med. Jour., 1902, xii, 257. 2 Trans. Path. Soc, 1865, xvii, 45.
 
s Brit. Med. Jour., 1877, ii, 378.
 
 
 
 
 
 
The pulmonary artery may be obliterated for some distance above the
valve, forming a fibrous cord, which may emerge suddenly from the fleshy
outer wall of the ventricle and give no sign of its origin from within, or it
may be patent throughout, diminishing toward the orifice in a funnelshaped way. In such cases the cusps may be seen thickened and fused
with each other at the bottom of the cul-de-sac formed by the artery,
or they may form a triradiate elevation of three fleshy cushions; or no
trace of them may remain. The aorta is usually very large. When a
defect in the septum is present it rides over it, or in many cases arises
entirely from the right ventricle.
 
 
 
Fig. 36
 
 
 
 
Heart and lungs of an infant, cyanotic from birth, showing (A) atresia of the pulmonary artery; B,
patent ductus arteriosus suppljdng lungs; C, defect of interventricular septum at pars membranacea,
guarded by (D) an anomalous valve with false chordse tendinese; E, tricuspid orifice; F, left pulmonary
artery; G, right pulmonary artery; H, cord passing through the septal defect; LA, left auricle. (From
a specimen in the McGill Pathological Museum, presented by Sir Wilham Osier.)
 
The foramen ovale is frequently widely patent. It may be the only
means of communication between the two sides of the heart, the interventricular septum remaining entire. Such cases are less frequent, and
the condition is more serious than that associated with septal defect.
 
The alterations in the cavities of the heart vary with the condition
of the interventricular septum. When this is entire the left ventricle
is greatly hypertrophied and dilated, and both auricles share in the
enlargement, while the right ventricle undergoes a true concentric hypertrophy, its wall becoming greatly thickened and its cavity aplastic and lined with opaque thickened endocardium, or in some instances completely
obliterated. When a defect of the septum exists, the right ventricle is
greatly hypertrophied and is dilated as well, and the right auricle is
correspondingly enlarged, the left chambers remaining relatively small.
When the aorta rides over the defect in the septum, the left ventricle
may share in the hypertrophy.
 
The Pulmonary Circulation. - When the pulmonary artery is obliterated
the blood supply usually reaches the lungs through the widely patent
ductus, but this is sometimes closed or absent. IMeckel first suggested
that in these cases the dilated bronchial arteries might perform this
function, and this is usually the case. The lungs were supplied from the
left subclavian in Chambers' case, from a dilated ductus arising from the
left subclavian in Ramsbotham's. In a case reported by Voss two large
bronchial arteries passed into each lung, and were accompanied by an
anomalous branch from each coronary. In Koller-Aeby's case the ductus
was absent, and three large vessels, equalling the carotid in size, arose from
the upper thoracic aorta at the site of origin of the bronchial arteries.
The first turned to the right lung, following the bronchi ; the others were
given ofi^ as a common trunk, which divided into a larger branch going to
the right and a smaller to the left lung.
 
Pathogenesis. - Two problems are presented. (1) Is the stenosis of
inflammatory or of developmental origin? (2) What is the relation of the
septal defect so often associated?
 
It must be recognized that a small group of cases occurs in which
the stenosis is strictly limited to the valves and no septal defect is associated and which present appearances identical with those produced by
the chronic valvular disease of postnatal life. Such cases must be supposed to be the result of an endocarditis in later fetal life.
 
In the large majority of cases a defect of the interventricular septum is
associated, indicating that if the stenosis be due to an endocarditis, this
must have occurred before the development of the heart was complete
at the end of the second month of gestation. It is upon these cases
that the discussion really turns. It is evident that if endocarditis can
take place during the later stages of gestation, it may occur earlier as
well. On the other hand, there are many cases in which the presence of
associated defects and the absence of inflammatory action show positively
that arrest of development has been the cause. The view is now generally held that in the past far too great weight has been laid upon the
part which inflammatory processes take in the etiology of the many
forms of pulmonary stenosis which date back to early embryonic life,
before the development of the heart was completed. The theory of
fetal endocarditis as a cause must now be considered to be of but
limited application. The fault lies undoubtedly in most cases in a
primary arrest of development.
 
Reference has been made to the explanation communicated by Keith,,
which is now generally accepted, that in the majority of cases the stenosis j
is primary in the conus, and is the result of an arrest of development'
at a stage when there existed in the heart a fourth primitive chamber,
the bulbus cordis. In accordance with the researches of Greil, he describes three changes as taking place in the evolution of the mammalian
from the primitive heart of the fish and reptilia: (1) The division of the
primitive auricle and ventricle; (2) the submerging of the sinus venosus
in the musculature of the right auricle; and (3) the separation of the
bulbus cordis from the left ventricle and aorta, and its complete incorporation in the right ventricle as the infundibulum of that chamber.
This last change takes place by an upgrowth of the ventricular musculature around the cavity of the bulbus, the musculature of the latter
being replaced by that of the ventricle, in the same way as the musculature
of the auricle replaces a great part of that of the sinus venosus. The
author considers that "the submergence of the bulbus constitutes a critical
phase in the developmental metamorphosis of the heart, and it is during this
time that malformations are apt to occur."
 
Four different types of conus stenosis are distinguished by him, of
which the first is that well-differentiated form in which the conus forms
a separate chamber, being separated from the sinus by a muscular
partition. Peacock, in describing a similar case, compared it to the threeventricled heart of the curtle, and considered that it represented an arrest
of development. Keith explains the condition as being simply an arrest
of development in which the infundibulum and body of the right ventricle
have developed to a normal extent, while a constriction has remained
between them, representing the Dentricular origin of the bulbus or a persistence of the lower bulbar orifice. The other forms of conus stenosis,
in which there is a constriction more or less complete of the whole
infundibulum, he explains as an arrest of development of the bulbus
as a whole, its musculature failing to become submerged in that of the
right ventricle proper.
 
Associated Anomalies. - Grave cardiac defects are frequently associated, especially in pulmonary atresia, and constitute another argument
in favor of a developmental origin. In Ettlinger's case there was a large
defect in the interauricular septum above, with multiple defects of the
interventricular septum, and the pulmonary veins opened into the right
auricle. In Habershon's there was false dextrocardia, tricuspid stenosis^
defect of the interventricular septum, and horseshoe kidney.
 
A fact of much importance is the presence of associated anomalies in
cases of atresia with closed ventricular septum, which might reasonably
be considered to be of inflammatory origin. It seems probable that the
primary condition here was a narrowing of the conus or orifice in an
arrest of development, and that the obliteration was produced by an
endocarditis supervening in later fetal life.
 
Symptoms. - The majority of cases of pulmonary stenosis and atresia
present the classical picture of congenital cyanosis in all its details. So
frequent is the association between the two conditions, that morbus
cceruleus and pulmonary stenosis have been considered almost synonymous terms. The clinical aspects vary to a certain extent with the presence or absence of defects of the interventricular septum, and with the
degree of deformity. In stenosis with closed septum cyanosis is usually
slighter and of later incidence, and the duration of life much longer.
The most typical instances of congenital cyanosis with bluish discoloration of the skin, becoming pronounced on exertion, clubbing of the fingers,
dysnpoea, and cyanotic attacks are seen in the many cases in which
pulmonary stenosis is combined with defect of the septum and rechtslage
of the aorta. Pulmonary atresia differs from a simple stenosis in the
more extreme degree of the cyanosis. These are the cases of true morbus
cceruleiis, in which a constant deep blue, or even purple, discoloration
exists, increasing to black on violent exertion. Here the opposite condition in relation to septal defects is seen. When the septum is closed the
cyanosis is more extreme and the duration of life correspondingly shorter.
Pulmonary stenosis with defect of the septum in which no cyanosis is
present is exceedingly rare, but a few cases are on record.
 
Physical Signs in Pulmonary Stenosis. — These are generally distinctive, but may be obscured by those of the septal defect so often associated. In typical cases, enlargement of cardiac dulness to the right and
above, precordial bulging, epigastric and precordial pulsation indicate an
enlargement of the right heart. Sometimes the cardiac impulse may be
so violent that the head and neck share in the vibration of the chest.
A thrill, localized to the second and third left spaces, or diffuse over the
precordium, is fairly frequent. Its presence seems to depend somewhat
upon the condition of the septa. Rolleston,^ in reporting a case of stenosis
with rechtslage, in which there was no precordial thrill, explains this by
the presence of a large defect of the septum, through which the blood
current passed with ease into the aorta. He says that the evidence in
recorded cases is contradictory upon this point, and suggests a statistical
study of it. A thrill was present in 17 of the 82 "primary" cases analyzed.
In these the condition of the fetal passages was as follows:
 
Cardiac septa. Number analyzed. Number with thrill.
 
F. O. and V. S. closed 7 " 3
 
F. O. patent and V. S. closed .... 11 4
 
F. O. patent, defect V. S 24 7
 
F. 0. closed, defect V. S 40 3
 
That is to say, in more than a third of the cases with closed ventricular
septum a thrill was present, as also in 7 of the 24 in which both foramen
ovale and ventricular septum were opon. But in the 40 cases with
closed foramen ovale and with defect of the septum a thrill was absent
in all but 3; in one of which there was a large patent duct, which was
apparently the cause.
 
These figures are puzzling at first, but interesting on reflection, and are
large enough to be of value as facts. The inference is that a thrill is
frequently present when the interventricular septum is entire, and also
when a defect of that septum coexists with a widely patent foramen
ovale; when the interauricular septum is closed and the interventricular
open a thrill is rare, and when it does occur may perhaps be ascribed to the
associated septal defect. Further statistics are needed.
 
