Download Use of Contrast Echocardiography for Evaluation of

Use of Contrast Echocardiography for Evaluation
of Right Ventricular Hemodynamics
in the Presence of Ventricular Septal Defects
GERALD A. SERWER, M.D., BRENDA E. ARMSTRONG, M.D., PAGE A.W. ANDERSON, M.D.,
DOUGLAS SHERMAN, B.A., D. WOODROw BENSON, JR., M.D., PH.D., SAM B. EDWARDS, M.D.
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SUMMARY Intracardiac blood flow is altered in the presence of a ventricular septal defect (VSD), with different sizes of
defects producing different flow patterns that can be visualized by peripheral injection contrast echocardiography. The utility
of these patterns in allowing estimation of right ventricular pressure and resistance to ejection of blood from the right ventricle (RV) was investigated. Forty-four patients underwent 46 contrast echocardiographic procedures, all within 24 hours
before cardiac catheterization. All patients were placed in one of four groups based on catheterization findings. Group I,
which consisted of patients with no VSD, or a small VSD with a pulmonary-to-systemic flow ratio (Qp/Qs) < 1.5:1 and
right ventricular pressure < one third systemic, had echo-dense material appear in the RV only. Group II consisted of those
with moderate-sized VSDs with Qp/Qs 2 2.5:1 and right ventricular pressure 60-80% systemic; in these patients echodense material appeared in the RV and in the left ventricle (LV) anterior to the mitral valve. The contrast appeared in the LV
in early diastole and cleared from the LV in systole, with none appearing in the aorta or left atrium. Time from the preceding
QRS to appearance of echo-dense material in the LV divided by the R-R interval (appearance time to R-R interval ratio) was
0.62 0.69. Group III consisted of those with large VSDs, Qp/Qs > 3:1 and systemic right ventricular pressure with low
pulmonary vascular resistance and no pulmonary stenosis. These patients had appearance of echo-dense material in the LV
during early diastole anterior to the mitral valve as in group II, but the material remained in the LV to be ejected into the
aorta. Appearance time to R-R interval ratio was the same as in group II. Group IV consisted of patients with tetrology of
Fallot and one patient with a large VSD and increased pulmonary vascular resistance. Echo-dense material again appeared
in the LV early in diastole and was ejected into the aorta during systole. The appearance time/R-R ratio was significantly
less than in groups II or III, allowing a clear separation between those patients in group III from group IV. Contrast
echocardiography allows estimation of the right ventricular pressure and evaluation of increased resistance to pulmonary
flow due to either pulmonary stenosis (PS) or increased pulmonary vascular resistance in the presence of VSDs. This
technique also provides a minimally invasive method which may be useful for serial evaluation of such patients, and will
permit early detection of developing PS, and possibly increasing pulmonary vascular resistance. Decreasing right ventricular pressure due to spontaneous closure of the VSD may also be detected by this technique.
-
CONTRAST ECHOCARDIOGRAPHY, initially
introduced clinically by Gramiak et al.,' has been used
previously to identify intracardiac structures
validating echocardiographic patterns,2' 3 to identify
various congenital anomalies4-7 and to demonstrate
valvular insufficiency.4 I Furthermore, as the echodense material is easily traced through the cardiac
chambers, significant information concerning intracardiac hemodynamic flow patterns can be obtained. In this manner, right-to-left shunting8 and leftto-right shunting9 can be demonstrated. However, the
emphasis in these previous studies was on the identification of structural defects rather than on the underlying hemodynamic states that existed in the
various forms of congenital heart disease evaluated.
In this study, children with various sizes of ventricular septal defects (VSDs), including large VSDs
with associated pulmonary stenosis or elevated
pulmonary vascular resistance, were evaluated using
the technique of peripheral injection contrast echocardiography. In each patient the flow patterns were cor-
related with the hemodynamic status observed at cardiac catheterization. This correlation demonstrated
that using specific contrast echocardiographic
patterns, the intracardiac pressure-flow relationships
could be determined accurately. In this manner, conclusions concerning the right ventricular and
pulmonary vascular hemodynamic states can be
drawn and serially re-evaluated in patients with VSDs.
