PRESENT
PROBLEMS
PERTAINING
TO
PATENCY
OF
THE
DUCTUS
ARTERIOSUS
I. Persistence
of
Growth
Retardation
After
Successful
Surgery
By Mary Allen Engle, M.D., George R. Holswade, M.D., Henry P. Goldberg, M.D., and Frank Glenn, M.D.
Departments of Pediatrics and Surgery, The New York Hospital-Cornell University Medical Center
(Submitted June 24, accepted August 8, 1957.)
This work was supported in part by grants from the United States Public Health Service, The New
York Heart Association, and the Walter Brooks Foundation.
ADDRESS: (M.A.E.) 525 East 88th Street, New York 21, New York.
70
Pxiwnucs, January 1958
T
HE GOALS of surgical correction ofpat-ent ductus arteriosus in the pediatric age group are: relief of present symptoms,
prevention of future difficulties, and restor-ation to normal of the cardiovascular sys-tern and the patient.
The success with which the first of these
goals has been achieved is evident from many reports of the results of ligation or di-vision of the abnormal channel. Infants in heart failure have been made well. Chil-dren unable to keep up with their
play-mates because of easy fatigue or breathless-ness have become normally vigorous and sturdy. Even for those considered asymp-tomatic at the time of surgery there is often the realization in retrospect that the child had indeed been below par in energy and
activities.
The second goal has also been realized. The risk of subacute bacterial endarteritis
or endocarditis in the years following
suc-cessful obliteration of the ductus is
mini-mal. In the combined experience of several large clinics in various parts of the world, there was no instance reported of this in-fection in a patient where the ductus was completely closed.’ The danger of heart
failure, formerly a leading cause of death in these patients, has also been abolished by eliminating the cardiac burden of an
arteriovenous fistula or of obstructive
pul-monary vascular disease.
The third goal, the restoration to normal of the cardiovascular system and the
pa-tient, has been fully achieved for many
children but unfortunately for some others,
only in part. The return to normal of the
cardiovascular system is complete
follow-ing successful surgery on the uncomplicated ductus. This effect is readily apparent from
the disappearance of the continuous
mur-mur, narrowing of the pulse pressure, re-turn to normal of the heart size and pul-monary vascularity and the electrocardio-gram. Physiologic studies have documented
reduction in previously increased cardiac
output, abolition of the arteriovenous shunt,
.and return to normal of the pressures in the greater and lesser circulations.
It is the failure to achieve in some
in-stances the second part of this goal-restora-tion to normal of the patient-that has prompted this report. Reference is made to
the extreme retardation in growth, both
weight and height, that has persisted after
successful surgery in many children. Almost
all of the children operated upon at the
New York Hospital, who were so affected
prior to surgery, have continued to be
re-tarded in growth. This group includes
nearly half of the children operated upon
in this clinic for patent ductus arteriosus.
PATIENT MATERIAL
Fifty-two patients between the ages of 3 and
14 years had ligation or division of a patent
ductus arteriosus 1 or more years before the
present analysis of the results; 22 exhibited
marked growth retardation in both height and
weight at the time of operation. Plotted on the
Iowa growth charts, the height was below the
line for the “mean minus 1 S.D.” and the
weight was at or below the 16th percentile
(Fig. 1). An additional child was equally
U)
I.
x
FIG. 1. Weight and height at operation of boys and girls with patent ductus arteriosus and retardation of
growth. Plotted on Iowa growth charts, the open circles indicate weight and closed circles indicate height
in this figure and subsequent ones.
2 3 4
AGE IN YEARS AGE IN YEARS
ARTICLES
RESULTS OF
ANALYSIS
In the postoperative periods of 1 to 10
years (average 4 years of observation
)
onlytwo of the children with retarded growth preoperatively reached the average height
for their age and sex. One child, who was retarded only in weight at the time of
op-eration, age 43 years, reached average weight and above average height within 1
year after surgery. Her growth has
pro-ceeded at this level to 10 years of age (Fig.
