VENTRICULAR
SEPTAL
DEFECT
IN
INFANCY
By MARY ALLEN ENcI, M.D.
New York City
Case
TABLE 1
hISTORY
1)uration
Sex of
Pregnancy
Birth ‘Vt.
(gui.)
Feeding I)ifflculty
Failure to Gain
Weight
1936 + + + 0
1-280 + + + +
2360 + + + +
231() + + + +
3460 + + + +
268 + + + +
3400 + + + +
214() + + + (I
2092 + + + +
16
LTHOUGH often compatible with a long
and symptom-free life, a ventricular
septal defect may at other times be such
a serious congenital cardiac lesion that it
leads to heart failure and to death in early
infancy. This is a report of nine such pa-tients, all of whom died prior to the age of 5 months as a result of their cardiac anomaly.
The following two case reports have
been selected as representative. The
ac-companying tables summarize the
import-ant features of the group. (See tables 1-4.)
CASE REPORTS
Case 1: J.R. was admitted at the age of 30
days because of breathing difficulty, muffled
cry, failure to gain weight, and a heart murmur from birth.
Although she was born after a full term,
uncomplicated pregnancy, her birth weight was only 2268 gm. Edema of the face and extremities was present at birth, and her cry
was scarcely audible. A systolic murmur and
a snapping P, were heard. Respirations
be-From the Department of Pediatrics, The New York Hospital-Cornell Medical Center, New York City.
(Received for publication Jan. 31, 1954.)
came labored, and during periods of increased
respiratory effort she appeared cyanotic. By the age of 2 wk. there was marked intercostal retraction, and rales were heard in the lungs. She was placed in oxygen, given antibiotics,
and fed by gavage. At the age of 1 mo., when
she was transferred to this hospital, she had
not regained her birth weight.
Physical Examination: Temperature 36.8#{176}C.;
pulse 165/mm.; respirations 60/mm.; blood
pressure 120/60 mm.Hg.; weight 2200 gm. She was a thin baby in great respiratory
dis-tress. She coughed weakly and cried without
making a noise. In oxygen there was no visible
cyanosis. There was a marked precordial bulge
with lower costal retraction. The heart was
enlarged to percussion, a systolic thrill was palpable, and there was a long systolic
mur-mur heard with maximal intensity at the third left interspace. P2 was snapping, and there was a gallop rhythm. Rales were scattered through-out both lung fields and the liver edge extended
half way to the umbilicus, but there was no peripheral edema. Femoral pulses were readily
palpable.
Laboratory Studies: Fluoroscopy of the chest revealed cardiac enlargement to the right and
left in the frontal view and both anteriorly
and posteriorly in the oblique views. The
cardi-othoracic ratio was 70%. Pulsations of the
.I.C.
S.-’.
R.R.
S.L.
ES.
JR.
E.M.
B.K.
TB.
F’ Tern
F 74 mo.
(Twin)
M Term
F’ Term
F Term
F’ ‘Ferni
F’ ‘l’erm
‘l’erni
F Term
Respiratory Aphonic
‘ Combined ventricular hypertrophy.
Gallop Rhythm
EM. + ii + + 0 0
B.K. + Poor + 0 + +
,1’.B. ? P2Forceful + 0 + 0
Gallop Rhythm
cardiac chambers were normal. The pulmonary Hgb. was 12.2 gm./100 cc., RBC count 3.6
arteries were enlarged, and the lung vascularity million/cmm., WBC and differential count was greatly increased. Barium swallow deline- were normal. Urinalysis on one occasion
ated an enlarged left auricle and a left aortic showed a trace of albumen.
arch. There were no retroesophageal vessels. Course: She was placed in oxygen. The
These findings were confirmed by chest RG. calculated digitalizing dose (0.035 mg./kg.) of Electrocardiogram (see Fig. 1) showed high digitoxin was given in 12 hr., and for the first
and peaked P-waves, a sharply inverted T, week she received 0.01 mg. daily. The main-and biphasic QRS,. Multiple unipolar pre- tenance dose was then doubled and later
cordial leads were suggestive of combined tripled. She was given injections of a mercurial
ventricular hypertrophy. There was a reversal diuretic 3 times/wk. Although she was afebrile
of the normal T-wave pattern in the chest and there was no leukocytosis, penicillin and
leads: the T-waves were positive in V4R and later aureomycin were added because of the
VSR but were deeply inverted in V1-6. The possibility of a superimposed
bronchopneu-rhythm and conduction times were normal. monia. When sucking she became exhausted,
TABLE S
LABORATORY STUDIES
(‘hest RG ECG
-
Pulnio-
Cardio-- mmar’ Case thoracic ,
- (
on-Ratio
-gestion
.
