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


BY
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See the links below for the current notes pages.
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{{Heart Abnormal}}
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[[File:Abbott_1915.jpg|thumb|300px]]
=Congenital Cardiac Disease=
 
By




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


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




BEPRINTED FROM
Reprinted From


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


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


{{Historic Disclaimer}}


CHAPTER X.  CONGENITAL CARDIAC DISEASE.


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


By MAUDE E. ABBOTT, M.D.


Definition. - Congenital cardiac disease may be defined as that condition  
Definition. - Congenital cardiac disease may be defined as that condition  
in which, through arrest of development or disease occurring in intra-uterine life, anomalies in the anatomical structure of the heart or great  
in which, through arrest of development or disease occurring in intra-uterine life, anomalies in the anatomical structure of the heart or great  
vessels exist, leading to irregularities in the circulation. It is frequently  
vessels exist, leading to irregularities in the circulation. It is frequently  
associated with congenital cyanosis and clubbing of the fingers, and constitutes in extreme cases the morbus ccBndeus of the older writers.  
associated with congenital cyanosis and clubbing of the fingers, and constitutes in extreme cases the ''morbus cordis'' of the older writers.  




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


==Table of Contents==
# [[Book_-_Congenital_Cardiac_Disease_1|The Development of the Heart]]
# [[Book_-_Congenital_Cardiac_Disease_1|The Development of the Heart]]
# [[Book_-_Congenital_Cardiac_Disease_2|Literature]]
# [[Book_-_Congenital_Cardiac_Disease_2|Literature]]
Line 52: Line 67:
# [[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|Pulmonary Stenosis and Atresia]]
# [[Book_-_Congenital_Cardiac_Disease_10|Pulmonary Stenosis and Atresia]]
# [[Book_-_Congenital_Cardiac_Disease_10|Dilatation of the Pulmonary Artery]]
# [[Book_-_Congenital_Cardiac_Disease_10#Dilatation of the Pulmonary Artery|Dilatation of the Pulmonary Artery]]
 
# [[Book_-_Congenital_Cardiac_Disease_11|Congenital Aortic Stenosis or Atresia]]
==Congenital Aortic Stenosis or Atresia==
# [[Book_-_Congenital_Cardiac_Disease_12|Primary Patency and Anomalies of the Ductus Arteriosus Botalli]]
 
# [[Book_-_Congenital_Cardiac_Disease_13|Coarctation of the Aorta]]
 
# [[Book_-_Congenital_Cardiac_Disease_14|Hypoplasia of the Aorta and its Branches]]
Subaortic Stenosis. - This term has been applied to a curious annular
# [[Book_-_Congenital_Cardiac_Disease_15|Diagnosis, Prognosis, and Treatment of Congenital Cardiac Diseases]]
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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This historic 1915 paper by Abbott describes abnormal human heart development.


See the links below for the current notes pages.

Heart Abnormal: Tutorial Abnormalities | atrial septal defects | double outlet right ventricle | hypoplastic left heart | patent ductus arteriosus‎ | transposition of the great vessels | Tetralogy of Fallot | ventricular septal defects | coarctation of the aorta | Category ASD | Category PDA | Category ToF | Category VSD | ICD10 - Cardiovascular | ICD11


Anderson RH. Teratogenecity in the setting of cardiac development and maldevelopment. (2016)

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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)
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Congenital Cardiac Disease

By


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

Mcgill University, Montreal, Canada


Reprinted From

Osler & Mccrae's Modern Medicine

Vol. IV, 2d Edition, 1915


Lea & Febiger

Philadelphia and New York


CHAPTER X. CONGENITAL CARDIAC DISEASE.

By MAUDE E. ABBOTT, M.D.


Definition. - Congenital cardiac disease may be defined as that condition in which, through arrest of development or disease occurring in intra-uterine life, anomalies in the anatomical structure of the heart or great vessels exist, leading to irregularities in the circulation. It is frequently associated with congenital cyanosis and clubbing of the fingers, and constitutes in extreme cases the morbus cordis of the older writers.


Table of Contents

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



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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)


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

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


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Cite this page: Hill, M.A. (2024, March 28) Embryology Book - Congenital Cardiac Disease (1915). Retrieved from https://embryology.med.unsw.edu.au/embryology/index.php/Book_-_Congenital_Cardiac_Disease_(1915)

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