2). The other child, retarded in both areas
at age 41%2 years, advanced to average height and weight in 6 s#{241}onths (Fig. 3).
The remaining 21 children continue to be
retarded, at the 16th percentile or below, in
weight, and with one exception, equally re-tarded in height. This exception was the youngest child operated upon in this group,
at 3%2 years. Within 6 months she
be-came average in height and gained slightly
to reach the 16th percentile in weight. Her growth to 6 years of age has not advanced
beyond this level (Fig. 4).
Five of these twenty-one children who
remained retarded did demonstrate some improvement in growth within 1 to 2 years
postoperatively but did not pass the 16th percentile line in weight nor the line for “mean minus 1 S.D.” in height within 4 to
7 years of observation (Figs. 5 and 6). The
other 16 patients failed to improve in
growth.
One other patient in this age range
showed equally extreme growth retardation
at the time of operation but was excluded from this analysis because the operation
was not completely successful; there was
evidence of a persistent aortico-pulmonary
shunt, albeit smaller than the original one.
As to the other children operated upon
but who did not present retardation in growth: 10 were above average in height and weight; 13 were average; and 6 were
slightly below par. Within 6 months to a
year postoperatively, 17 of the 29
120
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90
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2 3 4 5
AGE IN YEARS
FIG. 2. Growth chart of a child retarded in weight only at time of
who subsequently achieved average weight and height.
I 6
operation, and
along the normal or near-normal pattern ex-hibited preoperatively.
COMMENT
It is the group that was retarded in growth at the time of operation, and
re-mained so, to which attention is directed.
The reason for physical underdevelopment
in the unoperated patient seems
attribut-able to the patent ductus arteriosus;
di-rectly, to the large shunt of blood from
systemic to pulmonary circulation and,
in-directly, to the frequent respiratory
infec-tions and impaired appetites which
some-times accompany such an arteriovenous
shunt.
Stunting of growth has previously been
noted among patients with large
left-to-right shunts, irrespective of the site of the
shunt whether auricular, ventricular, or
aorticopulmonary.2 Among children with
(I)
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120
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105
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95
90
85
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75
70
I.-65
0./
x:
55
50
45
FIG. 3. Chart of the only child in the series who had preoperative growth
retarda-tion, in both height and weight, and who subsequently reached the norm.
I 2 3
4
5
6
AGE IN YEARS
ARTICLES
Gibson,1 and Gross5 have commented on
retardation of growth. Lynxwiler and
Wells,6 in their review of 101 children with
this malformation, found that 50 patients
were 10% or more below the minimal
stand-ard for height and weight for the age and sex; 15 of these were more than 20% below. Ash found that a little more than half (55%)
of 138 children with patent ductus
arterio-sus were of good nutritional status, but 38%
showed “obviously poor nutrition.” Adams,
who studied the postoperative growth of 53
children with this lesion, remarked that
there was more growth retardation in this
group than would be expected among chil-dren in the community.8
That such growth failure is not limited to American children with patent ductus
arteriosus is indicated in a study of the
phys-ical development of 77 Swedish children
with this lesion, by Kjellberg
et al.9
It seems reasonable to expect a correlation
between the degree of growth failure and
the volume of blood shunted through the
ductus.’#{176} In the present series there is little information on this point because cardiac catheterization was performed in only a few
74
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DUCTUS ARTERIOSUS
U
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AGE IN YEARS
FIG. 4. Chart of the youngest child operated upon in the series. Postoperatively
there was improvement in growth, though not to normal.
or the ductus was atypical. There was no correlation between the greatest external diameter of the ductus, measured at opera-tion, and growth retardation; the average diameter in the age groups 3 to 5 years and 6 to 10 years was about 8 mm (8, 8.3 mm, respectively
)
, regardless of the stature ofthe children.