, Bi-
Pre-A\ . RS - Abnormal
, . Axis - phasic , , Cordial
(onduction Amplitude I -W ayes
QRS Leads
J.C. 61% + N It Wide + + 0
S.A. 65% + N R-O N 0 N CVH*
R.R. 70% + N It Wide + + RVH
S.L. 68% + N It (extreme) Wide + + 0
ES. 6% + N R N + + CVII*
JR. 71% + N R N + + CVH*
EM. 77% + N R Wide + + 0
BK. - - Prolonged R N 0 + 0
(‘ase
Age
at
1)eath (lunar
mo.)
F’orainen Ovale
Ductus
Arteri-osus
Ventricular
Septal Defect
Loca Diame-tion ter
(mm.)
\Vt. of Heart
-- Nor-Observed ma!
(gin.)
Ventricular
Wall Thickness
---Right Left
(mm.)
Medial Hyper-trophy
Pulino-nary Arteries
J.(’. 4 Closed Closed hugh
S.A. 4 Closed Closed high
It.lt. 4 Patent Patent high
15 75
TABLE 4
SUMMARY OF I’OSTMOHTEM FINDINGS
S.L. ‘l Closed by thick
valve though ana-tomically patent
ES. 2 Patent
J.lt. 1 Patent
EM. 1 Patent
BK. 14 Patent
TB. Patent
6 55
30 5 Heart and
Lungs-ISO
Patent: high 10 48
I i-HIll.
Patent Low 18X7 ?
Closed high 4 48
Patent high 15 35
Patent high 8 57
Patent high 7 4.5
10 9 +
5 7 (1
8 5 +
18 7 7 +
(; 7 +
18 5 (; +
25 10 10 +
20 6 7 +
15 7 5 +
cyanotic, and still more dyspneic; therefore,
her feedings were gavaged. Despite these measures she did not improve. Her tachycardia lessened, and she even became dehydrated
following diuresis; nevertheless, the rales in her lungs remained, and neither the heart nor
the liver decreased in size. She died at the
age of 51 days.
Clinical Impression : Congenital malforma-tion of heart, possibly large auricular or yen-tricular septal defect. Because of the abnormal
T-waves in the ECG, the diagnosis of endo-cardial sclerosis or myocarditis was considered.
Postmortem Examination: Autopsy No.
13788 was performed by Drs. Helper and Mason. The heart weighed 48 gm. (normal for
age 23 gm). The wall of the moderately di-lated and hypertrophied right ventricle was
5 mm. in thickness and the left ventricular wall 6 mm. Venous return was normal. The foramen ovale was not stretched open. There
was a high ventricular septal defect 4 mm. in diameter located just anterior to the
mem-branous septum. The pulmonary artery was
dilated. The aorta arose in normal fashion from the left ventricle without over-riding the septal
defect. The coronary arteries were normal, and
the ductus arteriosus was closed. The heart valves were normal in circumference, but the
pulmonic valve was bicuspid, and a congenital
median raphe bisected the base of the larger valve. Smooth, nodular excrescences measur-ing no more than 1 mm. in diameter, a few
Fic. 2. Chest RGs, in ES. (a) and EM. (b). Cardiac enlargement and increased pulmonary vascular markings are seen.
of them blood-filled, studded the heart valves. Microscopically, they consisted of fibrous
con-nective tissue. Neither on gross nor microscopic
examination were any abnormalities of the
endocardium or mvocardium noted. There was marked hyperemia of the lungs with hemor-rhage into the tissue and alveolar spaces and
thickening of the alveolar walls. The small
muscular pulmonary arteries exhibited medial hvpertrophy. In addition to the cardiac
anomaly, there were multiple, small
colloid-containing cysts of the thyroid gland, a double right ureter, and a large right kidney with 2
pelves.
Case 2: S.M.L. was admitted at the age of
8 wk. because of respiratory and feeding diffi-culty and poor weight gain. She was born
after an uncomplicated pregnancy at term; however, her birth weight was only 2310 gm. A heart murmur was discovered at birth, and
because of slow progress she was kept in the hospital until 5 old. At home she gained
only 120 gm. When crying or slowly taking 30
to 60 cc. of formula, her respirations became
labored and she had “choking spells.”