The pulmonary second sound is weak or absent in a certain proportion of cases. Much stress has been laid upon the absence of pulmonary
accentuation as a diagnostic sign of pulmonary stenosis, but in a number
it has been distinctly louder than normal.
 
----
 
^Trans. Path. Soc, London, 1892, xliii, 32.
 
 
A prolonged, harsh, rasping, or blowing systolic murmur heard over the
whole cardia, but chiefl}^ at the base, with its point of maximum intensity
over the upper part of the sternum and the second left space, is present
in the great majority of cases. It is transmitted upward toward the
clavicle, along the course of the pulmonary artery, and over the sternum,
but is faint or inaudible at the apex and to the right of the sternum.
It may be so loud as to be heard over the whole chest. From this type
important variations occur. (1) The murmur may be heard over the
whole cardia, but with maximum intensity at the apex, as in Cassel's
case, a boy aged thirteen years, w^ith pulmonary stenosis and a patent
foramen ovale, but the ventricular septum entire. In a case of Peacock's
there was a loud systolic murmur over the whole heart and along the
sternum, the maximum intensity of which was at the inner side of, and
immediately above, the left nipple. In this instance a septal defect was
associated. (2) In cases in which the septal defect is present the murmur
may be heard over the aortic area and along the carotids. Eisenmenger
mentions this as a diagnostic point for the association of pulmonary
stenosis with a septal defect. In Scheele's^ case, a girl aged fifteen years,
with marked cyanosis, the pulmonary orifice admitted a thin pencil,
the valves were small and shrunken, the conus was reduced to the size of
a pea, and the septum was defective at the base. There was a systolic
murmur along the course of the pulmonary artery and at the left sternoclavicular articulation, which was transmitted far up the carotids and
along both subclavians, and was most marked over the left carotid. It
was heard also at the aortic cartilage. (3) The murmur may be heard
in the back, in the left infrascapular region. This occurred in a number
of the cases in this series, but in all a septal defect was associated, to which
the transmitted murmur was probably due. (4) In a few cases physical
signs are absent. Variot reports a child aged five years, with a large
defect of the septum, and the pulmonary a small thin cord with rudimentary valves, who presented marked cyanosis with clubbing, but
whose heart sounds were clear.
 
Diagnosis. - In the majority of cases the decided localization of murmur
and thrill, the increased cardiac dulness to the right, the absence of pulmonary accentuation, and the presence of the distinctive symptoms of
pronounced congenital cyanosis make a positive diagnosis possible. On
the other hand, the variation in the character of the murmur and of the
pulmonary second sound, and the occasional absence of cyanosis, render
the diagnosis indefinite in a certain proportion of cases. That such
atypical cases occur also makes it very difficult to exclude the possibility
of pulmonary stenosis in the difi^erential diagnosis of other cardiac defects.
The presence of constant and marked cyanosis, the distinctive character
of the murmur, and the fact that it is not usually heard in the back, are
points in favor of stenosis. In patency of the duct, pulmonary accentuation is the rule, in pulmonary stenosis it is the exception.
 
Both the associated defect in the interventricular septum and the
pulmonary stenosis have frequently been diagnosed. The presence of a thrill speaks rather for a closed septum, or for cases in which the foramen
ovale also is patent. In a large number of cases the presence of the two
distinctive murmurs can be easily traced, that due to the pulmonary
obstruction heard best at the base and transmitted beneath the clavicle,
that due to the defect localized at the fourth space, heard also in the
back, both harsh, but of the two, the pulmonary usually of a more
blowing character.
 
----
 
1 Deut. med. Woch., 1888, xl, 294.
 
 
 
 
 
Course. - The duration of life in pulmonary stenosis with closed
interventricular septum is relatively high. Peacock reports a patient
dying at forty-five years, and the lowest age in this series was four years.
The possibility exists in such cases that the stenosis had advanced, or
even originated since birth. In stenosis with septal defect death occurs
earlier, but adult life is also sometimes attained. The maximum age in
this series was twenty years. In pulmonary atresia life is very short.
The patients with closed septum all die within the first few months.
When a defect of the interventricular septum exists these subjects may
live some years. The highest age recorded was thirteen years, in one of
Peacock's cases. The table gives the duration of life in the cases in the
series in which this point is mentioned:
 
 
Many patients who survive until early adult life die, not of the lesion,
but of pulmonary tuberculosis. The frequency of this disease in pulmonary stenosis and its grave prognosis can be no longer disputed.
The cause of the predisposition seems to be: (1) The reduced blood
supply to the lungs produces an anemic condition which favors infection;
(2) the marked cyanosis usually present depresses the general powers of
resistance and tends to destructive tissue metabolism; (3) the subjects
of pulmonary stenosis frequently live to an age when tuberculosis is likely to invade the organism when the nutrition is low. This last point
is illustrated in an interesting way in this series. Among the 18 patients
with closed septum, in whom the duration of life was longer, pulmonary
tuberculosis occurred 7 times. Among the remaining 64 patients with
defect of the interventricular septum (in whom life was shorter) it occurred
in only 9 cases, making an incidence of 16 cases in the 82.
 
Another not infrequent termination is by infection from an acute
endocarditis developing at the seat of the defect. Robinson reports
two instances in patients who both died at the age of twenty years, in
both of whom the conus formed a separate chamber with narrow bulbar
orifice. In the one case there were large vegetations on the conus wall,
in the other these formed a fine fringe around its ventricular orifice,
and coarse outgrowths about the associated defect in the interventricular
septum. Acute endocarditis appears to be especially common in this
form of conus deformity, and among the 19 cases in which the conus
formed a separate chamber, recent vegetations fringing the conus orifice,
on the wall, or on the tricuspid valve were present in 6. In Saundby's
case there was a vegetative arteritis of the pulmonary artery, but none
of the valves.
 
==Dilatation of the Pulmonary Artery ==
 
Dilatation of the pulmonary artery is very common in combination
with certain cardiac anomalies but is rare as an isolated condition. A
few cases are recorded in which it appears to be primary and to originate
in an irregular division of the common arterial trunk. The main vessel
is diffusely enlarged and its branches are tortuous and dilated, but the
heart and lungs are otherwise normal. The artery is usually dilated in
persistence of the fetal passages connecting the two sides of the heart,
especially in patent ductus and defects of the lower part of the interauricular septum, or in widely patent foramen ovale. In the two latter
conditions hypoplasia of the aorta is frequently associated and it is
difficult to say which is the primary condition. The dilated artery may
be atheromatous even in young subjects.
 
In defects of the interventricular septum at the base, the hypoplasia
of the pulmonary artery so often present may give place to a marked
dilatation. This was the case in 9 among the 34 primary defects of
the septum in this series. The clinical manifestations of pulmonary
dilatation are discussed by Abrahams^ with the report of a case in which
this was diagnosed as the primary condition.


==Congenital Aortic Stenosis or Atresia==
==Congenital Aortic Stenosis or Atresia==

Revision as of 16:50, 28 September 2012

Abbott 1915.jpg

CONGENITAL CARDIAC DISEASE

BY


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

MCGILL UNIVEESITY, MONTREAL, CANADA


BEPRINTED 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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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

Table of Contents

  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. Book_-_Congenital_Cardiac_Disease_10|Pulmonary Stenosis and Atresia
  15. Dilatation of the Pulmonary Artery

Congenital Aortic Stenosis or Atresia

Subaortic Stenosis. - This term has been applied to a curious annular thickening of the endocardium of the left ventricle, a few millimeters below the aortic valves, which involves the base of the aortic segment of the mitral valve, and encircles the ventricular wall at this point, and leads in most of the cases, to a localized narrowing of the cavity. The cases recorded are not numerous (seven in all), but the condition, when present, usually leads to serious results, and is therefore important. The thickened ring of tissue is often the seat of a chronic inflammatory process, probably of later incidence, but there can be little doubt that it is itself of congenital origin. Microscopic examination of the ring in a case reported by Moore showed it to be non-inflammatory in character. Keith explains it as an arrest of development, analogous to the conus stenosis of the right ventricle, the bulbus failing to atrophy about the root of the aorta.


1 Jour. Am. Med. Assn., 1913, Ix, 1150.



Endocarditis frequently develops both at the defect and at the aortic valves above it, and may lead to further contraction at these points. Shennan^ and Smart^ report two such cases under the term "double aortic stenosis," and a third is recently recorded by Thursfield and Scott.^ In the last, the aortic orifice was narrowed by a fibrous ring, situated on the interventricular septum, just below the undefended space and extending over the anterior mitral segment, and the aortic valves were thickened and fused; there was a thin line of fibrosis in the otherwfse healthy aorta just above the margin of the valves, and slight coarctation at the isthmus. In Shennan's patient, and in that of Fletcher and Beattie, a thick calcareous ring lay below the thickened and ulcerated valves which were the seat of a malignant endocarditis.

Most of these patients reach adult life, and the clinical significance of the condition lies chiefly in the frequent incidence of acute endocarditis. The picture is that of an acquired aortic stenosis. Slight symptoms may exist from childhood, or no sign of the presence of the defect may be given until an acute endocarditis develops, or failing compensation sets in. The systolic murmur produced by the constriction may be very rough, and audible some distance from the chest wall. A precordial thrill existed in some cases.

Congenital Stenosis and Atresia of the Aortic Orifice. — Aortic stenosis of antenatal origin is not common and the duration of life with it is very short. Two forms may be distinguished; those apparently inflammatory, with the stenosis limited to the valves and the ventricular septum entire, and those apparently due to an arrest in development. Unlike pulmonary stenosis, the inflammatory forms are here the commoner, fetal endocarditis, although rare in the left heart, usually involving the aortic orifice. Theremin collected 17 cases, in only 2 of which was there a defect of the septum. In our series there are 6 cases, 5 of which are inflammatory.

