544
Pediatrics
VOLUME 16 OCTOBER 1955 NUMBER 4
AMERICAN ACADEMY OF PEDIATRICS, INC.
PROCEEDINGS
AND
REPORTSHEART
CATHETERIZATION
IN
INFANCY
Physiological
Studies
By F. Mason Sones, Jr., M.D.
T
HE USEFULNESS of cardiaccatheteniza-tion in demonstrating the nature of
congenital malformations of the heart was
established a decade ago by Counnand,
Bing, and Dexter, and was subsequently
confirmed by a host of others. The com-plexity of the procedure and the dangers
inherent in its use have been greatly
stressed. It is widely believed that
cathe-terization is particularly dangerous, and
usually technically impossible, when
ap-plied to small infants and children. Work
done in our laboratory during the past 43
years has substantially modified these beliefs. Efforts toward adapting the technic
for study of infants have been stimulated both by inability to make precise diagnoses
on the basis of more readily obtainable
clinical information, and by the frequent
observation of deaths during the first 2
years of life, due to surgically correctable
lesions.
As a general rule we resort to heart
catheterization for aid in diagnosis in any
patient with the stigmata of a potentially
dangerous congenital lesion, in whom a
definite anatomic diagnosis cannot be
Presented at the Spring Session, American Academy of Pediatrics, April 4, 1955.
0 ADDRESS: Cleveland Clinic, Cleveland, Ohio.
established on the basis of clinical findings.
History, physical examination,
electrocan-diognaphic study, fluoroscopic examination,
and roentgenograms of the chest make up
the essential clinical data. In many
in-stances these are adequate for final
diag-nosis, but in the majority of problems en-countered in seriously ill infants, they may be only tile basis for establishment of an intelligent differential diagnosis. When the
data provided by adequately performed
catheterization is added to such a back-’
ground of clinical information, the
diag-nosis can usually be established. There are
few contraindications to heart
catheteniza-tion. Patients with active bacterial
endo-carditis are not studied for fear of
dis-lodging infected thrombi. Ventricular rate
in the patient with chronic auricular
fibnil-lation should be controlled by adequate
digitalization. Manifestations of congestive
heart failure should be eliminated as
corn-pletely as possible. No patient should be
denied the opportunity for precise
diagno-sis and possible cure or improvement
be-cause he is “too small,” or because the
functional severity of his lesion appears so
great that death might occur during or
shontby after catheterization. For such a
continu-ing failure to make an exact diagnosis upon
which effective treatment may be based. One hundred eight heart catheterizations
have been done in our laboratory imi
cliii-dren younger that 18 months of age.
Seventy-three of these patients were
studied during the first year of life, 51 were
less than 6 months of age, and 6 were less
than 1 month. All children in this group
were judged to have congenital defects
potentially severe enough to cause death
within 6 months of the time of study. In
none of them was the diagnosis established on the basis of clinical findings.
Twenty-seven of these children were
found to have lesions amenable to surgical
procedures. In all but 2 of these the
pre-operative diagnosis, established by cardiac
catheterization, was confirmed by
opera-tion. In none of the group declared to be
inoperable, has a correctable lesion been
demonstrated by subsequent study during
life or by post-mortem examination. A final
statement of diagnostic accuracy cannot
yet be made, but a reasonably conservative estimate based on presently available in-formation is that correct anatomic
diag-noses have been established in at least
80 per cent of the patients studied in this
age group.
. The technic of catheterizing small
chil-dren differs in some respects from that
used in older patients. It is important to
avoid pain and fright. Preliminary sedation
consists of morphine and sodium
pheno-barbital which may be supplemented with
small doses of seconal sodium, given by
way of the catheter, if the child becomes
restless during the procedure. The
saphe-nous vein offers the best approach. It is
easily mobilized through a small incision
over the superficial femorai artery. The
vein is large enough to permit introduction
of the catheter in most patients. In 27
pa-tients in whom it was found to be
made-quate in size, the superficial femoral vein
was used without difficulty. Arterial blood
specimens and pressure tracings are
ob-tamed by puncture of the superficial
femo-ral artery under direct vision. A cardiac
catheter (No. 5) 50 cm. long is routinely
used. During the period of intracardiac
manipulation the electrocardiogram is
con-stantly monitored by means of an
oscillo-scope. Equipment for tracheal intubatiomi
and positive pressure oxygen therapy is
kept available for instant use.