Physical Examination: Temperature 37.3#{176}C.;
pulse 190/mm.; respirations 60/mill.; weight 2600 gm. She was a pale, poorly nourished
infant whose cry was so weak it was barely audible. Even at rest and in oxygen she had rapid and labored respirations with retraction
of the suprasternal notch and lower ribs. No
cyanosis or clubbing was seen. There was a precordial bulge, and the heart was enlarged
to the anterior axillarv line. No thrill was
palpable. The heart sounds were loud and banging, and P2 was especially accentuated.
A loud, precordial systolic murmur could be
heard with maximal intensity at the base of
the heart on the left. In the lung fields
bi-laterally were numerous fine rales. The liver edge was felt 3 cm. below the costal margin and was not tender. No edema was present.
The peripheral pulses were equally strong.
Laboratory Studies: Fluoroscopy and RC of the chest in the frontal and oblique views with
barium swallow showed an enlarged heart with
a cardiothoracic ratio of 68% (see Fig. 2a). The
right auricle and ventricle and the left auricle
were markedly enlarged, and the pulmonary vessels were congested. The standard lead ECC showed extreme right axis deviation with
an S-wave as the major QRS deflection in all
leads. The amplitude of the QRS complexes was wide. T, was negative and T2 flat. Con-duction times were normal. Hgb. was 12.5
gm./100 cc., RBC count 4.0 million/cmm.,
WBC count 6.3 thousand/cmm.
Course: She was placed in oxygen,
digital-ized, and given injections of a mercurial diu-retic and penicillin. Feedings were gavaged.
Nevertheless, she developed progressive
car-diac decompensation, with an increase in rales in the lungs and with edema of the feet, legs, sacrum, and periorbital tissues. At the age of
74 days she died.
Clinical Impression: Congenital
malforma-tion of heart, possibly auricular septal defect
and mitral stenosis.
Postmortem Examination: Autopsy No.
12601, performed by Dr. White. The heart
weighed 48 gm. (normal for age 23 gm.). The wall of each ventricle was 7 mm. thick. The
20
vas siiiall. Both auricles were dilated, the left more than the right. The endocardium of the left auricle was slightly thick and opaque;
microscopically slight fibrous thickening was
seen. In the superior portion of the interven-tricular septum there was a large defect meas-uring 1 cm. in diameter. Although anatomically patent, the foramen ovale was closed by a thick flap. The ductus arteriosus had a 1 mm. lumen. The venous return, cardiac valves, the great vessels, and the coronary arteries were normal. There was no apparent dextroposition of the aorta. The lungs were congested. On micro-scopic examination medial hypertrophy of the small muscular pulmonary arteries was seen.
ANALYSIS OF FINDINGS
Roger’s description of the clinical pic-ture of ventricular septal defect stressed the absence of any other sign of heart dis-ease except the characteristic murmur and accompanying harsh thrill. Abbott,2 Brown,3 Gibson,4 and others agreed that the
char-acteristic feature of ventricular septal de-fect is the absence of symptoms in the
presence of distinctive physical signs. In Selzer’s recent analysis of the cause of
death in 85 cases of ventricular septal de-fect, he listed only one patient who died in the first year of life in cardiac failure.5
While Taussig6 recognized the essentially
benign course of most patients with simple ventricular septal defect, she at the same time described the picture of “high en-tricular septal defect where there was early
cardiac enlargement, pulmonary congestion, and heart failure.” The symptoms were attributed to the propulsion of blood from the left ventricle directly into the pulmo-nary artery. Marquis,7 too, pointed out that ventricular septal defect is more frequently responsible for death in early childhood
than is appreciated. He described the clini-cal and postmortem findings in two young
children, aged 1 and 2% years, who were similar to the infants herein presented. It is apparent, then, that there is a particular
group of children in whom a defect in the ventricular septum leads to cardiac dis-ability early in life.