Monckeberg^ reports a case of inflammatory origin in an infant of four days, and refers to 12 cases in the literature. He ascribes the occurrence of the fetal inflammation to probably the fifth month of gestation, for the left heart was very small, and the right side so hypertrophied that the apex of the right ventricle formed a recess-like cavity below the left, a condition typical of a heart in which the right ventricle had carried on the systemic circulation through the ductus arteriosus for a long time. In aortic atresia the left ventricle is aplastic, and when a defect of the septum exists, may even be obliterated. The foramen ovale and ductus, are nearly always widely patent, the latter supplying the systemic circulation.


1 Lancet, 1905, vol. i, 21. ^ /f,^^,^ 1904, vol. u, 1417.

3 Brit. Jour. Child. Dis., 1913, x, 104. * Deut. path. Gesell, 1907, xi,'224.



Cyanosis is usually slight or absent in cases of aortic stenosis, but marked in atresia. Physical signs may be absent, or there may be a loud systolic or double murmur heard over the whole chest. In both conditions the duration of life is very short. In aortic atresia the highest age attained was twenty-seven weeks. Simmons^ described an interesting case in an infant aged sixteen weeks, cyanotic from birth, with widely patent foramen ovale and ductus arteriosus, the left ventricle aplasic, the right ventricle greatly hypertrophied, and the aortic cusps fused to form a cone.

Left-sided Conus Stenosis. - Schmincke^ describes two cases in adults of a peculiar muscular stenosis of the conus of the left ventricle, with healthy aortic valves, and no apparent cause, which he thought must be of congenital origin, due to a primary asymmetry in the formation of the left ventricle.

Anomalies of the Semilunar Cusps

These cusps may be increased or diminished in number and defective, fenestrated, or otherwise malformed. A row of supplementary cusps may exist or they may be the seat of attachment of anomalous bands.

Increase in Number. - Supernumerary cusps sometimes occur in the pulmonary artery and, less frequently in the aorta. A more or less perfectly formed fourth cusp of varying size, but frequently smaller than normal, may be inserted between two of the others. Or the usual number of segments may exist, and the sinus behind one of these be divided by a raphe which runs from the back of the cusp to the aorta, indicating fusion of the additional segment or imperfect division from its fellows. In rare instances five cusps occur. Peacock figures a case of five aortic cusps, and Dilg enumerates from the literature 4 cases, in 2 of which the five cusps were in the aorta, and in the pulmonary artery.

The supernumerary cusps have sometimes been explained as an effort at repair of some inflammatory process of long standing, but when the fourth segment is perfectly formed, or the raphe indicating it shows no sign of thickening (as in a case in the McGill Museum), a true malformation must be concluded, which is usually explained as a formation by excess. As this condition is of congenital origin, the cusps are generally so adapted to each other as to be competent to close the orifice, no insufficiency resulting; they occur usually in a heart free from other malformations, and are of very infrequent occurrence. Their clinical significance is slight, and lies chiefly in their tendency, like all valvular anomalies, to become the seat of endocarditis.


1 Intercol. Jour, of Austral., February 20, 1906.

2 Deutsch. med. Wchnschr., 1907, xxxiii, 2082.


Diminution in Number. - A bicuspid pulmonary valve is not uncommon with other cardiac anomalies, especially transposition and septal defects. A bicuspid aortic valve usually occurs in an otherwise normal heart or associated with coarctation or hypoplasia of the aorta. In some instances both pulmonary and aortic valves may be bicuspid. The anomalous segments may be large, with smooth surfaces, showing no sign of further division, a true reduction in number existing; or one or both may present on the arterial aspect a ridge or raphe imperfectly dividing the sinus behind it into two parts, and indicating either a fusion of two formerly independent segments or a beginning separation of a single cusp into two elements ; in other instances a single membranous ring or diaphragm may exist with two such raphes on its aortic surface, indicating the union with each other of all three cusps. Where such a raphe is absent, the condition is undoubtedly a true malformation, but where this exists, the origin of the bicuspid state of the valve admits of much discussion. Peacock arrived at the conclusion that the majority of cases were congenital, due either to an original malformation or to fusion in a fetal endocarditis.

A series of cases illustrating the same line of thought was published by Osier. ^ As pointing to a fusion originating in fetal life, he enumerates

(1) the presence of a low, sometimes half obliterated, raphe behind one of the cusps; (2) compensatory changes in the fused cusps, so that their free edge becomes equal to or even shorter than the single segment; and (3) the fusion of the coronary or right and left segments of the valve. That the lateral (right and left) segments are the seat of the congenital fusion, the posterior cusp remaining always single, is stated also by B ir ch-Hir s chf eld .

Thickening of cusp and raphe does not prove that the fusion is not of "congenital origin, for endocarditis is likely to supervene on any valvular anomaly. On the other hand, it is admitted that many cases are of postnatal inflammatory origin. Such postnatal fusion may be reasonably deduced when (1) the two sections of the cusp are approximately equal;

(2) when the raphe dividing them has its superior origin on a level with the superior origin of the unaffected cusp (instead of at the lower level, as described by Osier); and (3) when in addition the only thickening observable is in the angle betiveen the fused cusys. This latter point has been called to the attention of the T\Titer by Dr. Adami, who points out that the free part of the cusps, being in constant motion, is but little liable to undergo inflammatory adhesion, and that this will be prone to occur at the point of their insertion into the orifice wall, which is the only portion of their edge that is fixed and relatively motionless. Therefore, inflammation originating at the angle of junction of contiguous cusps may lead to localized obliteration of the prinaary angle, or to fusion of the cusps with a new immovable angle of junction further removed from the aortic wall, a progressive fusion occurring.

When a thin delicate raphe exists behind one of the segments of a bicuspid valve vdthout any trace of thickening. Babes insists that it cannot be ascribed to a fusion in fetal endocarditis, but must be regarded as a true malformation dependent perhaps on incomplete division of an originally single cusp. The semilunar cusps originate from the four distal bulbar endocardial ridges, two of which are divided by the descent of the aortic septum, so that a third segment is placed in each artery. The four cusps of the undivided truncus are sometimes seen in the cases of common arterial trunk.


1 Montreal General Hasp. Reports, 1880. YOL. IV — 26


The results upon the heart and circulation of a reduction in the number of the aortic cusps maj'- be summed up as follows:

1. The segments may approximate and be perfectly competent, no pathological effects ensuing. This is proved by the occasional finding of a bicuspid valve in healthy adults even of advanced age.

2. The gradual bulging of the cusps, their greater length, the free space that sometimes exists between them, perhaps a yielding of the aortic ring, lead frequently to a valvular insufficiency or to a narrowing of the orifice.

3. Endocarditis commonly supervenes, either as the acute, often malignant form, or as a chronic inflammatory process, leading to thickening and deformity of the cusps and to subsequent valvular disease.

4. Atheromatous changes at the base of the aorta have been frequently noted, and in 6 of the 11 cases described by Babes and Deteindre there was an aneurismal bulging of the right posterior wall of the aorta, which formed in 5 instances a definite aneurism, from the rupture of which, in 2 cases, death ensued. This is seen also in two cases in the McGill Museum.

This remarkable combination of an aneurism of the base of the aorta with a bicuspid valve is believed by Babes to be directly connected with the bicuspid character of the aortic cusp below it, and is ascribed by him (a) to an extension of the same thinning or trophic process that led to the anomalous condition of the cusp, (b) to the lack of support given to the aorta at this its weakest point, and (c) to the frequent insufficiency of the cusps and the yielding of the aortic ring.

Miscellaneous Anomalies. — Dilg reports a remarkable case, in a child aged two years, of an endocardial fold divided roughly into two cusps with their convexity toward the ventricle, just below the base of a bicuspid aortic valve, both coronaries being behind one cusp. Banks^ reported a woman, aged thirty-four years, with physical signs of aortic insufficiency and a loud, musical murmur at the base, audible at some distance from the chest, whose heart was hypertrophied and presented a cribriform condition of the aortic valve, and one-quarter inch below it in the left ventricle three other rudimentary cusps. These may be of compensatory, postnatal origin, as in a number of other cases recorded of long-standing aortic insufficiency.

In a case in the McGill Museum, reported by Campbell and Hepburn,^ of pulmonary conus stenosis with septal defect, two well-formed valves are situated 1.8 cm. behind the pulmonary semilunar cusps, at the point which marks the lower bulbar orifice, and the site of the proximal bulbar swelling in the embryo. This is an entirely unique finding and suggests an atavistic reversion to the cusps seen at this point in the dipnoan heart.

In one of Babes' cases of bicuspid aortic valves, a peculiar band, like a papillary muscle of the mitral valve, traversed the sinus of Valsalva. Hektoen^ quotes from the literature several other instances of anomalous cords at the level of the valves, and a case observed by himself of a large defect at the base of one of the segments, all of which he ascribed to defects in the development of the aortic septum.


1 Dublin Hosp. Gazette, 1857, p. 330.

2 Can. Med. Assn. Jour., 1913, iii, 871.



Defective Development of the Semilunar Cusps. - In a few instances of bicuspid valve a gap may be left on the wall of the vessel between the segments where evidently no third cusp has formed. This occurred in two of Deteindre's series. A remarkable instance of such a defect in the pulmonary valve is recorded by Stinzing. Here there are only two pulmonary cusps, and a large free space occupying the position of the third was traversed by two low ridges, evidently its rudiments. The heart was from a woman, aged sixty-four years, presenting signs and symptoms of pulmonary insufficiency, a history of pneumonia eight months before death, and failing compensation since.