Methods
Patient Population
Forty-four patients between the ages of 2 weeks and
16 years comprised the study group. Two patients
each underwent two echocardiographic and
catheterization studies approximately one year apart.
Based on hemodynamic data obtained at catheterization, all patients were grouped into one of four
categories. No patient included in this study had an
atrial septal defect or a communication at the great
vessel level. Catheterization data is presented in
table 1.
I
From the Division of Pediatric Cardiology, Department of
Pediatrics, Duke University Medical Center, Durham, North
Carolina.
Supported in part by NIH grants HLl 1307, HL20677 and
HLO7lOl.
Address for reprints: Gerard A. Serwer, M.D., P.O. Box 3090,
Duke University Medical Center, Durham, North Carolina 27710.
Received November 7, 1977; revision accepted March 31, 1978.
Group I
Patients included in this group (mean age 3.8
years ± 1.1 SEM) had either no VSD (n = 9) or a small
VSD with pulmonary to systemic flow (Qp/Qs) less
than 1.5:1 (n = 8). All but one (patient DR) with
small VSDs had peak right ventricular pressures less
327
VOL 58, No 2, AUGUST 1978
CIRCULATION
328
TABLE 1. Catheterization and Echocardiographic Patient Data
Age
Pt
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Group I
6 wks
CG
DR
6 wks
9 mos
SG
4 yrs
RD
1 yr
JK
KT
6 yrs
TS
16 yrs
2 yrs
DH
3 mos
JM
7 mos
TH
2 yrs
BM
3 yrs
CF
3 yrs
SB
3 yrs
TK
4 yrs
MH
5 yrs
LS
14 yrs
KP
RV press
LV press
Qp
Qs
Ao 02
sat
0.31
0.42
0.26
0.33
1.4
1.4
1.3
1.3
1.1
1.1
1.4
97
94
96
94
98
95
97
1.1
1.0
1.0
1.0
1.0
1.0
1.0
1.0
1.0
1.0
95
93
94
95
0.28
0.28
0.25
0.33
0.40
0.35
0.34
0.63
0.73
0.50
0.27
0.40
0.23
Rp
R,
(Units/m2)
Rs
1.7
2.4
1.1
2.2
1.9
1.1
1.2
3.3
0.11
0.15
0.15
0.10
98
95
98
1.9
1.4
1.1
1.8
1.8
2.8
1.3
2.3
2.6
94
95
94
95
1.6
3.4
1.3
1.0
95
1.3
1.4
1.5
4.9
1.5
95
95
95
Appearance
tpie
R-R
(sec-
Interval
0.25
0.25
0.28
0.27
0.25
0.22
0.30
0.25
0.28
0.38
0.37
0.45
0.43
0.36
0.35
0.45
0.40
0.47
0.67
0.63
0.60
0.26
0.25
0.26
0.27
0.29
0.34
0.27
0.39
0.36
0.38
0.39
0.47
0.54
0.43
0.67
0.69
0.68
0.69
0.62
0.63
0.63
AT
R-R
0.17
0.17
0.10
0.15
Group II
CC
HE
ES
CH
MD
RG
DH
GS
MC*
2
2
4
4
8
8
4
9
11
wks
wks
wks
wks
wks
wks
mos
mos
mos
0.80
0.65
0.70
0.65
0.65
0.63
0.60
0.76
0.64
5.6
2.5
4.6
7.7
2.9
4.0
4.5
3.0
95
2.5
94
5.0
3.2
93
92
3.4
4.0
6.5
3.2
3.0
94
93
93
0.07
0.18
0.09
0.07
0.07
0.14
0.12
0.32
0.17
0.66
0.68
0.62
0.63
0.69
0.63
Group III
MC
RG
JP
JT
SL
Jp*
TA
2
4
6
2
2
11
2
wks
wks
wks
mos
mos
mos
yrs
1.0
1.0
1.0
1.0
1.0
1.0
1.0
1.4
1.5
4.9
2.0
3.6
5.0
2.3
88
87
93
92
0.06
0.12
0.28
0.18
0.10
0.24
0.15
Group IV
0.23
3 mos
1.0
84
0.58
0.24
79
0.71
1.0
5 mos
0.23
92
0.87
6 mos
1.0
0.29
58
0.26
17 mos
1.0
0.26
2 yrs
69
0.54
1.0
0.32
77
0.62
3 yrs
1.0
0.27
3 yrs
56
0.33
1.0
0.31
3 yrs
77
1.0
0.59
0.29
78
3 yrs
0.43
1.0
0.31
82
0.70
6 yrs
1.0
0.31
85
0.60
1.0
9 yrs
0.40
84
15 yrs
0.41
1.0
0.38
0.95
16.7
13 yrs
83
1.0
1.0
*Indicates data of the two follow-up studies.