This group, illustrated by the nine babies in the present report, is characterized by
the following features. Common to all were failure to gain weight, feeding difficulty, cough and respiratory distress. In all but two the cry was inaudible. On examination there was a chest deformity consisting of a precordial bulge and retraction of the lower ribs anteriorly at the level of the
diaphragm. There was a loud systolic mur-mur, accompanied by a thrill, maximal over the body of the heart near the left sternal border. The pulmonary second sound was accentuated, sometimes strikingly so, and in three patients there was a gallop rhythm. All gave evidence of congestive failure, in-dicated by rales in the lungs more often than by hepatic engorgement or peripheral edema. Cyanosis, if present, seemed to be associated with periods of particular reSpir-atory difficulty.
On fluoroscopy and roentgenogram of the chest there was cardiac enlargement
usually involving all four chambers, and the pulmonary congestion was so marked that it was often mistaken for bilateral
broncho-pneumonia. (See Figs. 2 and 3 and table 3.)
The ECG was variable; however, the fol-lowing changes were frequently found.
(Figure 1 and table 3). The QRS complexes in the standard leads were biphasic in one or more leads and often of wide amplitude. Multiple unipolar precordial leads indicated either right ventricular hypertrophy or com-bined ventricular hypertrophy. In the pre-cordial leads, the normal T-wave pattern (negative in right heart and positive in left heart leads) was reversed, and in the stand-ard leads, Ti and 2 were low, flat, or in-verted. In two patients the PR interval was prolonged. The changes preceded digitali-zation.
At postmortem examination (see table 4) the heart weight was usually more than
double the normal value, and both
ventri-cles were dilated and hypertrophied. Ex-cept for one patient (E.S.) in whom the
defect was low in the muscular septum, the
Fic. 3. Chest RGs of S.L. (a) and J.C. (b), showing greatly increased pulmonary vascuhar
markings and moderate cardiac enlargement.
in the membranous Septum just beneath
the aortic and tricuspid valves. The size of the defect ranged from 2 to 18 mm. The
course of the babies with the smaller de-fects did not differ appreciably from that of the infants with the larger openings. The aorta was not dextroposed. The pulmonary artery was dilated, and hyperemia of the lungs was a constant finding. The fetal char-acter of the small muscular pulmonary ar-teries was retained in all but one child (S. A.) who died at the age of 4 months.
The closure of the ductus arteriosus and the foramen ovale took place at the normal
time (see table 4). These structures are
usually not closed in the normal infant
until the second or third month of life. In the two babies who survived to the
age of 120 days, both fetal passages were occluded. In the remaining infants death
occurred before the age of three months. It is believed that in the normal newborn infant these two structures cease to function in the first few hours or days after birth
although anatomically they may still be patent for the first few weeks of life.8’9 From a review of over 500 consecutive
autopsies on normal hearts, aged 1 day to 1 year, Christie10 found that by the age of 8 weeks 88% of the ducti were closed, and by 12 weeks in 87% of the hearts the
fora-men ovale was closed. Prior to the age of
3 months, therefore, anatomic patency of
the foramen ovale or ductus arteriosus
should probably not be considered a cardiac
abnormality.
Whether the patency of the fetal passages was of significance is speculative. It is
un-likely that the foramen ovale was function-ally important, since when protected by a
flap it prevents a left-to-right shunt of blood and permits a right-to-left shunt only if right auricular pressure is elevated to a level exceeding that in the left auricle. It is possible, however, that a small patent
ductus arteriosus added to the pulmonary
congestion and cardiac enlargement.
Never-theless, the fact that the course of all nine
babies was essentially the same, whether or not these structures were found at
post-mortem examination to be patent, points to the septal defect as the primary, if not the sole, cause of the difficulty.
It is remarkable that 7 of the 9 babies
had birth weights in the “premature” range despite the fact that with one exception the
pregnancies were full term. Among an
ad-ditional group of seven infants with a simple ventricular septal defect at post-mortem examination, not included in this report because of associated conditions
which might have contributed to their
death, there were five who had birth
con-genital malformations, including those of the heart and great vessels, is higher than
among full term babies of normal weight.11 Araya and Whitel2 found that the incidence of prematurity in patients with congenital cardiac anomalies was twice as great as in the population at large. In his analysis of
patients with patent ductus arteriosus, Lynxwiler found a 12.5% incidence of
pre-maturity. The association of prematurity in
this group of infants with ventricular septal
defect (three-fourths of the babies) was much higher than in the two reports above and higher than it was in other types of congenital cardiovascular anomalies autop-sied at this hospital during the same period.