Heart catheterization is essentially an
intracardiac exploration. From the inferior
caval approach it is particularly easy to
reach all areas of the articular septum with the catheter tip. When there is an auricular
septal defect, or patency of the foramen
ovale, which is common in infants less than
6 months of age, it is usually possible to
catheterize the left auricle and ventricle
and the pulmonary veins. The advantage
of being able to measure pressures and
concentrations of oxygen in the blood from
the left side of the heart, particularly in
complex lesions, is obvious. When possible
these determinations are made routinely in
all 4 chambers of the heart and in the
pulmonary artery. When severe pulmonary
stenosis is present, it is occasionally
im-possible to catheterize the pulmonary
artery; and this, of course, cannot be done
in a patient with complete pulmonary
atresia or truncus arteriosus. The tip of the
catheter often passes through the aortic
valve orifice when it is located over the right ventricular outflow tract. If direct
catheterization of the aorta cannot be
ac-complished, the presence of an abnormally placed aortic valve orifice is easily recog-riized by the presence of a short circulation
time, less than 4 seconds, measured from
the catheter tip in the right ventricle to
the tongue. If the ductus arteriosus is
patent it may usually be catheterized from
the pulmonary artery.
The problems encountered in 5 infants
younger than 14 weeks of age illustrate
some of the specific applications of cardiac catheterization to clinical diagnosis in this age group.
Case 1
K. M. (702-225) was 12 weeks of age. At
per-sistent cough, a heart murmur was detected.
His mother noted that lie breathed rapidly
(luring sleep. There was no evidence of cyano-sis or clubbing. Wet rales were present
through-out both lung fields and the liver edge was palpable 2 cm. below the night costal margin. A grade III systolic murmur was loudest in the second to fourth interspaces oml the left at the sternal border. No diastolic murmur was
pnes-ent. The electrocardiogram revealed sinus
tachycandia and incomplete night bundle branch
block. Fluonoscopy showed the heart to be
about 25 per cent larger than normal due to
enlargement of the left auricle, left ventricle
amid right ventricular outflow tract. Hilar
mark-ings were prominent but did not show visible
intrinsic pulsations. The vascular pedicle was obscured by a barge thymus.
Data obtained by catheterization is
shown in Figure 1. There was a large shunt
of arterial blood into the right ventricle due to the presence of an interventricular septal defect. Systolic pressures in the pul-monary artery and right ventricle were
elevated because of the greatly
aug-mented blood flow through the lungs.
Cm-culation time from the right ventricle to
the tongue was more than 4 seconds. Blood
obtained from the femoral artery was
nor-mally saturated with oxygen. The aorta
therefore, arose in normal position from the
left ventricle. The aunicular septum was
intact. The ductus arteriosus was closed.
Case 2
T. M. (741-861) was a 13-week-old child
who presented clinical findings quite similar to those noted in the previously described patient.
Tachypnea and cough had been present for 1
month. The heart murmur, first noted at the
age of 4 weeks, was identical in time, location, intensity, and transmission with that noted in the previous patient. Moist nales were present throughout both lung fields. The
ebectrocardio-gram was normal. Fluonoscopy nevealed 30
per cent cardiac enlargement involving the left auricle and both ventricles. Hilar markings were increased, but “hilar dance” was not pres-ent. Patchy infiltrates were observed in both lung fields.
Catheterization findings are shown in
Figure 2. A large patent ductus arteniosus
was easily catheterized from the pulmonary artery. Blood! specimens from the
pulmo-nary artery demonstrated a lange left to right
shunt through the ductus. There was no
shunt of venous blood into the aorta.