Primary Defects of the Auriculoventricular Orifices

Congenital disease of the auriculoventricular valves differs from that of postnatal life chiefly in its infrequency, in the more extreme character of the process, atresia being more common than stenosis, and in the fact that the right side of the heart is usually affected rather than the left. Owing to the rarity of the cases, to the short duration of life, and to the fact that in the infant heart the picture presented is hard to distinguish from that of the more frequent lesions at the arterial ostia, this subject is not of great clinical importance, and its chief interest lies in the contribution which it brings to our information upon the question of the pathogenesis of cardiac defects.

Tricuspid Stenosis. — Although this lesion is not very uncommon in adults the cases which can be proved to have originated in intrauterine life are very rare. Vierordt knew of only three instances, unassociated with disease of the pulmonary valves, in the literature. In combination with pulmonary stenosis or atresia it is more frequent. A good illustration of the latter combination is seen in a specimen in the McGill Museum, presented by Sir William Osier. In the heart of a cyanotic infant aged four months, both pulmonary and tricuspid valves are thickened, shortened, and fused, and their orifices markedly reduced; the ventricular septum is entire, the foramen ovale widely patent, the right auricle hugely dilated, and the tricuspid surmounted by recent vegetations. Such cases are undoubtedly of inflammatory origin, and are of value as proving that fetal endocarditis, although it has been overrated as. a cause, certainly has its place as an etiological factor in congenital cardiac disease.

Tricuspid Atresia. — Although in itself rare, tricuspid atresia is the commonest of all congenital lesions of the auriculoventricular cusps. Rauchfuss collected 16 cases from the literature of which 5 were due to a defect in development, 5 were apparently inflammatory, and the remainder were of "doubtful" origin. Since then additional cases have been reported by Chapotot (quoted by Vierordt), Sieveking, Kiihiie^ (two cases) Bernstein^ and Wieland.^


1 Chicago Path. Soc, 1905.


Pathological Anatomy and Pathogenesis. - Cases of inflammaton' origin must be distinguished from those due to a defect in development, for the latter present several points of special interest. Those of inflammatory origin have usually progressed tlirough a stenosis and show distinct evidence of an antenatal valvuhtis in the form of an extensive cicatricial contraction of the endocardium adjacent to the obliterated tricuspid orifice, and often of the pulmonary valves. The developmental cases, on the other hand, may present no sign of inflammation, but the tricuspid orifice is absent, and either shows no trace of its presence, or this is marked by a shallow groove, the tricuspid segments are lacking, and the right auricle is divided from the right ventricle by a thick muscular septum. Kiihne, and subsequently Wieland, subdivided these developmental cases into a group of (a) "isolated" primary atresias in which certain pathological changes of a secondary nature are constant so that a definite type is set up, and (6) tricuspid atresia complicated by other grave cardiac anomalies of independent origin, such as transposition of the arterial trunks, pulmonary atresia, etc. Eight cases of the "isolated" form that constitutes the first group were separated by Kiihne, and later by Wieland, from the others. To these may be added one by Bernstein from our series. The changes in all of these are practically identical and clearly indicate the sequence of events. There is an entire absence of the tricuspid orifice, and the body of the right ventricle is an aplastic structiu-e, while the left is highly hypertrophied and dilated, appearing at first sight to form the whole heart, with the right chamber as an appendage to it; the right auricle is also hugely dilated and the foramen ovale is widely patent, or a defect of the interventricular septum exists; in addition there is always a defect in the muscular interventricular septum leading from the cavity of the left ventricle into the dilated conus of the right ventricle and thence into the pulmonary artery. The course of the circulation is necessarily from right to left auricle through the foramen ovale, and thence to the left ventricle, from which the blood is distributed in part to the aorta and in part through the septal defect to the pulmonary artery. The aorta is usually dilated, and the pulmonary is normal or somewhat reduced in size owing to the smallness of the chamber from which it springs.

These cases of "isolated primary tricuspid atresia" are of interest from the standpoint of the pathogenesis of ventricular septal defects, for they show that the old mechanical or congestive theory of septal defects in pulmonary stenosis, in which the defect was thought to be secondary to the raised pressure in the right ventricle, which has long been justly discarded in this connection, at least in regard to the developmental forms of pulmonary stenosis, must be accredited here. That is to say, the septal defect in tricuspid atresia is here evidently secondary to the congestion in the left ventricle which forces an outlet in the conus of the pulmonary artery and this allows the circulation to be maintained. Wieland points out and insists on the importance of Kiihne's separation of this group, with its constant secondary complex, on this account.


1 Jahrb. f. Kinderh., 1906, Ixiii, 235.

- New York Path. Soc. Reports, February, 1906.

3 Jahrb. f. Kinderh., 1914, Ixxix, .320.



It should be remembered that cases of developmental tricuspid atresia with or without complicating cardiac anomalie, constitute one well recognized form of the cor biatriatrum triloculare. The subject is discussed under that head.

Etiology. - The causation of the cases of inflammatory origin is that of fetal endocarditis elsewhere. In tricuspid atresia the secondary ventricular septal defect is bound to occur, for the proper maintenance of the circulation as well here as in the developmental forms.

Much interest attaches to the causation of the developmental forms of tricuspid atresia. In the embryo the auricular canal opens at first by a common orifice into the left side of the common ventricle, and later by a process of shifting to the right comes to lie more in the median line. The theory had been advanced that either a lack or an exaggeration of this shifting to the right would lead to a wrong adjustment of the parts, and to a mitral or tricuspid atresia. Again, the auriculo ventricular orifice is divided into the mitral and tricuspid ostia b}^ the growth of endocardial cushions in its centre, and by a union of these with the interauricular and interventricular septa. Should these cushions become deviated to the right or to the left in their formation they may become adherent to the corresponding wall of the common ostium and thus lead to tricuspid or mitral atresia. Rokitansky thus explains his case of mitral atresia. In that by Robertson the tricuspid atresia present was ascribed to a fusion of the endocardial cushions with the malposed auricular secondary septum, in consequence of a persistence of the valvulae venosse which formed a coarse network across the cavity of the right auricle. The suggestion has also been made that a premature obliteration of the ductus arteriosus during early fetal life, might lead to aplasia of the right ventricle and tricuspid orifice by cutting this part of the heart out of the fetal circulation. This possibility is disproved, however, by the fact that in several of the cases this passage is freely patent. The conclusion remains that the probable causation of mitral and tricuspid atresia lies in the mal-position and irregular union of those parts of the cardiac septa dividing the mitral from the tricuspid ostium.

Symptoms and Signs of Tricuspid Stenosis not Atresia. — Cyanosis may be present from birth, or may develop after a few days or weeks. In the classical developmental type described above it is usually extreme in the end, though its onset may be delayed for some time. In Bernstein's case, aged two years and eight months, it did not appear until the sixteenth month, but then became marked with clubbing, and a polycythemia of 10,000,000 developed. This late appearance was possibly explained by the absence in this case of the auricular septum, a condition which must have facilitated the circulation. On the other hand, Ivelley's patient, a delicate, unhealthy child, showed only slight lividity on crying or wdien he had a cold, and in Sieveking's case, dying at nine weeks, cyanosis was absent throughout, but dyspnoea was a prominent symptom.


Dyspnceic attacks are a prominent feature, are often of daily occurrence and are frequently the direct cause of death.

Physical signs are not very characteristic, being obscured by those of the septal defect that in tricuspid atresia is always present, and by the fact that a systolic murmur with maximum intensity over the right ventricle, such as is usually produced in these cases, may with difficulty, be distinguished from one generated at the pulmonary area. The marked hypertrophy of the left combined with the smallness of the right ventricle is of assistance in the differential diagnosis from pulmonary valve disease, although allowance must be made for the increased cardiac area produced by dilatation of the right auricle. This feature was indicated in Wieland's patient by a zone of dulness to the right of the vertebral column behind. His case was characterized also by a strong systolic murmur and precordial thrill of maximum intensity at the apex, both of which were of a curiously intermittent character.

Duration of Life. — -Very few of the developmental cases of tricuspid atresia live more than one year. Bernstein's patient reached two years and eight months, a relatively high age that is perhaps explained by the almost complete absence of the interauricular septum that was present. In the inflammatory cases, in which the atresia has probably progressed through a stenosis, adult life is frequently attained. Such was Bierdach's case dying at twenty-eight years.

Congenital Mitral Stenosis. — This is even rarer than the same lesion at the tricuspid orifice. A typical case evidently due to an antenatal valvulitis, in a child of ten months, with cyanosis and dyspnoea from birth, is reported by Simmons. A curious combination of dwarfism and mitral stenosis in patients who have attained adult life has been observed. L'Abbe reports a case in a woman, aged twenty-seven years, of extremely small stature (1 meter high, 43 kilos weight), puerile intelligence, and marked infantilism. There was a clear history as well as physical evidence of congenital syphilis, and a pure mitral stenosis.