Whether the ventricular septal defect was responsible for growth retardation in
utero is conjectural. If during fetal life the pulmonary resistance is high and the pres-sure in the two ventricles is essentially the
same, as Hamilton’s work on rabbit and dog fetuses indicates,” then one would not expect any appreciable flow of blood across the septal defect until after birth. If, how-ever, resistances in the two circulations
were out of balance, as for example because of too early opening up of the pulmonary vascular bed or inadequate vasomotor
con-trol of the fetal pulmonary circulation, then even in fetal life a shunt of blood
away from the systemic circulation might take place to a degree sufficient to impair normal fetal weight gain. In support of the
concept of the septal defect’s functional im-portance in utero is the fact that in one in-fant (Case 1) heart failure was present at birth.
Of interest in this group of infants was the frequent occurrence of a hoarse or inaudible cry. Such a finding is not un-common among patients who for any reason have marked enlargement of the pulmonary artery or dilatation or displacement upward
of the left auricle or left pulmonary veins.15 Under these circumstances Fetterolf and
Norris’6 have shown that the left recurrent laryngeal nerve is squeezed between the
enlarged left pulmonary artery and the
aorta or the ligamentum arteriosum as the nerve loops around the latter structure on its way to the neck. The damage to the nerve results in paralysis of the left vocal
cord and hoarseness. Sometimes the right cord compensates for the paralyzed left
cord so that the patient’s voice improves.’5
COMMENTS
Why should these infants with a simple ventricular septal defect die in heart failure
whereas most people with the same
mal-formation have little or no cardiac embar-rassment? It is clear that a balance between systemic and pulmonary blood flow was not
established. Possible reasons for this failure
include the size of the defect, its location,
the relation of the opening to the origin
of the aorta and the structure and function of the pulmonary vascular bed. It is
prob-ably the interrelationship of cardiac and
pulmonary factors that is responsible for the cardiac insufficiency rather than any
feature singly.
Size of the defect alone is an inadequate explanation of the severity of the picture. If the openings are classified, as Seizer5 sug-gested, according to their greatest diameter into large (10 mm. or greater),
medium-sized (5-10 mm.), and small defects (less than 5 mm. across), the nine cases were
equally distributed among the three groups. In relation to the small heart size of in-fants, however, even the “small” defects
may actually be larger than mere measure-ment of diameter would indicate.
Nor is the location of the defect high or
low in the Septum solely responsible for the clinical manifestations. Although their courses were indistinguishable, in eight in-fants the openings were high in the mem-branous septum and in the ninth, low in
the muscular septum. This proportion of high to low defects is similar to the ratio in Selzer’s review where there were only 6 out of 65 with a defect low in the septum.5
group of babies may be at the borderline
of classffication as Eisenmenger complex or simple ventricular septal defect.’7
It is the interdependence of the cardiac factors just mentioned and the structure
and function of the pulmonary vascular bed that is probably the most important deter-minant of the course of patients with a
ventricular septal defect.’8 Particularly if the defect is large so that the pressures in the two ventricles equalize or if it is high and located in such close proximity to the aortic valve that both right and left ventri-cles communicate with the aorta, then a situation exists in which according to
Edwards18 there is a “ventricular ejectile force common to the pulmonary and
sys-temic circulations in the absence of pul-monary stenosis.” Under these circum-stances the relationship between the sys-temic peripheral resistance that is normally high and the pulmonary resistance which
is normally low favors the passage of most of the blood into the low-pressure circula-tion. This results in a tremendous overload-ing of the pulmonary circulation and
con-comitant deprivation of systemic blood
flow, to such a degree that heart failure ensues and the condition becomes
incom-patible with life. Such a course was fol-lowed by the infants in this report.
This sequence of events may be altered or prevented if pulmonary resistance is in-creased above its usual low level. Civin and Edwards pointed out that the retention
into postnatal life of the fetal character of the pulmonary muscular arteries serves to
ment of a balance between the two
circula-tions.