Observation of the parents and siblings
of the children with patent ductus arterio-sus and retarded growth indicated that the
diminutive size is not familial. The group
studied included identical twin girls, one of whom had a ductus; lag in growth in the
affected twin began shortly before the
sec-ond birthday and preceded excessive fatig-ability. A report by Porter included a set of identical twins, only one of whom had a
ductus and was retarded in growth.
If growth failure in these patients is
re-lated to the presence of a shunt of blood from systemic to pulmonary circulation, one would anticipate that obliteration of the
shunt would result in a spurt in growth. There actually was acceleration of growth
postoperatively in some of the children; in
17 of 29 who were near-normal, and 5 of
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190
180
170
160 U)
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I30
120
IIO100
90
FIG. 5. Slight improvement after operation, but not to normal, 7 years after surgery.
Patient has no cardiac murmur. Size of heart and the electrocardiogram returned to
normal after ligation of a large ductus.
5 6
7
8
9
10 II
12 13 14 15 16
AGE IN YEARS
ARTICLES
It is noteworthy that, in the patients who
needed it most, a spurt in growth did not occur or was not sufficient to bring them to
normal within the period of observation. Whether a period of rapid growth during adolescence will compensate for the earlier
failure to achieve adequate growth is un-known. This has not yet happened to the few patients thus far who have reached adolescence.
This persistence of growth failure was
noted among some of the postoperative
patients studied by Adams,8 Richards,12 Cosh,13 and others. Among 101 patients reviewed by Lynxwiler and Wells,6 there
was improvement within 6 months post-operatively in some of the patients, -but 35 remained 10% or more below the minimal standard for height and weight for the age and sex, and 8 of these were still
20%
or more below the minimum value.GIRLS (s-s7vRs.1
2 3 4
AGE IN YEARS
I90
I 80 I 70
160
I50
140 ‘.)
I.-x ‘I
AGE IN YEARS
FIG. 6. Persistent retardation in growth 62/i2 years after operation in a girl. No heart murmurs since
liga-tion of ductus. The slight growth spurt after operation was inadequate to bring her into a normal range.
otherwise successful surgery for patent duc-tus is both difficult to explain and
dis-appointing. It is not related to frequent infections as this has not been a problem after operation. It is not due to
recanaliza-tion or persistent patency of the ductus, for none of these children had a continuous murmur or other signs of inadequate closure
of the ductus. As many children in the near-normal group (29.6%
)
have systolic murmurs 6 months after operation as in the physically retarded group (27.4%) . Most of thesemur-murs are considered innocent, but three of the retarded group are known to have
addi-tional lesions (one a ventricular septal de-fect, another congenital mitral stenosis, and
the third, coarctation of the aorta), and three
of the near-normal group are considered to
have a coexistent lesion (pulmonary stenosis in two and aortic stenosis in one).
The most important factor in causation of continued growth failure seems to be the age of the patient at the time of surgery. It
appears that the continued presence (during
the period of most rapid growth) of a shunt
of blood away from the systemic circulation
is associated with a stunting of growth
which is not readily corrected after aboli-tion of the shunt. Even 4 and 5 years of age
may be too late for operation to be
com-pletely corrective in this regard.
Nine of the patients in the group with
slow growth were seen before 4 years of
age. In all of these the failure to gain was
evident by 3 years of age. Of six who were first seen within the first 2 years of life, fin-paired physical development was apparent in five from the first examination. One of
these was observed from birth. The sixth
infant developed normally until the age of
18 months, when her weight dropped to the
16th percentile and then fell below that level by 2% years. Lag in weight gain
pre-ceded lag in height, which did not occur until between 23 and 3 years.