Mitral Atresia. - A complete obliteration of the mitral orifice is still rarer than stenosis. The same remarks apply in regard to etiology as in tricuspid atresia, but here a primarj'^ defect in development may be almost constantly assumed. Grave associated anomalies are also nearly always present, and give additional proof of a teratological origin. In Theremin's observation of an infant aged two days, the left auricle and ventricle were aplastic without any trace in the latter of a mitral orifice, its walls being formed throughout of finely reticulated muscle fibres; the foramen ovale was closed, the interventricular septum defective, the pulmonary valve bicuspid, and the aorta appeared to arise from the right ventricle; there was a horseshoe kidney and double ureter. Lawrence and Nabarro give a similar case of mitral atresia, defect of the septum, aplastic left ventricle, the aorta arising behind the pulmonary artery from the right ventricle, with coarctation of the aorta, transposition of the stomach, absence of spleen and hepatic section of inferior vena cava; anomalies in form of liver and lungs. In Rokitansky's case of aortic and mitral atresia with defect of the septum, patent foramen ovale, and dilated pulmonary artery, in a child aged twelve days, there was an accessory right bronchus.

Congenital Mitral and Tricuspid Insufficiency. - These lesions may result from a primary malformation of the cusps or from secondary deformity in the arrest of development of neighboring structures, as in persistent ostium primum. Or they may be due to thickening and shortening of the valve in a fetal endocarditis; thus Barth and Roger describe a case in which, on auscultation before birth, a long, loud, rough murmur was heard accompanying the heart sounds. The child was stillborn three days later, and the right ventricle was found dilated, the tricuspid orifice enlarged, and its cusps shrunken and insufficient, and evidently the seat of an endocarditis. In the case of Steffen, of a child aged ten and a half months, there were no tricuspid segments, but the valve formed a low ridge which was thickened, reddened, and slightly jagged. The mitral cusps were similarly thickened and reddened, and one of them was reduced likewise to a narrow ridge.

Congenital Mitral Insufficiency. - Steffen's case is the only instance of congenital mitral insufiiciency found in the literature. True congenital tricuspid insufiiciency is also rare, probably there are not a dozen cases.

Anomalies of the Auriculoventricular Cusps

Double Auriculoventricular Orifice. - A second valvular opening supplied with its own cusps, chordae tendinese and papillary muscles, may lie within the segments of an otherwise normal auriculoventricular valve. Seven such cases are recorded, six of double mitral orifice by Greenfield,^ Cohn,^ Degen,^ Stuhlenweisenberg,* and Camisa,^ and one of double tricuspid by Pisenti.*' In Stuhlenweisenberg's case, and in one of Camisa's, the two orifices were of equal size, and were separated by a bridge of valve tissue which supplied a cusp to either opening; in all the other cases the second opening was much smaller, and lay in one of the segments of the primary orifice.

Two hitherto unpublished cases of the latter description, are in the collections of the Harvard and the McGill Medical Museums. In the Harvard case, an opening 2 cm. long lies in the aortic cusp of the main mitral orifice, and leads into an aneurismal pouch formed by the apex of this segment, which communicates with the cavity of the ventricle by numerous fenestrations. The McGill heart (Fig. 37) is of bizarre external form owing to its bifid apex, deep auriculoventricular groove, and hypertrophied right chambers. The inter auricular septum presents a small valvular patent foramen ovale above, and is absent in its lower two-thirds, a large crescentic defect {persisient ostium jprimum) existing. The mitral valve is replaced by a single large segment which is cleft in its anterior portion, passing forward from either side to be inserted into the middle of the base of the interventricular septum where this bounds the interauricular defect below. The secondary mitral ostium lies in the posterior half of this large primary segment, 7 mm. back from its free margin. It is a perfect valvular opening admitting a lead-pencil with two well-formed cusps attached to slender chordae, arising from_ two short papillary muscles which lie behind and independent of the single group from which the chordae of the primary segment spring. The right auriculo ventricular valve is malformed and an irregular excavation in its septal cusp suggests an unsuccessful attempt at a double tricuspid ostium. The aortic valve is bicuspid. The endocardium is healthy.


1 Trans. Path. Soc, 1876, xxvii, 128 (with plate).

2 Inaug. Dissertation, Konigsberg, 1896 (with plate).

3 Inaug. Dissertation, Greifswald, 1903.

4 Centralb. f. Pathol, 1912, xxiii, 1027. _ ^ /^^(^^ p. 342 ^ Di una rarissima Anomalia della tricuspide, Perugia, 1888.





Fig. 37



Heart of a child, aged five years, showing; A, defect of lower part of interauricular septum; B, patent foramen ovale; C, double mitral orifice; D, cleavage of mitral segment. (From a specimen in the Pathological Museum, MoGill University.)


Etiology. - Camisa believed a fetal endocarditis had led to a fusion of segments at their apices and to the formation of secondary orifices. Cohn and Stuhlenweisenberg suggest a malformation by excess, a view supported in the latter's case by the equal size of the two orifices. Pisenti supposed a fenestration of the endocardial cushions, which had transmitted the blood stream in early embryonic life and had become transformed into a second valvular orifice by a natural adaptation of growth, the papillary muscles and chordae growing up to its borders.

In the two specimens seen by us, Pisenti's explanation seems to apply, the marked irregularities in both auriculoventricular valves due to the auricular septal defect in the McGill specunen, and the multiple fenestrations in the Harvard case, alike arguing for such accidental origin at an early embryonic period. Camisa's theory of a fetal endocarditis is not tenable in the case of the McGill specimen and others in which the endocardium is free from every trace of sclerotic change.


Symptoms. - The double orifice is in itself of no clinical significance, the secondary segments functioning as normal valves. In the majority of the cases, including Cohn's patient, who died at seventy-one years, both sets of valves were thin, healthy and competent. Chronic endocarditis had supervened in both Camisa's cases and in Stuhlenweisenberg's. In the latter a loud systolic murmur over the precordium was associated with insufficiency and sclerosis of the segments of the anterior mitral ostium, the posterior remaining free.

Displaced Orifice. - A double mitral orifice is described by Andrewes,^ in which two orifices separated by a fibrous septum lay one behind the other in the left ventricle. The right ventricle was rudimentarj'^, the interventricular septum defective and the tricuspid valve absent. A deflection of the septum to the right so that both orifices are placed in the same ventricle was assumed.

Miscellaneous Anomalies. -” Various minor defects, as irregularly formed or accessory leaflets and anomalous arrangement of the chordae tendinese or papillary muscles occasionally occur, and may in some instances contribute to an insufficiency of the valves.


Primary Patency and Anomalies of the Ductus Arteriosus Botalli

The ductus arteriosus of the fetus is a short, thick trunk, 10 to 15 mm. long, running from the left branch of the pulmonary artery directly after the bifurcation to the under side of the arch of the aorta just beyond the origin of the left subclavian artery, which serves to carry the unaerated blood, returned from the head and upper extremities, to the descending aorta, whence it passes to the placenta. At birth the ductus undergoes a rapid involution, its lumen becomes practically impermeable about the third week of life, the alterations in its wall, which lead to its permanent obliteration, going on for some months, and finally transforming it into the ligamentum^rt^riosum of Jater life. The average diameter of the patent ductus at birth is given by^VTerordt at 5 to 6.8 mm. and by Theremin as 4.8 mm. But when filled with fluid during life, or experimentally injected directly after death, it is found to be much larger. Thus in a series of infant hearts prepared by Klotz, in which he injected the ductus from the aorta with gelatin at autopsy, it was found in the newly born to be fully equal in size to the main pulmonary trunk. He ascribes its apparent smallness as usually seen postmortem to the firm contraction of the muscular wall.

The ductus may (1) remain patent throughout life, (2) undergo aneurismal dilatation, (3) it may be absent, or (4) it may have an anomalous origin or course.



^ Trans. Path. Soc, London, 1903, liv.


Patency of the ductus is not infrequent in combination with other cardiac defects, especially those in which there is some serious interference with the pulmonary circulation. It occurred in 166 of this series of cases, in 21 of which it was combined with pulmonary atresia, in 14 with pulmonary stenosis, and in 23 with transposition of the great trunks. As an isolated condition it is among the more infrequent of cardiac anomalies. The first carefully recorded case of primary patency with autopsy findings, was diagnosed before death and published by Bernutz^ in 1849. Six cases were collected by Almagro^ in 1862, 12 by Gerhardt^ in 1867, 20 by Wrany^ in 1871, and 26 by Vierordt in 1898. Herxheimer enumerated all the above in 38 cases collected in 1910, while Wells,^ in 1908, found 41. A careful anal}' sis of 34 cases with, and 37 without, autopsy report was published by Goodman^ in 1910, and there are important clinical studies by Hochsinger, Gillett, Taylor,^ and Wessler.^ From these and other sources, 64 cases of uncomplicated primary patency with clinical history and autopsy reports, have been analyzed in this series. Of these, 18 are in infants under two years, and 46 in "adults" over this age. As of special interest, or not included by other writers, may be mentioned the cases by Hewitt,^*^ Hall,^^ Kingsley^^ Thompson^^ and Carpenter^* in infants; and Kaulich,^^ Fagge,^^ Darier,^'^ Schrotter,^^ Drasche,^^ Garipuyj^" Crouzet,^^ Greenhow,"^ Gibson,^^ Wells, Schnitzler, and Mead,-^ in adults, as well as 13 adult cases quoted below, in which the patency was complicated by acute infective pulmonary endarteritis.

Pathogenesis. - The causes of persistent patency of the duct are to be sought in the conditions of its normal closure, and this must depend upon the influences, mechanical or otherwise, of the changes in the circulation at birth, and upon the consequent alterations in the vessel wall, itself a fetal structure destined to involution. As possible factors in the process of closure may be enumerated: (1) peculiarities in the histological structure of the ductus wall, (2) alterations in the bloodpressure at birth, (3) modifications at birth in the position of the ductus relative to the aorta and pulmonary artery, and other mechanical factors preventing entrance of blood from the aortic side.