On review of the pulmonary vasculature in these nine infants it was seen that the
fetal character of the small muscular pul-monary arteries was retained in all but one
of the babies (S.A.). In comparison with a control group of 18 infants who died at the same ages of causes other than cardiac or pulmonary, the medial hypertrophy and luminal narrowing of the vessels in the pa-tients with ventricular septal defect was more pronounced. Nevertheless, these
in-fants died in heart failure. This implies that the pulmonary vascular resistance was still too low and suggests that although the
structural arrangement for maintenance of a high degree of resistance may have been present, that this function was not fulfilled. Possibly there was a failure of response to
substances that increase musculo-vascular tone or a deficiency in their elaboration. Little is known at the present time con-cerning vasomotor control of the small pul-monary arteries and arterioles. It is
un-fortunate that in this group of infants pul-monary artery pressure and blood flow and
the pulmonary vascular resistance could not be measured. All were so small and
their conditions so critical while in the hospital that cardiac catheterization was not performed.
progres-sively increasing pulmonary vascular re-sistance and pulmonary hypertension. Then the pulmonary blood flow in time may
be-come reduced below normal, and the
pa-tients present symptoms and signs akin to those of pulmonary stenosis : exertional
dyspnea and fatigue, increasing right heart
enlargement and eventually right heart failure. Shunt reversal with intermittent or persistent cyanosis takes place.’#{176}
Thus there is a spectrum of pictures that may be seen in patients who have a ven-tricular septal defect. The healthy condi-tion of many patients contrasts with the disability of those at the other end of the
scale who have signs and symptoms of cardiac insufficiency either because of too little resistance or too great impedance to
pulmonary blood flow. Intermediate is the group who have established a balance be-tween systemic and pulmonary blood flow with only slight or moderate changes in pulmonary vascular resistance and pulmo-nary artery pressures. Cardiac
catheteriza-tion studies on patients who illustrate these different stages have been reported by
Damman and Muller,’#{176}Handelsman et al.,”
Mannheimer,” Adams,’3 Wood24 and others.
The diagnosis of ventricular septal defect early in life may be difficult. It must be differentiated from other conditions in which there is cardiac enlargement and pul-monary congestion. Many of the malforma-tions which produce large left-to-right shunts may during infancy and even later
resemble one another: anomalous pulmo-nary venous return to the right auricle, auricular septal defect, ostium atrio-ventric-ulare commune, ventricular septal defect,
Eisenmenger complex, single ventricle without pulmonary stenosis, truncus
arteri-osus, aortic septal defect, and large patent ductus arteriosus. Furthermore, the fre-quency with which abnormal T-waves are found in the EGG of infants with ventricu-lar septal defect necessitates its separation from conditions where there is cardiac en-largement and “myocardial ischemia”:
en-docardial sclerosis, anomalous origin of the
coronary artery from the pulmonary artery,
myocarditis, and glycogen storage disease
of the heart. Although in the latter four
situations there is predominantly left-sided cardiac enlargement,21 when the heart
en-larges to a considerable degree and when the patient is in heart failure, the right
side of the heart also appears enlarged, and there is pulmonary congestion. The similar-ity to ventricular septal defect is thus
en-hanced.
Probably the most useful clinical features in the differentiation during infancy of a
ventricular septal defect from these other lesions are the location and quality of the
systolic thrill and murmur. Their character
was clearly described by Roger.1 They are
maximal over the body of the heart around the third left interspace. The thrill is coarse.
and the murmur is loud, long and harsh. Following it an accentuated pulmonary sec-ond sound can be heard. Such a thrill and murmur in the absence of cardiac signs
and symptoms have been considered by Abbott,2 Brown,’ Gibson,4 Seizer,5 and Taussig6 and others to be almost
path-ognomonic of ventricular septal defect. This group of cases would suggest that in infancy such a thrill and murmur even in
the presence of signs and symptoms of excessive pulmonary blood flow, cardiac enlargement, and heart failure is almost pathognomonic of a defect of the
ventricu-lar septum.
If technically feasible in a small infant and if the baby can tolerate the procedure,
the diagnosis may be confirmed by cardiac catheterization,26 particularly if dye dilu-tion curves from the right auricle, right ventricle and pulmonary artery are
re-corded. Angiocardiography with rapid serial exposures may reveal a momentary shunt of contrast agent from right to left ventricle’7 or the dilution of contrast substance in the right ventricle by the shunt of nonopacified blood from the left to right ventricle. An ir-regularity of the outlined ventricular sep-tum may be noted. Infrequently
ization and the use of oxygen, mercurial
diuretics, and a low salt intake, and toward improvement of the nutritional status by
gavage-feeding of high caloric mixtures. An-tibiotics were used to prevent or control in-fections. These measures were ineffective. It is hoped that in the near future surgical re-pair of the defect can be accomplished be-fore cardiac failure is severe and before
ir-reversible changes in the pulmonary
vascu-lar bed or in the heart have occurred. Technics of correction are now under
inves-tigation by Bailey and his group,’8 King and his associates,’9 Muller’#{176} and others. An
awareness of the seriousness of this malfor-mation in certain patients, even during baby-hood, and the early recognition of such in-fants are necessary preliminaries to success-ful surgery. The combination of increased clinical acumen and surgical skill should prove as valuable in the relief of this mal-formation as they have in other congenital cardiovascular anomalies that are now amenable to surgery.
