SPECIAL ARTICLE
695
MANIFESTATIONS
.AND
RESULTS
OF
TREATMENT
OF
PATENT
DUCTUS
ARTERIOSUS
IN INFANCY
AND
CHILDHOOD
An
AnaIyss
of
138 Cases
By Rachel Ash, M.D.,* and Doane Fischer, M.D.t
T
HE PURPOSE of this paper is to reviewthe manifestations and results of
treat-ment of children with a proven diagnosis
of patent ductus arteriosus who have
come under observation at the Children’s
Hospital of Philadelphia. The diagnosis
was established by operation in 134 cases
and by necropsy in 4. Children in whom a
patent ductus served as a compensatory
mechanism for a cyanotic lesion or was
associated with coarctation of the aorta
were not included. Many were observed
over a period of years.
ANALYSIS OF CLINICAL RECORDS
Family History
A survey was nmade of the records of 126
children in whom the diagnosis of
uncom-plicated patent ductus was established by normal cardiac findings following closure
of the ductus, or by necropsy. In this
group the lesion occurred 3 times as
fre-quently in girls as in boys. About 50 per cent of the children were the product of a
first pregnancy. Four were twins, the
sib-lings being normal and not identical. The
majority were born of a young mother.
Twelve of the births were premature. A
familial history of miscarriages was noted
in 15 cases (Table I).
Rubella during the first trimester
oc-From the Children’s Hospital of Philadelphia, I)epartnment of Cardiology, and the Department of Pediatrics, University of Pennsylvania.
Presented before the Centennial Medical Convo-cation of the Children’s Hospital of Philadelphia,
June 2-4, 1955.
#{176}ADDRESS: 1740 Bainbridge Street, Philadelphia
46, Pennsylvania.
f Trainee, National Heart Institute.
TABLE I
PATENT Duc’rus ARTERIOSUS
(196 Cases)
Sex: M. SI (23%) F. 95 (76%)
Premature: I (9%) Twins 4 (3%)
First born 46%; second, 24%; third or more, 30%
Age of mother: Below 5 yr., 38%; below 30 yr., 70%
Age of father: Below 25 yr., 18%; below 30 yr., 47%
Miscarriages: 15 (12%)
curred in 7 instances. Other conditions
oc-curning early in pregnancy (viral disease
other than rubella, vaginal bleeding, severe
vomiting, psychic disturbances) brought
the total of cases of prenatal illness of the
mother to 26 (20 per cent).
In 8 instances a cardiovascular anomaly
was present in siblings or other relatives,
including 2 sisters and 2 cousins, each with
patent ductus. A sibling of 1 child had died
with tracheoesophageal fistula. Associated
anomalies in other parts of the body were
found in 22 of the children with patent
ductus.’
Physical Findings
Seventy children (55 per cent) were of
good nutritional statws and asymptomatic.
Obviously poor nutrition was noted in 49
individuals (38 per cent). Eight of these
poorly nourished children were in
conges-tive failure. Cyanosis was observed in 8
cases. In 3, transient cyanosis in the
neo-natal period may perhaps have been of
pulmonary origin. Three infants presented
episodes of cyanosis on crying during the
first months of life. In 2 children cyanosis
was associated with congestive failure.
When normal pressure differences exist
TABLE II
RELATIVE SIZE OF THE HEART AND OF THE DUCTUS PATENT CORRELATED WITh TILE PULSE PRESSURE
(122 Operated Cases)
Heart Size No. of
Cases
Size of Ductus
Average Pulse Pressure (mm. Jig.)
Small
(No. of Cases)
Average Large Not
Known
Small
Moderate
Large
38
43
41
10
4
0
19 4
4 9
10 26
5
6
5
46
54
66
Totals 122 14 53 39 16
shunt of blood away from the aorta into
the pulmonary artery may lead to a fall
in diastolic pressure with resultant
widen-ing of the pulse pnessure. Not all children
with patent ductus, however, show a wide
pulse pressure. Although there are
indivi-dual exceptions, there would seem to be
good correlation between cardiac size and
pulse pressune (Table 2). In 6 children
with massive cardiac enlargement the
aver-age pulse pressure was 73. A pulsating
fontanel was visible in one infant with
wide pulse pressure; a second infant
showed nodding of the head synchronous
with the cardiac pulsations.