Figure 3 depicts the pressure tracing
as the catheter was withdrawn from the
aorta, through the ductus and pulmonary
artery, to the right ventricular outflow
tract. There is a wide difference in systolic
pressure between the aorta and pulmonary
artery, but the difference in diastolic
pres-sure between the two vessels is small,
cx-plaining the absence of a diastolic
com-ponent in the murmur. The usual
“continu-ous” murmur of a patent ductus anteriosus
is audible only if there is a significant
pres-sure gradient between the aorta and
pul-monary artery during diastole. The dias-tolic component of the murmur is
character-istically absent in the children with a large
patent ductus accompanied by pulmonary
hypertension. The experience with this
child was typical of that of 13 patients
with non-specific systolic murmurs in
whom this curable lesion was demonstrated by direct catheterization of the ductus.
Surgical division of the ductus was
per-formed in each case with 1 postoperative fatality.
Case 3
K. M. (717-753) was first seen at the age of 8 weeks. A heart murmur had been recognized when he was 6 days old. He was asymptomatic. Except for the presence of a grade III systolic murmur at the left sternal border, all physical findings were normal. The electrocardiogram
showed no abnormality. Fluonoscopy did not reveal any cardiac enlargement. The cardiac apex was slightly displaced upward, suggesting right ventricular hypertnophy. The aunieles and left ventricle were not enlarged. Pulmonary vascular markings appeared normal. The
vas-cular pedicle was obscured by the thymus gland. It was thought that he had a functionally benign septal defect and further study was
deferred.
Two weeks later he suddenly became deeply cyanotic and lost consciousness for 5 minutes.
A similar attack 4 days later persisted for
Pulmonary artery
Vena cavo 02 72 O/
02=560/0 68,# mmHg.
Right ventricle
#{176}2 72 O/
. 72/ u
Right auricle , 6 mm g.
02 59 O/
2.5 mmHg. emoral artery
02= 95 O/
fl2/70 mmHg.
Circulation time; Right ventricle to tongue
4.8 seconds. #{176}2Capacity g 13.5 Vol. /o ( lOO#{176}/.)
FIG. 1. Data obtained at cardiac catheterization of patient KM. (702-225) with interventricular septal defect. The tip of the catheter is located in the right inferior pulmonary artery.
Vena cava Patent ductus
02= 48 O/ arteriosus
Right auric Main pulmonary
#{176}2 46 O/ artery
02* 79 O/
a.o
mmHg.46/24 mmHq.
Right ventri
02 46 /o Aorta below
d tic t us
44/3 mmHq 95 O/
80/34 mmHg.
#{176}2Capacity : 12.5 Vol. /o (100#{176}f.)
n.y.
t
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1
50
20
- DUCTUS PA.
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FIG. 3. Pressure tracing of patient TM. (741-861) with patent ductus arteriosus. The changes brought
about by withdrawing the cardiac catheter from the aorta through the ductus into the right ventricle may he seen.
Cardiac catheterization (Fig. 4) dem-onstrated a tetralogy of Falbot. The
aunicu-bar septum was intact and there was no
intracardiac left to night shunt. Circulation
time from the night ventricle to the tongue was abnormally short, imldicating direct
passage of venous blood from the right
ventricle into the aorta. The reduced
oxy-gen content of blood in the femoral artery
confirmed the presence of a right to left
shunt of moderate size. Systolic pressures
in the right ventricle and femonal artery
were nearly equal. The pulmonary artery
was catheterized. Figure 5 is the pressure
tracing recorded as tile catheter was
with-drawn from the pulmonary artery to the
right ventricle, clearly demonstrating the
presence of severe pulmonic stenosis. An
anastomosis was performed between the
aorta and the left pulmonary artery with
an excellent clinical result. Case 4
S. S. (732-966) was a 10-week-old infant who
had been cyanotic since birth. Physical examina-tion disclosed a grade II systolic murmur at the left sternal border. The liver was not enlarged.
The lungs were clear. The electrocardiogram
revealed the presence of imicomplete night
Vena cavo 02 57 #{176}f.
Pulmonary art.
02$ 54 #{176}/o
14,4 mm Hg.
Right auricle
02 55 #{176}/o 3.0 mmHg.
Right ventricle
02 .1.
90/4 mmHq.
Femoral art. 02 R 72 #{176}I.
96/4 m m Hg.