tlndicates the patient with a VSD and increased pulmonary vascular resistance
Abbreviations: Pt = patient; RV press/LV press = ratio of the RV to LV peak systolic pressure; Qp/Qs
to systemic blood flow; Ao 02 sat = aortic oxygen saturation; Rp = pulmonary vascular resistance; RP/Rs
VJ
MS
AB
BM
MF
ML
FA
CS
JY
DJ
VW
CK
GVt
vascular resistance to the systemic vascular resistance; AT/R-R
to the eiectrocardiographic R-R interval ratio.
than one-third that of the systemic pressure. Those patients with no VSD had valvular pulmonary stenosis.
This ranged from mild to moderate in severity. None
of the patients in this group had any evidence of rightto-left shunting by catheterization techniques.
=
the echo-dense material appearance
0.39
0.41
0.41
0.49
0.44
0.54
0.48
0.54
0.49
0.58
0.56
0.69
0.81
t.me
ratio of the
0.59
0.59
0.56
0.59
0.59
0.59
0.56
0.57
0.59
0.53
0.55
0.58
0.47
pulmonary
ratio of the pulmonary
as defined in the text
Group II
These patients (n = 9, mean age 0.32 ± 0.11 years)
had moderate sized membranous VSDs with a Qp/Qs
ratio . 2.5: 1. All patients had right ventricular
pressures 60%-80% of the systemic pressure. None
CONTRAST ECHO IN VSD/Serwer et al.
had pulmonary stenosis, and all had a pulmonary
vascular resistance less than 5 units/M2 (1 unit = 1
mm Hg/1/min). None had evidence of right-to-left
shunting as demonstrated by either Cardio-Green indicator dilution dye curve, Fick data or angiographic
techniques. However, right ventricular cineangiocardiography did show diastolic flow of radiographic contrast material into the left ventricle. With ventricular
systole, contrast material returned to the right ventricle, and none was ever visualized in the aorta.
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Group III
These patients (n = 7, mean age 0.54 ± 0.29 years)
had large VSDs, with Qp/Qs ratios > 3: 1 and
systemic pressure in the right ventricle. No patient had
pulmonary stenosis. All had a pulmonary vascular
resistance < 5 units/M2. Cineangiocardiography
demonstrated a large left-to-right shunt at the ventricular level. In addition, by right ventricular
cineangiography, a small right-to-left ventricular
shunt was noted with radiographic contrast material
appearing in the aorta. Two of the six patients had
aortic desaturation indicative of a small right-to-left
shunt (< 25%). The two older patients had CardioGreen dye curves performed with injection into the
right ventricle showing small (< 20%) right-to-left
shunts.
Group IV
Of the patients in this group (n = 13, mean age
4.6 ± 1.3 years), 12 had tetralogy of Fallot with rightto-left ventricular level shunting. This was substantiated by right ventricular cineangiocardiography and
aortic desaturation. The remaining patient had an
isolated VSD with an elevated pulmonary vascular
resistance of 16.7 units/m2. The right ventricular and
pulmonary artery peak pressures were systemic. There
was aortic desaturation and right-to-left shunting
demonstrated by right ventricular cineangiography.