A thrill could be felt in 90 individuals
(71 per cent). Twice it was palpable only
in the suprastennal notch. In 12 children,
a supnastennal thrill was associated with a
left parasternal thrill. A thrill to the left of
the sternum could be felt in only a single
interspace, usually the second, in 18
chil-dren; in 2 interspaces in 23 children, and in
the first 3 interspaces in 37 children. In 10
children a coarse systolic thrill was
pal-pable along the entire left side of the
ster-num, sufficiently intense in 1 girl to be felt
through a heavy winter coat. The maximum
intensity of the more diffuse thrills was
frequently at the mid-sternum, rather than
at the second interspace where the greater
number of thrills could be felt best.
The presence of a heart murmur was
recognized during the first year of life in
59 per cent of the children, although it was
frequently not noted during the neonatal
period. In 14 children the lesion was first
noted by the school physician on entrance
to school.
The pathognomonic murmur of
persist-ent patency of the ductus arteriosus is a
continuous murmur situated below the left
clavicle which begins at various intervals
after the first sound and attains its
maxi-mum intensity late in systole and early in
diastole, after which it fades away. This
characteristic murmur was audible in 119
of the 126 children whose physical
find-ings were analyzed. In 10 cases it was a
soft sound, restricted to a single interspace,
usually the second, and heard immediately
to the left of the sternum. In at least 1
child this murmur could be heard only near
the left shoulder. In 24 cases a continuous
murmur was present in 2 interspaces, in 73 it was audible over 3 interspaces, and in
12, over 4 or more. In 14 children the
con-tinuous murmur was maximum over the
mid-sternum. In cases where this murmur
was heard best below the second or even
the third interspace, the ductus was
usu-ally found to be directed medially toward
the main pulmonary artery, rather than
laterally toward the left branch.#{176}
Ex-tremely loud continuous murmurs tended
to be transmitted with diminished
inten-sity below the right clavicle and to the
posterior left chest. In the majority of
cases the systolic component of the
697
tinuous murmur was audible over the ne-maining precordial region and to a varying
extent over the entire chest. This systolic
murmur was frequently transmitted into
the carotid and axillary arteries.
In 24 children a continuous murmur was
heard during the first year of life. A systolic
murmur had been noted prior to the
ap-pearance of the continuous murmur in 12
of these infants. The continuous murmur
was heard as early as 3 months in 3 babies,
and was present below the age of 6 months
in 12. One infant presented no murmur
when first seen at the age of 5 months in
congestive failure. A continuous murmur became audible below the left clavicle
following digitalization. Many children
were first seen at a later age so that the
exact percentage of infants with persistent
patency of the ductus arteniosus who
de-velop a continuous murmur within the first
year cannot be determined from the
pres-ent group. The latest appearance of a
con-tinuous murmur in a child who had pnevi-ously presented a systolic murmur was at
the age of 3% years.
In 5 children a mid-diastolic blow was
present to the right of the apex. These
in-dividuals all had large hearts associated
with a well-marked, continuous murmur,
the systolic component of which was
audi-ble over the entire chest. In all cases the
mid-diastolic blow disappeared after the
closure of the ductus.
Seven children presented only a systolic
murmur. Two had died in infancy of
mili-ary tuberculosis and bronchopneumonia
respectively. Two older girls (8 and 17
years of age) presented a pulmonary
sys-tolic murmur associated with a diastolic
blow to the left of the sternum. In one the
diagnosis was established by cardiac
cathe-terization; the other was subjected to
thonacotomy without further diagnostic studies. In both cases an extremely large
patent ductus and large pulmonary artery
were found at operation. In the remaining
3 children the presence of a patent ductus
was established pneopenatively by
aortog-raphy. Two of these (5 months and 3 years
of age) were in congestive failure. The
third, an infant 7 months of age, showed
extreme malnutrition and delayed
develop-ment.
ELECrROCARDIOCRAMS: One hundred and
fifty-six electrocardiograms, obtained from
117 individuals, were available for study. No
axis deviation was noted in the standard
limb leads of 141 of these tracings; right axis
deviation was present in 8, and left axis
devi-ation in 7. Precordial leads had been
ob-tamed in 136 tracings-CF2 and CF4 in 5
children; CR’, CR3, and CR5 in 65 children,
and unipolar precordial leads together with
augmented extremity leads in 66 children.