1. Histology. - The ductus wall is poor in elastic tissue as compared with the aorta and pulmonary artery, but is relatively rich in muscular elements, which, as well as the elastic tissue are known to undergo marked increase during the later months of intra-uterine life. More particularly a loose, subintimal layer of muscle is present (Thoma^ ; this evidently corresponds to Jore's musculo-elastic layer of the arterial wall, which is here developed both at an earlier period and to a greater extent than in the aorta and pulmonary artery. It is especially marked at either extremity of the duct where it can be seen to pass into and, indeed, to form, the musculo-elastic layer of the aorta (Klotz). When the canal is emptied of contents, as happens after birth, this increased muscularity enables it to contract firmly, so that its walls remain in juxtaposition and may undergo obliterative endarteritis.


1 Arch. -Gen. de Med., 1849, xx, 415. \These de Paris, 1862, p. 67.

^ Jena. Zeitsch. f. Med. u. Naturwis., 1867, Bd. iii. Westr. Jahrb.f. Padiatr., 1871, Bd. i, p. 1.

5 Am. Jour. Med. Sc, 1908, pp. 136, 381.

6 Univ. of Penn. Med. Bull., 1910, xxiii, 509. ^ Gaz. d. hop., 1910, 83, p. 1419. 8 Guy's Hosp. Gaz., May, 1901, p. 197.

^ Am. Jour. Med. Sc, 1913, cxlv, 543. " Trans. Path. Soc. London, ix, 48.

^^Arch. Middlesex Hosp. Clin., Series XIII, 1913, p. 39.

^ Johns Hopkins Hosp. Bidl., 1911, xxii, 239.

13 Edin. Hosp. Reports, 1900, vi, 57.

" Proc. Roy. Soc. Dis. Ch., 1909, ii, part 1, p. 163.

15 Viert. f. prak. Heilk., 1862, ii, 92.

1^ Guy's Hosp. Reports, 1873, xviii, 22.

" Bull, de la Soc. Anat. de Paris, 1885, x, 55.

^^Zeit.f. klin. Med., 1901, xliii, 161.

" Wien. klin. Woch., 1898, xi, p. 1195.

20 Bull, de la Soc. Anat., February, 1907, p. 179.

^1 Ibid., 1869, xiv, 323. 22 QHn. Soc. Trans., 1876, ix, 152.

^^Edin. Med. Journ., 1900, n. s., viii, 1, 212, 436.

^ Jour. Am. Med. Assn., December 24, 1910.



2. Alterations in the Blood pressure at Birth. - Previous to birth, the pressure is highest in the right side of the heart; the pulmonary arteries are small, and almost all the blood passes through the ductus into the aorta. At birth the lungs are expanded, their capillaries are opened, and there is an immediate lowering of pulmonary blood pressure. Dr. Adami suggests that during the first few days of life the aortic tension, and therefore the mean blood pressure in the body as a whole, is also lowered, owing to the reduced amount of work which the heart is called upon to perform, after the cutting off of the placental circulation, and that this reduction in the mean blood pressure is the cause of the collapse of the ductus, and the main factor in closure. Kirstein also believes that a pressure equilibrium is established between the aortic and pulmonary circulations, which prevents a current through the ductus and thus permits of its obliteration, while Klotz thinks that alterations in the pulmonary pressure at birth, and the relative muscularity of the ductus wall are together sufficient to account for closure.

3. Nevertheless, mechanical conditions preventing the flow of blood through the ductus from the side of the aorta have been adduced by many workers, and may reasonably be supposed to assist in the process of closure, especially when the pressure in the aorta comes to exceed that in the pulmonary circulation. Schantz supposed a stretching of the duct by the movement of the pericardium, pulmonary artery, displaced thoracic organs, and sternum, in the initial respiration, and Strassman described, on the basis of a large number of injection experiments, a fold in the aortic wall at the upper border of the mouth of the duct, which appears about the seventh month, and which he thinks closes its opening in a valvular manner when the pressure rises in the aorta at birth. This theory of a valvular aortic fold has been widely accepted and has received recent confirmation in the experimental work of Fromberg.2 Nevertheless, its constancy in infants, and its valvular action when present, has been gravely disputed by such careful observers as Klotz, Kirstein and Stienon.^ The last word on this subject has come from Stienon, who has found that Strassman's fold is not shown on plaster casts of the ductus and adjacent vessels in the newly born, made under low pressure, and ascribes its appearance postmortem to the falling together of the canal after its evacuation. From the study of a large number of such casts of the aortic isthmus and of patent ductus at various ages, he concludes that the essential mechanical factor in closure is the dilatation of the fetal isthmus, which is produced by raised aortic tension after birth, so that the latter has the secondary effect of favoring closure by pressure of the dilated isthmus on the aortic end of the duct. Dislocation of the thoracic organs in the establishment of respiration probably also assists in diverting the circulation.


1 Virchows Archiv, 1883, vol. xciii, 443.

2 Baumgar. Arbeit, aus. d. Geb. d. Path., 1914, ix, 198.

3 Archiv. de Biol., 1912, xxvii, 801.


From the above considerations the general conclusion may be drawn that continued patency will occur (1) in conditions in which the blood pressure, either in the aorta or pulmonary artery, is maintained at a level approximating that before birth (as in atelectasis of the lungs), or in which, for any other cause, a high positive pressure in the ductus is maintained; (2) when a congenital defect in the structure of the ductus wall exists. That such a defect is not uncommonly the cause of patency is suggested by the frequent association of anomalies elsewhere in the body and by the not uncommon occurrence of a history of syphilis, or of anomalies in other members of the same generation, as in De la Camp's remarkable series of six brothers and sisters all with characteristic physical signs of patent duct.

That raised pulmonary pressure is usually at fault is evidenced by the frequent history of atelectasis of the lungs, diflBculty in suckling or prolonged delivery in the mother, seen in the present series. In support of Stienon's view, that dilatation of the fetal isthmus is an important factor, we may note that persistent patency is associated in most adult cases with a certain degree of coarctation of the aorta, and that the process of closure after birth is, like dilatation of the isthmus, a gradual one, extending over the first weeks of life and often not completed until the third month.

Pathology. - Three principal types of patency may be distinguished: (1) The duct may be greatly shortened upon itself so that its ends are approximated to each other, and it disappears as a canal, remaining as a simple aperture between the two great trunks. (2) More frequently the ductus persists as a short canal from 0.4 to 2 cm., long (Vierordt), with a lumen varying in size from one just admitting a bristle to one allowing the passage of a "goose-quill," "pencil," or even, as in Luys' case, the "finger." A patent ductus of long standing is usually shorter and broader than that of infancy or later fetal life. In form this canal may be (a) cylindrical, as is usual in infants, and as was seen in the cases by Fagge, Almagro, Gerhardt, and White^ in adults; or (b) funnel-shaped (i. e., conical, as in a funnel without a stem), with its larger end toward the aorta, as in a case by Murray, in which in a woman aged thirty-six years, it formed a truncated cone three-eighths of an inch long, just admitting a quill, and lying with its base to the aorta. Finally (following Gerhardt's classification into four types, of which the above forms 1, 2a and h constitute the first three), the patent duct may exist (3) as a canal which has undergone aneurismal dilatation.

In a patent ductus with otherwise normal conditions, the blood stream will be directed chiefly from the aorta, where the blood pressure is


1 Trans. Path. Soc, London, 1885, xxxvi, 182.


PLATE VI


Microscopic appearances of wall of patent ductus and adjacent aorta and pulmonary artery in a case of patent ductus arteriosus with acute infective pulmonary endarteritis, showing site of initial lesion at pulmonary end of ductus. Drs. Hamilton and Abbott. (Colored drawing by Dr. J. H. Atkinson.) (Haem. and eos. and elastic tissue stains. Low magnification.) A rectangular block has been cut to include the whole wall of the ductus (D, D') and a portion of the piilmonary artery (P.A.) and aorta {A) adjacent. A, wall of aorta which is quite healthy. P.A., wall of pulmonary artery. D, D', wall of ductus arteriosus in which the elastic tissue is almost destroyed, and which is surmounted by a thrombotic mass {B) . C, pulmonary end of ductus, showing destruction of elastica, and organization of inflammatory products (z. e., seat of initial lesion). E, aortic end of ductus showing zone of recent inflammatory exudate and invasion of tissue between aorta and pulmonary artery by acute inflammation. F, necrosed area below ductus wall. G, recent acute inflammatory process extending from aortic end of ductus into cellular tissue between aorta and pulmonary artery. H, thrombotic mass overlying wall of pulmonary artery and becoming incorporated with it in neighborhood of pulmonary end of ductus higher, into the pulmonary artery. This is evidenced by the funnel-shaped form with its base toward the aorta, which the canal usually assumes in adults, and by the presence of mycotic vegetations on the adjacent wall of the pulmonary artery in all the cases of acute infective endarteritis in the neighborhood of a patent duct. Wagener's 3 cases, in which the membrane at the pulmonary end bulged into the artery, also indicate this direction of the stream. Dilatation of the pulmonary artery, and hypertrophy and dilatation of the right ventricle, are usual results of patency of long standing. Rauchfuss thought them characteristic of all cases, but exceptions occur. The left ventricle may share in the hypertrophy and the aorta be moderately dilated. In Fagge's case, a woman aged forty-two years, the right ventricle was greatly hypertrophied, being equal to the left in thickness; the right auricle was dilated, and the main pulmonary branches, especially the right, were much dilated. The left ventricle is occasionally hypertrophied in excess of the right. In rare instances, as in Walsham's and Drasche's cases, aged respectively forty-seven and twenty-nine years, the heart may not be hypertrophied at all.