SUMMARY
The clinical features and postmortem findings have been analyzed in nine infants who had a ventricular septal defect and
died in heart failure. Possible explanations
for the severity of the manifestations of this simple and common defect were
con-sidered. The differential diagnosis from
other conditions associated during infancy with cardiac enlargement and pulmonary
congestion was discussed. It is hopea that an awareness of the serious consequences
Cardiac Disease, New York, American Heart Association, 1936, pp. 36, 37 and
60.
3. Brown,
J.
W., Congenital Heart Disease,London, Staples Press Limited, 1950, p. 152.
4. Gibson, S., and Clifton, W. M., Congenital
heart disease, Am.
J.
Dis. Child. 55:761, 1938.5. SeIzer, A., Defect of ventricular septum, Arch.
mt.
Med. 84:798, 1949.6. Taussig, Helen B., Congenital Malforma-tions of Heart, New York, The Com-monwealth Fund, 1947, p. 390.
7. Marquis, R. M., Ventricular septal defect in early childhood, Brit. Heart
J.
12:265,1950.
8. Lind,
J.,
and Wegelius, C., Atrial septal defects in children: Angiocardiographic study, Circulation 7:819, 1953.9. Patten, B. M., Closure of foramen ovale,
Am.
J.
Anat. 48:19, 1931.10. Christie, A., Normal closing time of
fora-men ovale and ductus arteriosus:
An-atomic and statistical study, Am.
J.
Dis.Child. 40:323, 1930.
11. Dunham, Ethel C., Premature Infants: Manual for Physicians, Washington, D.C., Children’s Bureau, Publication 325, 1948, p. 188.
12. Araya, E., and White, P. D., Relationship of congenital heart disease to premature
birth, Am. Heart
J.
25:449, 1943. 13. Lynxwiler, C. P., and Wells, C. R. E.,Patent ductus arteriosus: Report of 180 operations, South. M. j. 43:61, 1950. 14. Hamilton, W. F., Woodbury, R. A., and
Woods, E. B., Relation between
sys-temic and pulmonary blood pressures in
fetus, Am.
J.
Physiol. 119:206, 1937. 15. Erlanger, H., and Levine, S. A., Atrialseptal defect: Report of 2 cases in which there was recurrent laryngeal nerve paralysis, Am. Heart
J.
26:520, 1943.Anatomi-26
cal explanation of paralysis of left
re-current laryngeal nerve found in certain
cases of mitral stenosis, Am.
J.
M. Sc.141:625, 1911.
17. Selzer, A., and Laqueur, C. L.,
Eisen-menger complex and its relation to
un-complicated defect of ventricular sep-turn : Review of 35 autopsied cases of
Eisenmenger’s complex, including two new cases, Arch. Tnt. Med. 87:218,
1951.
18. Edwards,
J.
E., Structural changes of pul-monary vascular bed and theirfunc-tional significance in congenital cardiac disease, Proc. Inst. Med. Chicago 18:6,
1950.
19. Civin, W. H., and Edwards,
J.
E.,Pathol-ogy of pulmonary vascular tree, Circula-tion 2:545, 1950.
20. Dammann,
J.
F., Jr., and Muller, W. H., Jr., Role of pulmonary vascular bed incongenital heart disease, PEDIATRICS 12:
307, 1953.
21. Handelsman,
J.
C., Bing, R.J.,
Campbell,J.
A., and Griswold, H. E., Physiologi-cal studies in congenital heart disease.Circulation in patients with isolated septal defects, Bull. Johns Hopkins Hosp. 82:615, 1948.
22. Mannheimer, E., Diagnostic value of
cardiac catheterization in isolated
pul-monary stenosis and large
interventric-ular septal defects, Arch. Dis.
Child-hood 24:264, 1949.
23. Adams, F. H., Pulmonary hypertension in children due to congenital heart
dis-ease,
J.
Pediat. 40:42, 1952.24. Wood, P., Pulmonary hypertension, Brit. M. Bull. 8:348, 1952.