Seventy per cent (96) of the tracings with
pnecordial leads were obtained from
chil-dren under the age of 5 years; 25 pen cent
(34) from those between 5 and 9 years of age.
In the sternal precordial leads a high R wave
exceeding the amplitude of the S wave,
which often was of minimal size, was found
in 86 per cent of the children below 1 year of
age, 68 pen cent of those below 3 years,
and 45 per cent of those below 5 years.
Be-tween the age of 5 to 10 years high R waves
indicative of the presence of a large right
ventricle were found in 12 per cent of the
right precordial leads. All of these tracings
showed normal QRS complexes in the left
precordial leads. The relatively few
chil-dren who were over the age of 10 all had
QRS complexes with low R waves
associ-ated with S waves of high amplitude in
leads to the right of the precordium. Seven
per cent of infants below 1 year and 12
per cent of those below 2 years of age
had similar QRS complexes in the sternal
leads, indicating the presence of a degree
of left ventricular enlargement not usually
seen at this age period (Table III; Figs. 1
and 2).
There were only 7 children who had an
amplitude of S greater than that of R in
the leads from the left side of the
precor-dium such as may be seen in association with
right ventricular preponderance (Table
III). None of these tracings showed other
evidence to indicate the presence of an
enlarged right ventricle. The standard limb
leads showed no axis deviation; the
TABLE III
Age No. of Cases
R>S Equip/i. S>R R> S
Left Precordiol Leads
Equip/i. S > R
No. % No. % No. % No. % No. % No. %
7 0 0
4 2 9
3 7 C) 0
1 3 3 9
0 0 1 17
Below 1 yr. 1- 2 yr. 3- 4 yr. 5-lOyr. 10-14 yr. 27 t3 46 34 6 23 11 9 4 0 86.0 48.0 19.5 12.0 0 2 4 9 3 0 7.0 17.0 19.5 9.0 0 2 8 28 27 6 7 35 61 79 100 25 20 43 30 5 93 87 93 88 83
Totals 136 47 35.0 18 13.0 71 52 123 90 7 5 6 4
698 ASH TREATMENT OF PATENT DUCTUS ARTERIOSUS
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FIG. 1. Relation of R to S in leads from the right
side of the precordium in electrocardiograms from
infants and children with patent ductus arteriosus. See Table III for numerical composition of group.
R in the sternal leads; the aVR lead was negative and the aVF lead positive. It
would seem, therefore, that though an
oc-casional electrocardiogram from a child
with an uncomplicated patent ductus will
show right axis deviation in the limb leads,
and those of many infants and younger
children will show R waves of high
ampli-tude in the sternal leads, changes
associ-ated with predominance of the right
ventri-cle (right axis deviation in limb leads; high
amplitude R, small S in Vi and aVR; high
a,
b
Fic. 2. a. Normal electrocardiogram from a
7-year-old girl with patent ductus arteriosus present-ing features characteristic of a normal heart with left ventricular doniinance; N.A.D. in stln(lar(l leads;
negative QRS in aVR; positive QRS in aVF; S of greater amplitude than R in V1; high R, small QS in \75
b. Normal electrocardiogram from a 3-year-old boy with patent ductus arterioshls. Although R is
high in Vi, all other findings are similar to those noted in A. Heart of normal size; ductus small in both children.
amplitude 5, small R in V5) rarely, if ever,
occur in the electrocardiogram of a child
with uncomplicated patent ductus.
Delayed ventricular activation in Vi
sug-gesting right bundle branch block was
ELECTROCARDIOGRAMS
Relative Size of R and S Waves in Precordial Leads
noted in 4 children.
Prolongation of the PR interval was
pres-ent in the electrocardiograms of 3 children;
broad notched P waves in those of 5
chil-dren; notched, diphasic or inverted T
waves in leads 1 and/or 2 in the
electrocar-diograms from 12 children; notched R in
standard leads in those from 3 children.