Arteriosclerotic patches are not uncommon in the neighborhood of the patent duct in the aorta, and extensive atheroma may occur also in the pulmonary artery. In Hebb's^ case the atheroma and dilatation of this trunk seem to be explained rather by the obliteration of its left branch through the pressure of the thrombosed duct.

Durno and Brown,^ report a case in a man of thirty-three, of widely patent ductus arteriosus, with extensive atheroma both of the walls of the ductus, and of the greatly dilated pulmonary artery. A small saccular aneurism of the pulmonary close to the ductus had ruptured, forming a dissecting aneurism which in turn burst into the pericardium.

Acute Infective Pulmonary Endarteritis. - Vegetations of a malignant character are not uncommon within a patent duct, about its aortic orifice, and on the adjacent wall of the pulmonary artery. There are 13 such cases in our series; in all, the pulmonary artery adjacent to the ductus was extensively diseased, and in all but one (Hamilton and Abbott^), the heart valves were also involved in a malignant endocarditis. This last case was of especial interest because of the strict localization of the infective process to the ductus and the pulmonary artery adjacent, which showed clearly that the acute inflammatory process had originated in the immediate neighborhood of the defect, a point confirmed by microscopic examination, in which the organization of the inflammatory products proceeding at the pulmonary end of the ductus was clearly seen (see Plate VI), thus demonstrating this to have been in all probability, the earliest initial seat of a process, which had elsewhere and later assumed a fulminating, highly destructive character.

The patient was a girl of nineteen years, who presented a clinical picture of septicemia for some weeks before death, and the characteristic physical signs of patent ductus, without valvular involvement, or cyanosis. Postmortem a huge thrombotic mass of vegetations lay in the lumen of the dilated pulmonary artery blocking the orifice of a large patent ductus, and extending into the left pulmonary artery (see Fig. 38). The aorta was stenosed at the isthmus but was otherwise healthy and the endocardium of the heart was free from every trace of disease. Embolic abscesses in the lungs, the vegetations in the pulmonary artery, and the blood culture during life contained swarms of pneumococci. Both the patent ductus and pulmonary endarteritis were diagnosed during life.


1 Trans. Path. Soc, London, vol. xliv, 45. - Lancet, 1908, i, 1692.

^ Trans. Assoc. Am. Phys., 1914, vol. xxix (gives full bibliography).




Fig. 38


Diagrammatic drawing showing acute vegetative endarteritis of pulmonary artery in the neighborhood of the patent ductus arteriosus, and consequent infarcts of the lung. A probe is seen passed through the patent ductus. (W. F. Hamilton and M. E. Abbott.)

The wall of the aorta opposite the ductus was the seat of the mycotic vegetations in some cases, indicating that the infection had proceeded with the current through the ductus and had impinged here.

Paradoxical Embolism. — In Schmorl's^ case, an embolus passed from a primary thrombus in the left auricle, through a patent ductus arteriosus to the pulmonary artery. In the cases of acute infective pulmonary endarteritis enumerated above, septic infarcts in both systemic and pulmonary circulations, evidently from emboli passing through the patent ductus, were extremely common, occurring even in those cases in which only the tricuspid valve and pulmonary artery were diseased. Hochhaus based a correct diagnosis upon this feature.



Verhandl. d. deutsch. path. Gesellsch., 1909, xiii, 217.


Symptoms and Signs. — Clinical evidence of patency of the ductus is to be sought rather in physical signs than in symptoms, for the latter are often obscure. Nevertheless, their very negative character when taken in combination with the distinctly characteristic physical signs, presents, in the majority of cases, adequate grounds for a correct diagnosis, and this can almost always be made. Careful contributions are now numerous, and a symptom complex has been built up which makes this chapter in congenital defects almost as legible to the clinician as that of any form of acquired cardiac disease. On account of the secondary anatomical changes that are usually induced in a patent ductus of long standing, such as shortening and widening of the duct and dilatation of the pulmonary artery, the picture in infants and early childhood, is somewhat diflferent, and much less distinctive than that in later life. This statement applies especially to the physical signs.

The typical appearance is one of anemia, sometimes profound, which has been described as wax-like. Cyanosis is usually absent; when present it is generally slight and transient, appearing only on exertion, and usually develops late, sometimes as a terminal event. Of the 57 cases in which this point is mentioned, cyanosis was entirely absent in 27, of which 19 were in adults and 8 were in children under two years. Cyanosis was noted as slight in 13 cases, in one of which, Bittorf's (aged eleven years), it was constant, in the others transient, appearing only on crying in Simmons' (aged sixteen weeks), and during anginal attacks in Hale White's case (aged fifty-three years). It was moderate in 4 cases only. In that by Carmichael, dying at three, it came on soon after birth, becoming extreme, with clubbing and a polycythemia of 8,100,000. Coarctation of the aorta was associated with the patent duct, and mitral stenosis with great dilatation of the left auricle was present as well, suggesting a rise of pressure in the pulmonary artery and a possible reversal of flow, venous blood from this vessel entering the aorta through the canal.

Dyspnoeic attacks usually accompanied by transient, but marked cyanosis, are relatively common in infants and are so characteristic of these cases that the name La Cyanose Congenitale Paroxystique has been proposed. Loss of consciousness may occur during the attack and the heart may stop beating, or death may supervene. Three typical cases were reported by Hall in infants, all of whom died during the attack, and others by Sanders, Carmichael, Luys, and Bommer.^ In the latter (aged sixteen weeks) the cyanosis was transient, coming on only during the attacks, which came on especially during feeding, and recurred at last so frequently that the child failed for lack of nourishment; during the attacks the breathing stopped suddenly and deep cyanosis developed, lasting two to four minutes; it passed off entirely as the breath returned, in the interval the color being normal. This is very suggestive of an admixture of venous with arterial blood as the cause of temporary cyanosis, the pressure becoming higher in the pulmonary artery and lower in the aorta during the act of suckling.

In older subjects cardiac seizures of various sorts may replace these suffocative dyspnoeic attacks. Paroxysms of extreme tachycardia (pulse 200), with dyspnoea and bloody expectoration, lasting for some hours, and recurring every few months, are described in a man aged thirty-six years, with dyspnoea and palpitation on exertion for years, but no cyanosis (Bommer). Hale White reports repeated angina-like attacks, in one of which death occurred, in a man aged fifty-three years, with a patent duct the size of the anterior tibial artery, but no hypertrophy of the heart or disease of this or of the aorta.


1 Freiburg Thesis, 1900.



Epistaxis, hematemesis, and hemorrhages from other mucous surfaces are not uncommon (Almagro, Carmichael, Darier, Duroziez). Unless death occurs from some intercurrent condition, as malignant endocarditis or endarteritis, the patients usually die with failing compensation, and dyspnoea is a remarkably constant feature. Sudden death occurred in eleven cases in our series; in six during dyspnoeic attacks, in three, those by Reid, Crouzet and Chessman, without apparent cause, in the case by Mead from rupture of the heart and in that by Durno Brown from rupture of the pulmonary aneurism above described.

Physical Sigm are almost invariably present, and are usually characteristic in older patients. In infants they are practically indistinguishable from those produced by auricular and ventricular septal defects. This is because the patent duct is at first a straight canal, which does not allow of the passage of a large volume of fluid and because in the absence of dilatation of the pulmonary artery there is less sound produced by the impinging of currents in this situation. Among our 64 cases physical signs were absent in only 8 cases. Absence of physical signs in the case of Walsham, quoted by Vierordt in this connection, must be pronounced doubtful, for the specimen came from the dissecting-room with an indefinite note that cyanosis and pericardial murmurs existed. A negative finding in the cases by Luys and Duroziez was also disputed by Almagro.

The distinctive physical signs (which develop as life proceeds), as well as the absence or late appearance of cyanosis, depend, as Gerhardt pointed out, on the fact that a patent duct of long standing usually has a short, wide lumen through which during systole blood flows freely from the aorta into the pulmonary artery, which dilates accordingly and becomes, with the ductus itself, the chief seat of whatever vibration or murmur the abnormal current may produce; the right ventricle behind it usually undergoes hypertrophy and dilatation as well. Gerhardt described as characteristic a visible systolic pulsation in the second left interspace (indicating the forcible closure of the pulmonary valves), an increased area of cardiac dulness, especially to the right, and a narrow zone of dulness 3 to 4 cm. wide (corresponding, he believed, to the dilated pulmonary artery) lying at the base of the heart, along the left sternal border from the third to the second or first rib, and extending a little way over the first piece of the sternum. This "ribbon-shaped" dulness has been noted by many other observers, and has recently been strikingly confirmed in a number of cases in which Gerhardt's dull area, with characteristic murmur or thrill localized over it, has been found by the a--ray to correspond with a pulsating shadow lying above the base of the heart, which was evidently from its size and position the dilated pulmonary artery. In Bittorf's case this shadow was seen, when looked at from the side, to be the size of a walnut and to pulsate a little later than the heart and synchronously with the aorta. In Arnheim's case the a'-rays showed, besides enormous hypertrophy of both sides of the heart, which occupied nearly the whole left thorax, the greatly enlarged shadow of the pulmonary artery placed above the cardiac shadow "like a cap," and numerous tortuous dilated vessels, indicating an extensive collateral circulation and a probable coexisting coarctation of the aorta. In the cases reported by Schrotter, Mead, and Hamilton and Abbott, the .T-ray cap, and Gerhardt's dulness, were found at autopsy to correspond vnth the dilated pulmonary artery.

When cardiac hypertrophy is marked, precordial bulging, diffuse pulsation, and other evidences will be present. An increased area of cardiac dulness, especiallv to the right, while usual, is not invariable, for the left ventricle may b/hypertrophied in excess of the right (Murray's case), or in rare instances there may be no cardiac hypertrophy at all (Drasche's case).