The ages at operation of the 52 children
in this report were from 3%2 to 14 years,
by Gross.5 Most had surgery at 4 or 5 years
of age. Of the 23 with growth failure, 13
were operated upon before the age of 6. The number of patients at each age is:
Age at Operation Number of Patients
(yr)
3 1
4
75 5
6 2
7 4
8 1
10-14 3
There were several reasons why ‘the
op-I
2
3
4
5
6
120
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105
I00
95
90
85
80
75
70
65
60
55
50
U)CD
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I
2
3
4
AGE IN YEARS
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CD
0
CD
Lu
ARTICLES
eration was performed at the ages
mdi-cated. First, the safety of the procedure was being established during the period when
these operations took place, and it was
be-lieved that the operation was easier for the
surgeon and safer for the child around
5
years of age than younger. Second, the child
is considered to be psychologically better
suited to hospitalization and operation at 5
years than earlier, and still better suited
around 10 years.’4 Third, the patient was
often not referred until a preschool examina-tion around 5 years of age. Even though
diagnosis was made earlier, operation was
5
6
Fic. 7. Prompt improvement to normal height and weight in a child whose ductus
120
“5
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90
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AGE IN YEARS
usually deferred, if the child were not faring too badly, until around 5 years of
age for the first two reasons. It was not ap-preciated that the poor growth pattern
might not be corrected by surgery after that age.
That growth may be restored to normal
in patients with large left-to-right shunts by
earlier operation is illustrated by Figures 7
and 8. In one child (Fig. 7) the
arterio-. venous shunt occurred through a large
duc-tus which was divided at the age of 24A2
I 2 3
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Ic
CD
0
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CD
years. Noteworthy are the poor weight gain from early infancy and the lag in linear
growth which appeared between 1% and 2 years. The prompt spurt in growth within
the year after operation brought the child’s
growth up to normal.
A similar result from earlier abolition of a large left-to-right shunt is shown by a boy
(Fig.
8)
whose large ventricular septal de-fect was closed at the age of 21%2 yearsby Drs. C. Walton Lillehei and Richard
Varco.
4 5 6
FIG. 8. Improvement to a normal range of height and weight following abolition
of large arteriovenous shunt through a ventricular septal defect at 21O/2 years
ARTICLES
If operation is desirable at an early age for those with growth retardation, what are
the psychologic and surgical implications of operation prior to 3 years of age? It
seems reasonable to accept what contemp-orary psychologic loss may result from
hospitalization of the preschool child for the much greater future psychologic gain of more nearly complete restoration to normal growth for the years to come. From a
surg-ical standpoint it is now evident that the operation can be performed safely by skill-ful surgeons, even in the very young.1519
Therefore, it is now the authors’ opinion
that development of growth retardation, in height and weight, in the young child with
a patent ductus arteriosus is an indication for early surgery. It is believed that the chance of the patient achieving normal
sta-ture will be increased if operation follows the appearance of marked growth failure
by no more than a few months. Practical
application of this concept will probably mean surgical correction of the patent
duc-tus arteriosus in this group of children
before 3 years of age.
SUMMARY
The incidence of marked retardation of growth in children with patency of the ductus arteriosus was reviewed in an
op-erative series from the New York Hospital
and from the literature. Retardation in growth was present in approximately
one-third to one-half of the children.
Almost all of the patients in the New York Hospital series with serious growth impairment remained retarded following
otherwise successful surgery on the ductus at ages 3 to 14 years. Periods of postopera-tive observation ranged from 1 to 10 years,
averaging 4 years.
Possible reasons for persistent impair-ment of growth are considered.
This observation is the basis for a recom-mendation of early operation for patients with patent ductus arteriosus who become retarded in height and weight. It is
antici-pated that this will afford a better chance of reversibility of the abnormal growth
pat-tern, if the retardation has not persisted too long during the period of most rapid
growth.
REFERENCES
1. Taussig, H. B., and Cain, A. S., Jr., edi-tors: World Trends in Cardiology. II. Cardiovascular Surgery. New York, Hoeber, 1956, p. 4.
2. Taussig, H. B. : Congenital Malformations
of the Heart. New York, Common-wealth Fund, 1947, p. 22.
3. Ibid., p. 335.
4. Gibson, S. : Symposium on congenital heart diseases. PEDIATRIcS, 2:325, 1948. 5. Gross, R. E., and Longino, L. A. : The
patent ductus arteriosus; observations from 412 surgically treated cases.