25. Rosenbaum, H. D., Nadas, A. S., and
Neu-hauser, E. B. D., Primary myocardial disease in infancy and childhood, Am.
J.
Dis. Child. 86:28, 1953.
26. Cournand, A., Baldwin,
J.
S., and Him-melstein, A., Cardiac Catheterization in Congenital Heart Disease: Clinical and Physiological Study in Infants and Chil-dren, New York, The Commonwealth Fund, 1949, p. 83.27. Dotter, C. T., and Steinberg, I.,
Angio-cardiography, Annals of Roentgenology,
New York, Paul B. Hoeber, Inc., 1951,
vol. 20, p. 194.
28. Bailey, C. P., and others, Experimental
and clinical attempts at correction of in-terventricular septal defects, Ann. Surg.
136:919, 1952.
29. King, H., Shumacker, H. B., Jr., and Deniz,
N., Experimental surgical repair of
yen-tricular septal defects, Surgery 34:1100,
1953.
30. Muller, W. H., Jr., and Dammann,
J.
F.,Jr.,
Treatment of certain congenitalmal-formations of heart by creation of pul-monic stenosis to reduce pulmonary hypertension and excessive pulmonary
blood flow: Preliminary report, Surg., Gynec. & Obst. 95:213, 1952.
SPANISH ABSTRACT
Comunicaci#{243}n Interventricular en la
Infancia
Se presentan 9 casos con comunicaci#{243}n in-terventricular que fallecieron antes de los 5
meses de edad por insuficiencia cardiaca. Esta
anomalla se juzga como compatible con una
vida larga y asintom#{225}tica, y se manifiesta,
segdn Ia opinion de Ia mayorla de los autores,
solo por un soplo caracterIstico y un thrill
intenso.
Los 9 ni#{241}osestudiados presentaron desde el
nacimiento problemas alimenticios, trastornos
respiratorios, tos y falta de aumento normal de peso; a Ia exploraci#{243}n se encontraron
defor-midades del t#{243}rax,consistentes en prominencia precordial y retracci#{243}n de las costillas inferiores a nivel del diafragrna, el soplo y el thrill men-cionados, m#{225}sacentuaci#{243}n del segundo tono
pulmonar; en dos de los ni#{241}oshubo ritmo de galope. Todos manifestaron insuficiencia
con-gestiva y cianosis con cierta frecuencia.
Radio-l#{243}gicamente se observ#{243} crecirniento de las
cuatro c#{225}maras cardiacas y congestion pul-monar marcada. El electrocardiograma fu#{233}
variable. Con frecuencia se observO un grito ddbil y ronco, a veces poco audible. Siete de
los nueve niflos presentaron al nacer pesos
dentro de lImites “prematuros,” a pesar de que todos los embarazos fueron a t#{233}rmino excepto
uno, dato que diversos autores han se#{241}alado ya en estos pacientes.
En el estudio post mortem el coraz#{243}n se
en-contrO generalmente pesando m#{225}sdel doble
de lo normal, con ambos ventrIculos dilatados e hipertrofiados; en 8 de los ni#{241}osla
comuni-caci#{243}n interventricular era alta, situada
imme-diatamente abajo de las v#{225}lvulas aOrtica y
tn-cuspIdea, con tamaflo de 2 a 18 milImetros; la arteria pulmonar estuvo dilatada y las peque-#{241}asarterias pulmonares mostraron catheter
fe-tal en su musculatura. El cierre del conducto
525 East 68th Street ellos da una explicaciOn satisfactonia para Ia
insuficiencia cardiaca. Es decir, la interdepen-dencia de los factones candiacos con la
estruc-tura y funciOn de la red pulmonar y vascular,
es probablemente el determinante de mayor
valor en Ia evoluciOn de los pacientes con
co-municaciOn interventniculan.
El diagnOstico temprano de esta anomalIa puede ser dificil; debe diferenciarse de diversas malformaciones, como comunicaciOn inter-aunicular, complejo de Eisenmengen, ventriculo
cuniales y administraciOn de poca sal, asi como mejorar el estado nutricional y prevenir y con-trolan los proceso infecciosos con antibiOticos. Todos los m#{233}todosfueron ineficaces. Se espera que en el futuro pueda realizarse un trata-miento quir#{241}rgico antes de que se establezca Ia insuficiencia en forma intensa y sean