Circulation time ; Right ventricle to tongue
2.8 seconds. 02 Capacity 19 #{176}f0Vol.#{176}fo(100#{176}f.)
bundle branch block. Fluoroscopy demomi- Case 5
strated 25 per cent cardiac enlargement with
increased prominence of the right auricle and
right vemitnicular outflow tract. Hilan markings were slightly enlarged, but intnimisic pulsations
in the pulmonary arteries were not recognized.
#{149}:
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.:
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--1 -‘ I
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FIG. 5. Pressure tracing of patient KM. (717-753) with tetralogy of Fallot. Effect of withdrawing cardiac catheter from pulmonary artery into night ventricle.
Figure 6a shows the tip of the catheter
in the left pulmonary artery. There was no
left to right shunt at the aunicular level.
A patent fonamen ovale allowed access to
the left auricle and pulmonary veins.
Pres-sures in the right ventricle and pulmonary artery were nearly as high as those
meas-ured in the femoral artery. There was a
large left to right shunt at the ventricular
level. Blood taken from the pulmonary
arteries and from the ventricle immediately
below the pulmonary valve showed a very
high oxygen content, indicating that the
pulmonary artery arose from the outflow
tract of the left ventricle.
Figure 6b is a “spot film” taken a few
minutes later after the catheter had been
introduced into the descending aorta
through a patent duetus arteniosus. The
content of oxygen in blood obtained from
the aorta and femonal artery closely
ap-proximated that of blood taken from low in the right ventricle near the tricuspid valve. The diagnosis was complete transpo-sition of the great vessels, intervemitricular sei)tal defect, patent ductus arteniosus, and patent forarnen ovale.
When D. W. (737-287) was 3 weeks old he
began to refuse feedings and developed labored, grunting respirations. Three days later he sud-deniy became deeply cyanotic and lost
con-sciousness.
At the time of admission to the hospital he was moribund and exhibited cyanosis and dis-temition of the neck veins. The liver was
pal-pable 6 cm. below the night costa! margin.
There was generalized edema and the lungs
were wet. The heart was enlarged. Heart rate
varied between 160 and 180. There was a
grade III systolic murmur, loudest in the see-ond and third imiterspaces at the left sternal border. There was also a short grade II late diastolic murmur at the cardiac apex. Arterial
pulses were easily palpable in the right arm, absent in the left arm, and vemy faintly palpable in the legs. Carotid artery pulsations were diffi-cult to evaluate but were palpable. The dcc-trocardiogram revealed sinus rhythm and
in-complete night bundle branch block. Fluoros-copy revealed 40 per cent cardiac enlargement vith reduced amplitude of pulsations. Vascular markings were increased throughout both lung fields, and small parenchmal infiltrates were present bilaterally. No pulsations were visible in the pulmonary arteries. The aortic arch and descending aorta appeared to be on the left.
After 10 days of medical therapy directed at control of congestive manifestations, cardiac catheterization was performed. It was our pne-liminary clinical impression that the child had
O2Capacity *18.8 Vol.#{176}f. (100%)
Fic. 6. Data obtained at cardiac catheterization of patient S.S. (732-966) with transposition of the great vessels, patent ductus arteriosus and interventricular septal defect. a (Upper). The tip of the cardiac catheter is located in the left pulmonary artery. b (Lower). The tip of the catheter has been passed into the (lescendmg aorta by way of the ductus.
Superior veno cava
02#{176}25 .1.
Right auricle
* 25 #{176}fo
2.5 mmHq.
Right ventricle at tricuspid valve
#{176}2#{176}40#{176}f. 72/6 mm Hg.
Main pulmonary artery
0 #{176}87 #{176}Io
66/31 mmHg.
Ventricle below
pulmonary valve
OZ: 84 #{176}fo
70/6 mmHg.
Femoral artery
42 of0 83/53 mmHg.
02 Capacity 18.8 Vol. #{176}fo(100#{176}f.)
Vena cavo
02 #{176}25 #{176}/.
Right aurici
02 25 #{176}/.
2.5 mmHg.
Right ventri at tricuspid valve
02 #{176} #{176}fo
72/6 mmHg.
probably accompamiied by a patent ductus
an-teniosus below the atretie segment.