ROENTGENOCRAMS : Films of the chest in
124 children with uncomplicated patent
ductus showed the heart to be within
nor-riml limits of size in 32 per cent, slightly or
moderately enlarged in 36 per cent, and
greatly enlarged in 32 per cent. In 6 of the
latter group the enlargement was massive,
involving all chambers. The degree of
car-diac enlargement tended to vary directly
with the size of the ductus (Table II). When
the size of the ductus was recorded at
operation, only 12 per cent of those with
small hearts were reported to have a large
ductus, as compared with 72 per cent of
those with large hearts. No small ductus
was found in a child with marked
candio-megaly.
A detailed study of the noentgenograms
of the chest in 113 children showed
in-creased prominence of the vascular
shadows at the hilum in 61 per cent: 18
per cent of those with normal sized hearts;
60 per cent of those with moderate cardiac
enlargement, and 98 per cent of those with
marked cardiac enlargement. The
pulmo-nary segment was enlarged in 76 per cent:
30 per cent of the small hearts; 92 per cent
of the moderately enlarged hearts, and 100
per cent of the greatly enlarged hearts.
Enlargement of the left atrium was noted
in 20 per cent of the group; being present
in 10 per cent of the moderately large
hearts, and 50 per cent of the greatly
en-larged hearts. The left ventricle was
in-volved in all enlarged hearts; the earliest
evidence of enlargement of the left
yen-tnicle usually being noted at the apex in the
antero-posterior view, and at the lower
posterior border in the left anterior oblique
view. Only in the greatly enlarged hearts
did the right ventricle and right atrium
seem involved.
DEATHS DUE TO MEDICAL CAUSES: Four
children in whom an uncomplicated
duc-tus was found at necropsy died before
sun-gical treatment was available. One, a
14-year-old girl, died of congestive failure of
sudden onset. The cause of the failure
ne-mained a mystery as no infectious process
seemed to be present. Two children died
of pneumonia at the ages of 11 months and
3 years, having developed congestive
fail-ure during the terminal illness. One
3-month-old infant died of miliary
tuberculo-sis; the patent ductus probably did not
contribute to the death of this child. A
systolic thrill and murmur had been noted
during life.
There were 2 additional deaths in
chil-dren who presented the continuous
mur-mur of a patent ductus but no necropsy
was obtained: One died at the age of 10
years of bacterial endocarditis; the other at
the age of 3 years of pneumonitis
associ-ated with generalized convulsions.
OPERATIONS: Of 134 operations in this
group, 116 were performed at the
Chil-dren’s Hospital of Philadelphia by Dr.
Jul-ian Johnson or his associates. There were
no deaths. The age range of these 116
children was 4 months to 14 years, the
greatest number being between 3 and 6
years. Sixteen were infants under 1 year
and 21 below 2 years. Seven children were
in congestive failure, 3 being infants 5
months of age.
Ligation with 3 silk ligatures was used
routinely except in children in whom the
ductus was short and wide, or seemed to
exhibit sclerotic changes. It was deemed
necessary to sever the ductus in only 8
children. In all cases the thrill and
continu-ous murmur due to the ductus disappeared
immediately following operation.
Course after operation: Eighty-eight
children were observed for periods ranging
from 1 to 8 years (average 3% years)
follow-ing operation. An additional 25 children
were seen within the first postoperative
year, a month or more following
opera-tion. In all of these individuals the ductus
FIG. 3. Roentgenograms from 3-year-old girl in congestive failure who had only a systohc murmur
below the left clavicle. Diagnosis of patent ductus made by aortogram. (Above) Films taken before liga-tion of the ductus. (Below) Note marked decrease in size of heart 4 months after operation.
700 TREATMENT OF PATENT DUCTUS ARTERIOSUS
The immediate postoperative period
fol-lowing closure of the ductus was
remark-ably uneventful in the majority of cases.
The younger the child, the less seemed to
be the discomfort. Infants were very little
disturbed by the procedure. The chest was
closed without drainage in 110 children.
Pleural effusion in significant amount was
observed roentgenographically in 8
chil-dren; in only 4 was thoracentesis necessary.
A hypertensive reaction occurred in the
immediate postoperative period in 20 per
cent of the children, an interesting
phenom-enon, but of no apparent clinical
impor-tance as it subsided without treatment in all
cases.
Disappearance of congestive failure and
decrease in cardiac size may occur
promptly following closure of the ductus.