Cir-culation, 3:125, 1951.
6. Lynxwiler, C. P., and Wells, C. R. E. : Pat-ent ductus arteriosus; a report of 180 operations. South. M.
J.,
43:61, 1950. 7. Ash, R., and Fischer, D. : Manifestationsand results of treatment of patent duc-tus arteriosus in infancy and childhood;
an analysis of 138 cases. PsnIAi’ruCS,
16:695, 1955.
8. Adams, F. H., and Forsyth, W. B. : The
effect of surgery on the growth of pa-tients with patent ductus arteriosus.
J.
Pediat., 39:330, 1951.9. Kjellberg, S. R., Mannheimer, E., Rudhe, U., and Jonsson, B. : Diagnosis of Con-genital Heart Disease. Chicago, Yr. Bk. Pub., 1955, p. 401.
10. Eppinger, E. C., Burwell, C. S., and Gross, R. E. : Effects of patent ductus arterio-sus on the circulation.
J.
Clin. Invest., 20:127, 1941.11. Porter, W. B. : The effect of patent ductus arteriosus on body growth. Am
J.
M.Sc., 213:178, 1947.
12. Richards, M. R. : Pre- and postoperative growth patterns in congenital heart cbs-ease as shown by the Wetzel grid. PEDIATRICS, 9:77, 1952.
13. Cosh,
J.
A. : Patent ductus arteriosus; afol-low-up study of 73 cases. Brit. Heart
J.,
19:13, 1957.
14. Prugh, D. G., Staub, E. M., Sands, H. H., Kirschbaum, R. N., and Lenihan, E. A.:
Study of the emotional reactions of
chil-dren and families to hospitalization and illness. Am.
J.
Orthopsychiat., 23:70, 1953.15. Potts, W.
J.
: Surgical treatment of patent ductus arteriosus. Pediat. Clin. North America, 1:159, 1954.ductus arteriosus in infants. Am. Heart
J., 43:553, 1952.
17. Adams, P., Jr., Adams, F. H., Varco, R. L.,
Dammann,
J.
F., Jr., and Muller, W. H.:Diagnosis and treatment of patent
duc-tus arteriosus in infancy. PEDIATRICS,
12:664, 1953.
18. Bauersfeld, S. R., Adkins, P. C., and Kent,
E. M. : Patent ductus arteriosus in
in-fancy.
J.
Thoracic Surg., 33:123, 1957.SUMMARIO IN INTERLINGUA
Problemas
Currente
Relative
a Patentia
del
Ducto
Arteriose
Le incidentia de retardation de crescentia in
infantes con patentia del ducto arteriose esseva
investigate super le base de un series de casos
operate a iste hospital e de datos in le
littera-tura. Un tal retardation esseva presente in inter
un tertio e un medietate del casos in
previe-mente reportate series e in 42% del patientes
operate a iste hospital. Ex un serie de 52
in-fantes, 22 esseva retardate in peso a infra le
dece-sexte centesimo in le schema de crescentia
de Iowa e esseva infra le linea “medie-ISD” in
grandor. Post alteremente successose
opera-tiones ductal a etates de inter 3 e 14 annos,
quasi omne le patientes con serie defectos
crescential vidite a nostre hospital remaneva
retardate durante periodos de observation
post-operatori de inter 1 e 10 annos (con un valor
medie de 4 annos). Le rationes possibile pro
le persistente retardo crescential es discutite. Es
formulate le opinion que le persistentia de un
grande derivation de sanguine ex le circulation
systemic durante le periodo del pius rapide
crescentia es responsabile pro le atrophia e que
iste effecto non es facilemente revertite per
operationes post un etate de 4 o 5 annos. Iste
conception justifica le recommendation de
prompte operationes ductal in patientes in qui
altor e peso comencia retardar se, con le
ex-pectation de prospectos meliorate pro le
re-version del anormal modo de crescentia si le retardation non persiste troppo longe durante Ic