Figure 7a shows the result of retrograde aontic catheterization from the right super-ficial femoral artery. Pressures in the aorta
Patent ductue
Main pulmonary art cry
O: 87 0/
66/31 mm Hg.
Ventricle below pulmonary valve
02 #{176}84 #{176}f.
TO/I mm Hg.
Pulmonary vein
#{176}2#{176}9 #{176}o
5.0 mm Hg.
Aorta below patent ductus
02 #{176}42 #{176}1. 72/35 mmHg.
inferior to the level of origin of the left
subclavian and carotid arteries were
re-duced. Pressure iii these vessels was found
to be correspondingly low. s’Iuch higher
Catheter In left
Transverse subclovian art.
oortic arch 9O/. mmHg.
152/84 mmHg.
Coarct ed
seg ment
Aorta below coarct at ion
92/72 mmHg.
Venous cathetei
Vena cava Transverse
02*61 #{176}f. aortic arch
02* 90 #{176}/.
. 152/84 mmHg.
Right aurici
02*85 #{176}fo
Left subclavian
2.0 mmHg. 02* 90 #{176}f. 90/70 mmHg.
Pulmonary a
0=85 #{176}fo
Left auricle
70/zs mmHg. 02* 91 #{176}/.
3.5 mm Hg.
Right ventric
1 _ #{248} 01 Aorta below
“2 0 coarctation
70/6 mmHg. 02*900/0
92/72 mm Hg.
02 Capacity * 14.9 Vol. #{176}fC 100#{176}f.)
FIG. 7. Data obtained at cardiac catheterization of patient D.\V. (737-287) with coarctation of the aorta and interatniab septab defect. a (Upper). Retrograde aortic catheterization. The tip of the catheter is located in the left subclavian artery. b (Lower). Venous catheterization. The tip of the catheter is
superior to the level of origin of the left
subclavian and carotid arteries and in the
transverse aontic arch and in the right
carotid artery. The point of obstruction was cleanly demomistrable at the site labeled. It
appeared that there was a short coarcted
segment involving the orifices of the left
subciavian and carotid arteries. Blood
specimens taken above and below the
seg-ment of coanctation were equally saturated
with oxygen. It was not possible to pass
the tip of the catheter into a patent ductus arteniosus inferior to the coareted segment.
Vemious catheterization was performed
with the result shown in Figure 7b. There
was severe pulmonary hypertension and
a large shunt of arterial blood into the
right auricle. The ventricular septum was
imitact and the ductus arteniosus was closed.
At operation, performed shortly
there-after by Dr. Donald B. Effler, a short
coarcted segment at the point of origin of
the left subclavian artery was found. The
ligamentum arteniosum joined the aorta at
the point of coanctation and carried no
blood. It was possible to excise the
co-areted segment and do an end-to-end
anastomosis without diminishing the size of
the orifice of either the subclavian or
ca-rotid arteries. The patient made an
unevent-ful recovery and since operation has
re-mained free of congestive manifestations. The hazard of catheterization in children
of this age is acceptable when one
con-siders the functional severity of the lesions
to which it has been applied. There has
been 1 death attributable to the procedure in this series of patients, a mortality rate
of 0.92 per cent. The death occurred in a
12-month-old girl with intermittent
cyano-sis, more than 50 per cent cardiac
enlarge-ment, evidence of severe pulmonary
hyper-tension, and congestive heart failure. Eight
to ten ml. of 2 per cent procaine
hydro-chloride were injected into the skin at the
site to be incised. Three minutes later the
patient suddenly developed a generalized
clonic convulsion which involved the
dia-phragm. Extreme anoxia developed
im-mediately and was accompanied by
over-whelming pulmonary edema. Within 30 seconds the electrocardiogram showed
veIl-tricular asystole. All efforts at resuscitation, imleluding cardiac niassage, failed. Necropsy revealed the presence of an atnioventnicu-lanis eommunis. This catastrophe occurred
before either the artery or vein had been
mobilized, and is believed to have been
due to an anaphylactoid reaction to
pro-caine hydrochloride in an infant unusually sensitive to the drug. Since this episode a 1 per cent solution of procaine has been
used. No other convulsive seizures have occurred.