A thrill, usually systolic, but sometimes continuous through the cardiac cycle, is fairly frequent, and was present in 17 of the 64 cases. It may be diffuse over the precordium, but is usually localized to the neighborhood of the second left interspace, in the region described above as Gerhardt's dull area, or at least is of maximum intensity here. Its transmission obliquely upward below the clavicle (along the course of the pulmonary artery) is said to be pathognomonic.

The auscultatory phenomena are the most important and constant. In infants a harsh systolic murmur with more or less of the above localization is the rule, but in adults a loud murmur is nearly always produced, which is characterized by almost all observers as peculiar, and is variously described as harsh, musical, scraping, scratching, humming, churning, rushing, rolling, and only rarely as blowing. Miiller compares it in his case to "rolling thunder," and says that two different listeners likened it independently to the noise made by a train in passing through a tunnel, and Thayer described it in Mead's case as a "machinery murmur." In rhythm several different t>^es may be made out: (1) The murmur is frequently systolic (as in the cases by Murray, Hale White, Simmons, and Bittorf). (2) It may begin with systole, but continue into and through diastole, either as a continuous hum (Chessman's case), or with a systolic rise (Bommer), or with a rhythmic systolic, and diastolic accentuation. Gibson^ describes as pathognomonic a continuous, rushing murmur which "begins distinctly after the first sound, accompanies the latter part of that sound, occupies the first pause, accompanies the second sound (which may be accentuated in the pulmonary area, or doubled), and finally dies away during the long pause." (3) Sometimes, as in Drasche's case, two independent murmurs are heard at the pulmonary area, the loud, peculiar, systolic one, and a low, short, diastolic, indicating a slight regurgitation into the aorta during the pause. (4) More rarely the murmur is diastolic in rhythm, as in Fagge's case, in which a diastolic murmur, musical and of a wavy character, was localized to the pulmonary cartilage. The point of maximum intensity is usually in the second or third left interspace, and it is often heard very loudly in the first left interspace below the clavicle and over the first part of the sternum and in the back to the left of the third and fourth dorsal vertebrae, and in the left suprascapular region. In Franck's case and in one by Gillet, the murmur was only heard posteriorly in this situation, and not in front at all. It is transmitted over the left ventricle, and its systolic element often is audible over the carotids, sometimes more distinctly over the left than over the right (Gerhardt). It may diminish abruptly below the third left costal cartilage. In this series of 64 cases, among the 18 cases in infants, in 8 a systolic, and in 1 a continuous murmur was present. Among the 46 adults, in 19 cases the murmur was systolic, in 9 "double" and in 14 it was the continuous harsh," "rumbling," "rolling," "churning," "humming" murmur usually with systolic accentuation, described by the earliest students of this subject as characteristic, and which Gibson rightly described as pathognomonic.


1 Medical Press and Circular, May 30, 1906. VOL. IV — 27


Franck mentions, as of diagnostic value, an inspiratory accentuation and an expiratory diminution both of the characteristic murmur and of the radial pulse (pulsus paradoxus), which they explain by saying that during respiration the pressure in the thorax is lowered, so that more blood can enter the pulmonary artery then than during expiration, and this will lead to a smaller pulse wave from the aorta, to a larger current through the canal, and a correspondingly louder murmur.

The second pulmonary sound is frequently much accentuated, and this is very important as distinguishing patency of the duct from pulmonary stenosis with somewhat similar localization of murmur or thrill. On the other hand, in some cases it may be weak or even inaudible.

In the cases by Schrotter and Mead paralysis of the left recurrent laryngeal nerve was present, due to pressure upon the nerve by the enlarged patent duct. Schrotter based a correct diagnosis on this feature. The nerve was degenerated on microscopic examination.

The physical signs are very often obscured by those of other lesions, as malignant endocarditis or arteritis, chronic valvular disease, or other cardiac anomalies so commonly associated. The peculiar character of 'the murmur, its more or less prolonged rhythm, its localization, and that of the thrill when present, high up toward the left infraclavicular region, with the results of .r-ray examination, remain, even in these complicated cases, of the first diagnostic value. Patent duct must be diagnosed also from perforation of the aorta and pulmonary artery just above the semilunar valves, whether oif inflammatory or congenital origin. Brocq^ gives a long series of cases of both types.

So-called aneurisms of the ductus Botalli give rise to no physical signs, being of small size and usually occluded by thrombus. They are generally said to be of little clinical significance, but death from rupture of their walls has been reported and embolism from the thrombus within may lead to a fatal result.


1 Rev. de Med., 1886, vi, 786.


So-called Aneurisms of the Ductus Botalli. — This term is used in the literature with a rather irregular application to denote a dilatation in whole or in part of a persistently patent duct. That the cases are not aneurisms in the strict sense is inferred by most writers. Rokitansky uses the qualifying word "so-called." Gruner says that arterial dilatation would often be a better word, as there is usually no change in the vessel wall, and he draws attention to the fact that in the usual beanshaped form the constriction at either end makes the ductus appear larger to the eye than it really is. Klotz has suggested that, as in his injection experiments the duct is seen to be much larger at birth when distended with fluid than when contracted at the autopsy, many of these small, so-called aneurisms, measuring less than 1 cm. in their greatest diameter, are really not even a dilatation, but are a simple distension of a patent duct to its full capacity by the coagulum within. Again, a further confusion exists in that the term is applied more widely by some writers than by others. Nevertheless, the cases recorded form a fairly well-defined group, which, from their rarity and from the fact that the duct is usually occluded by thrombus, are chiefly of pathological interest, although their occasional rupture, and also the risk of embolism from the thrombus within, increase their clinical significance. The first cases reported were by Billard, Thore, and other French writers; Rokitansky followed with his monograph in 1852, and Virchow in 1856; full studies of the literature with original cases are to be found in the theses of Westhoff,^ and Gruner.^

In what may be taken as the classical form (which is that described by Rokitansky) the ductus forms a spherical or ovoid tumor larger at the middle than at either end, but smallest toward the pulmonary artery, with which, as well as with the aorta, it communicates, filled with old or recent thrombus,-and varying in size from a "cherry stone" (Billard) to a "hazel nut" (Thore), or even a walnut (Hebb, Binzer). In Hebb's case,^ in a man aged forty years, an aneurism the size of a small walnut, filled with old clot, lay in the position of the duct, communicating with the aorta by an orifice one-eighth of an inch in diameter, and abutting against the obliterated left pulmonary artery and left bronchus. All the cases recorded are in infants excepting that by Hebb.

In Thoma's* patient, aged twenty-six years, the aorta, from the isthmus downward for about 4 cm., was dilated in the form of a spindle, was lined by atheromatous plaques, and on its right wall opposite the left subclavian artery was a saccular aneurism, in the floor of which lay a small hole representing the lumen of the greatly shortened ductus leading into the pulmonary artery, which was here firmly adherent to the aorta. Microscopic examination showed this aneurism not derived from an expansion of the aortic end of the ductus, but to be a bulging of the aortfc wall, which the writer thought was pulled to the right by the action of the contracted ductus. Rokitansky's 5 cases of funnel-shaped patency were explained by Thoma in the same way, and a special form of "traction aneurism of the infantile aorta" was thus established by him. In Wagener's 3 cases, aged respectively thirty-eight, forty-two, and twenty-three years, the duct formed a distinct canal with a small lumen open on the side of the aorta, where the orifice lay in the floor of a hollow in the wall of this vessel, and was sheltered by a distinct fold of aortic intima projecting downward from above while the pulmonary end was closed in by a thin membrane, which bulged into the pulmonary artery. Mycotic aneurism of the patent duct of the dissecting form has been described by Buhl.


1 Gottingen Diss., 1873 (quoted by Gruner and Voss). - Freiburg Diss., 1904.

^ Trans. Path. Soc, London, 1893, xliv, 45. 4 Virchows Archiv, 1890, Bd. 122, p. 535.


Absence of the Ductus. — Absence of the ductus may occur, and is usually associated with hypoplasia and shortening, sometimes with atresia, of the pulmonary artery. It is explained as due to a primary failure of development of the sixth left branchial arch (which persists as the ductus), the stenosis of the pulmonary being secondary. In these cases a septal defect is present, through which the aerated blood passes from the right heart to the aorta.

Anomalous Course. — Multiple origin is reported by Peacock in a case of pulmonary stenosis, two small trunks arising at the site of the normal ductus and passing, the smaller into the left, the larger (which was cut short) apparently into the right pulmonary artery. In several cases the canal has opened into the left subclavian. In one case, of right aortic arch, the duct entered the descending aorta below the right subclavian and itself gave off the left subclavian artery.

Coarctation of the Aorta

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

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

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

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


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

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

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


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

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

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

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

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

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

Series. Absent. Minor. Grave.

Barie 3 3

Bonnet 13 11 25

New cases 3 12

Total 13 17 40

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

Series. Absent. IMinor. Grave.

Barie 57 19 1

Bonnet 15 11 2

New cases 8 16 8

Total 80 46 11

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

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

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

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

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

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

Fig. 39



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


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


Fig. 40



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

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

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

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

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

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

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


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

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

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

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

Cases.

1 to 5 years 7

5 to 10 " 4

10 to 15 " 4

15 to 20 " 17

20 to 30 " 37

30 to 40 " 26

40 to 50 " 16

50 to 60 " 9

60 to 70 " 9

Over 70 " 1

Adults (exact age not mentioned) 12

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

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

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

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

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


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

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

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

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



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

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

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

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

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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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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