A dramatic example was a 3-year-old girl
weighing 18 pounds who was in extreme
congestive failure, with marked
hepatome-galy and splenomegaly, at the time of
opera-lion. Three hours after operation it was no
longer possible to feel the liver or spleen.
Remarkable decrease in the size of the
heart had occurred when she was seen 4
months later (Fig. 3).
A review of the roentgenograms of the
chest in 66 children, obtained from 10 to 18
months postoperatively, showed the heart
to be of normal size in 74 per cent as
com-pared with 35 per cent considered normal
in size preoperatively (Table IV). Only a
moderate degree of cardiac enlargement
was present in those whose hearts were
considered not to be of normal size. Five
TABLE IV
ROENTGENOGRAMS OF TIlE CIILST
(Preoperative and 10 to 18 nwn!/zs postoperative
in 66 cases)
No cardiac enlargement
Slight to moderate
car-dine enlargement
Marked cardiac
enlarge-ment
Enlarged left atrium
Increased hilar
vascu-larity
Enlarged pulmonary
seg-ment
23 35 49 74
9,3 35 17 26
20 30 None
10 16 None
40 61 20 30 45 71 32 50
SPECIAL ARTICLE 701
Preoperative Postoperative
No.
(‘ases
No. (‘ases #{176}
prior to operation showed a decrease in
cardiac size following operation. In only 2
children who seemed to show cardiac
en-largement preopenatively did the heart not
seem smaller postoperatively. In both
chil-dren the degree of enlargement was not
great. Decrease in size of the pulmonary
segment was not so marked as that of the
heart itself. Presumably, the changes in
the myocardium are more readily reversible
than those in the pulmonary artery. The
rapid decrease in size of the heart would
suggest that cardiac enlargement in
associa-tion with a patent ductus, at least in
child-hood, is due predominantly to dilatation.
ASSOCIATED CiiDIovAscui LESIONS: In 2 children the diagnosis of associated sub-aortic stenosis made preoperatively was verified by the persistence of a systolic thrill
and murmur below the right clavicle
fol-lowing closure of the ductus. In a
2-year-old boy a residual systolic murmur
associ-ated with a thrill in the lower sternal
re-gion was presumably due to a defect in the
ventricular septum. A left superior vena
cava was noted in 1 patient at the time of
operation, and an anomalous vessel of the
arch, presumably the right subclavian
ar-tery, in another.
There were 12 individuals in whom a
thrill could be felt by the surgeon over the
pulmonary artery or base of the heart
fol-lowing closure of the ductus, although the
thrill over the ductus itself had
disap-peared. In 3 of these children no evidence
of an associated lesion was noted
postop-eratively. One child presented a high
pitched, apical systolic blow which
disap-peared within a few months. In the
remain-ing 8 children a diffuse, low pitched
sys-tolic murmur was heard postoperatively,
maximum in the pulmonary region and
audible posteriorly. In 1 of these children
the ductus had been severed. The murmur
resembled that heard not infrequently in
association with a defect of the atnial
sep-tum; thought to result from the turbulence
of flow within the dilated pulmonary
an-tery. At the end of 1 year postoperatively
the murmur was restricted to the
pulmo-nary area in 2 children. In 1 girl it could still be heard posteriorly 4 years postoperatively,
but was audible only in the pulmonary area
when she returned 7 years after operation.
In 3 children, including the boy whose
due-tus had been severed, a systolic murmur
was still audible posteriorly 3, 4 and 7 years
following operation. In all but one of these
6 children the previously enlarged heart
had returned to normal size. Two children
have not been seen following discharge
from the hospital. It is possible that the
children with persistence of a systolic
mur-mun posteriorly possess a defect of the atnial
septum. This may certainly be the case in
the child who continues to show cardiac
enlargement. It is probable that in the
children in whom no abnormal murmur
was noted postoperatively, on in whom the
murmur regressed, no lesion other than
the patent ductus existed. In such cases the
thrill felt over the pulmonary artery
by
the surgeon following successful closure of theductus was probably due to eddies of blood
within the enlarged pulmonary artery
as-sociated with the ductus. A low-pitched
systolic murmur restricted to the
pulmo-nary area can be heard in many children for
some time following closure of a patent
due-tus, and presumably is due to persistent
702
DISCUSSION
The nature of the murmur heard in
as-sociation with a patent ductus depends on
the relative pressure in the aorta and
pul-monary artery. A continuous murmur is
heard only when the pressure in the aorta
is higher than the pressure in the
pulmo-nary artery, both in systole and diastole. If
such a murmur is heard in a noncyanotic
child who presents a normal
electnocandio-gram, no other confirmatory findings are
necessary before referral for surgery. In the
presence of pulmonary hypertension
shunt-ing of blood may occur only during systole,
and only a systolic murmur may be heard.