A second serious complication occurred
in a cyanotic infant who was studied at
the age of 6 months. The child had survived 3 fainting spells, which were interpreted
as being anoxic attacks. He showed 25 per
cent cardiac enlargement with definite
cvi-dence of right auriculan dilatation and right
ventricular hypertrophy. The pulmonary
arteries were larger than normal, but
in-trmnsic pulsations were not recognized at
the time of fluoroscopy. After blood
speci-mens and pressure measurements had been
obtained from the vena cavae, the right
auricle, and the right ventricle, the catheter was easily directed up the right ventricular
outflow tract to a point immediately below
the usual position of the pulmonary valve.
In this area a soft tissue obstruction was
encountered. Manipulation at this point
caused a short run of premature ventricular
beats, and the catheter was quickly
with-drawn to the right auricle. Sinus rhythm
promptly returned. At this time a loop
2.5 cm. long and 2.0 cm. wide was
recog-nized in the distal segment of the catheter.
The loop could not be straightened, and
was too large to permit withdrawal back
into the inferior vena cava. While the child slept, the available data were rapidly
ana-lyzed. The aunicular septum was intact.
There was definite evidence of left to right
shunt at the ventricular level. Right
yen-tricular pressure was 90/4 mm. Hg, closely
approximating the systolic pressure of the
femoral artery. The complete absence of
pres-ence of such a high right ventricular
pres-sure suggested the presence of pulmonic
stenosis, a dextroposed aorta, and an
in-terventnicular septal defect.
Left anterior thoracotomy was performed by Dr. Effler, in tile hope that an
anastomo-sis between the aorta and pulmonary artery
or a pulmonary valvulotomy could be
per-formed at the time the catheter was
re-moved. The pressure in the pulmonary
artery was found to be low. The great
yes-sels were transposed. Digital examination
of the root of the pulmonary artery
re-vealed the typical findings of valvubar pub-monic stenosis with poststenotic dilatation
of the pulmonary artery. A pursestring
su-ture was placed about the base of the right
auricular appendage. The loop of the
catheter was forced into the auricular
ap-pendage by manipulation from the
saphe-nous vein. The catheter was severed at the
point where it entered the vein and the
loop then easily withdrawn from the aunicu-lan appendage. Routine pulmonary
val-vulotomy was then done. The loop in the
catheter (Fig. 8) was found to be securely
tied by a small band of fibrous and
endo-cardial tissue. Its origin apparently was a
web of chordae tendinae beneath the
sten-otic pulmonary valve, which was pulled
onto the catheter loop when it was rapidly
withdrawn from the right ventricular out-flow tract. This child made an uneventful
recovery. One year later, the depth of
cyanosis was much diminished and there
had been no more anoxic attacks.
No other complications have occurred.
There have been no embobi, no serious or
prolonged arrhythmias, and no local on
systemic infections. We have not observed
ascending thrombosis following ligation of
either the saphenous or superficial femoral
veins and there has been no evidence of
chronic venous insufficiency in the lower
extremity.
SUMMARY
Cardiac catheterization is one of the
most useful diagnostic technics presently
available in the study of congenital heart
disease. Contrary to widely expressed
opinion, it may be safely and
advanta-geously applied to the solution of problems
presented by small infants and children.
The effective control of any disease process
depends in large measure upon early
diag-nosis and treatment. This fundamental
con-cept has often not been squarely faced in
The result is that all too frequently we have
been satisfied merely with treatment of the
survivors of the infantile period. It is true
that tile largest number of children who die
during the first 2 years of life because of
congenital heart disease have lesions that
are not presently amenable to surgical
ap-proach. It is obvious, however, that unless
precise diagnosis is first made possible
within the limited period of their survival,
the outlook for such children will remain
hopeless. The major stimulus for definitive diagnosis in early infancy has been
recog-iiitioii of the fact that a sigmiificant number of children die (luring this period with
undiagnosed lesions that could have been
cured or improved by surgical procedures.
This problem may be largely eradicated by