In such a case, the diagnosis must be
estab-lished by cardiac catheterization or
aontog-naphy. In rare cases no murmur can be
heard, presumably because of balanced
pressure in the 2 great vessels.’ It is not
uncommon for a murmur to be inaudible
in the first weeks or months of life in an
infant who subsequently presents a
con-tinuous murmur sometimes of sudden
ap-peanance, more often preceded by a
sys-tolic murmur. Later in life the continuous
murmur may disappear with the
appear-ance of pulmonary hypertension or during
congestive failure. This was well illustrated
in a girl in this series who had presented a
continuous murmur for years. At the age
of 14 she died of right ventricular failure.
During the early stage of this illness only a systolic murmur was audible; in the final
48 hours no murmur could be heard.
The electrocardiogram in uncomplicated
patent ductus should be within normal
limits or show evidence of left ventricular
enlargement. In infants and small children
the normal electrocardiogram shows an R
wave of high amplitude in leads from
the right side of the precordium. Other
findings indicative of the presence of right
ventricular preponderance, however, are
not present. If a tracing obtained from a
child with a continuous murmur shows the
characteristic features of right ventricular
preponderance, careful study should be
un-dertakeri to make certain that the ductus is
not compensatory for some such lesion as
tetralogy of Fallot or truncus arteniosus.
Electrocardiographic evidence of night
tricular preponderance may occur in
as-sociation with a patent ductus when a high
degree of pulmonary hypertension has
do-veloped, due to occlusive changes within
the pulmonary vessels. In such cases only a
systolic murmur may be present, or no
mur-mur may be audible. The shunt of blood
may be both from left to night and right to
left through the ductus. Ultimately the
blood will pass solely from the pulmonary
artery to the ductus. The closure of a patent
ductus in the presence of pulmonary
hy-pertension is less hazardous in the child
than in the adult, because changes in the
pulmonary vessels are more readily
revers-ible in the child.
There is little question as to the value of
closure of a patent ductus even in the child
with minimal findings. Hearts which seem
to be within normal limits of size prior to
operation may show a decrease in size
sub-sequently, as evidence of the fact that they
were actually enlarged. Children who have
seemed in normal health may show an
un-expected spurt in growth and weight
fol-lowing operation. Not uncommonly there is
a change in personality from an irritable,
introverted youngster to a pleasant child
who enters cooperatively into the activities
of playmates. Closure of a patent ductus
should be recommended as a prophylactic
procedure in all children who present this
lesion. Provided a clinic accustomed to the
use of intnatracheal anesthesia in infancy is
available, the operation should be
per-formed in infancy whenever the diagnosis
can be made, even in the first year of life.
The possibility of spontaneous closure of
the ductus in a child with a continuous
murmur is too slight to warrant
considera-tion. If the operation cannot be performed
in infancy, it should be deferred to the age
of approximately 4 years, when the child
should have passed the period of
negativis-tic behavior and can undergo the operative
procedure without undue psychologic
703
SUMMARY
A review is presented based on 138
chil-dren with a proven diagnosis of patent
due-tus arteriosus, including an analysis of the
physical, electrocardiographic and
roent-genographic findings. Operations on 116 of
these children were performed with no
deaths. Simple ligation with silk ligatures
was used in 110 cases. There has been no
instance of re-canalization. The criteria for
diagnosis and selection for surgical
correc-tion are discussed.
REFERENCES
1. Anderson, R. C.: Causative factors underly-ing congenital heart malformations. 1. Patent diictus arteriosus. PEDIATRICS, 14: 143, 1954.
2. Shepherd,
J.
T., Weidman, W. H., Burke,E.C., and Wood, E. H.: Hemodynamics
in patent ductus arteriosus without a