Echocardiographic Examination
Patients underwent peripheral injection contrast
echocardiography utilizing the microcavitation effect'0
within 24 hours before cardiac catheterization. Injections of either isotonic saline or the patient's blood
were made into either a leg or arm peripheral vein.
The injections did not exceed 2 ml of injectate per injection. Injections which produced the most reproducible records were made through either an 18 or 20
gauge indwelling catheter. Smaller catheters or
needles produced inconsistent echo-dense material.
Larger catheters also did not produce satisfactory
results due to inconsistent production of echo-dense
material. Echocardiographic examinations were performed with the patient in the supine position with the
EchoCardioVisor echocardiograph interfaced with a
Honeywell LS-6 strip chart recorder. A 4.5 mHz nonfocused transducer was used for all studies. Records
were recorded at paper speeds of 50 mm/sec and 100
mm/sec. Initial injections were recorded at a paper
329
speed of 50 mm/sec. If echo-dense material was then
found to be present in the left ventricle, repeat injections were recorded at a paper speed of 100 mm/sec.
for measurement of the time intervals described
below. Timing marks were inscribed on the paper
every 500 msec. Time mark calibration with an external time base generator was performed periodically to
ensure accuracy of the internal time base generator.
Two echocardiographic views were utilized for each
patient. Position #1 visualized the right ventricular
cavity, septum, left ventricular cavity and mitral
valve. Position #2 visualized the right ventricular outflow tract, aorta and left atrium. The reject control
was initially set at its minimal setting and the time
gain compensation controls adjusted to produce an
adequate echocardiographic record. The reject control
was gradually increased to the point that the overload
phenomenon, as described by Valdes-Cruz et al.,8 9
was eliminated while still detecting echo-dense material produced by the injection. The patients' electrocardiograms were recorded simultaneously for timing purposes. Diagnostic records were obtained on all
patients examined.
All records were independently reviewed by at least
three of the authors, and the results were collated
before cardiac catheterization. All records were
assessed for 1) presence or absence of echo-dense
material in each chamber, and 2) the time of
appearance of echo-dense material in the left ventricle.
This appearance time was measured from the onset of
the preceding QRS to the initial appearance of the
echo-dense material in the left ventricle measured at
the level of the left ventricular septal endocardium
(fig. 1). This value was expressed both in absolute time
(seconds) and as a fraction of the R-R interval. This
measurement was made on the first cardiac cycle
following the appearance of echo-dense material in the
right ventricle. The echo-dense material was allowed
to clear from the heart before the injection was
repeated. A minimum of three determinations were
performed for each patient with the results averaged.
It is emphasized that this measurement was made at
the first appearance of echo-dense material in the left
ventricle during the first cardiac cycle following its
arrival in the right ventricle. Thus, there was no
residual echo-dense material in the left ventricle
preceding the examined beat to obscure the correct
point for measurement. Statistical analyses were performed using the Student t test.
Catheterization Techniques
Hemodynamic data was obtained at routine cardiac
catheterization performed under sedation with
meperidine, promethazine and chlorpromazine.
Pressure data was obtained with a fluid-filled catheter
system before angiography. The zero reference was set
at the mid-chest level. Left and right ventricular
pressures were measured with the same cathetertransducer system in quick succession (< 5 beats) via
either a pullback across the VSD or introduction of
the venous catheter into the left heart via a probe pa-
CIRCULATION
330
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,~~~~~~~~~~~2
VOL 58, No 2, AUGUST 1978
............_-
FIGURE 1. Record from patient in Group III showing the method of measurement of the echo-dense
material appearance time. A T appearance time; R-R = electrocardiographic R-R interval. Paper speed
is 100 mm/sec.
tent foramen ovale. Pulmonary and systemic blood
flows were calculated utilizing Fick techniques. All
blood samples were obtained in rapid succession
before angiography and 02 consumption was assumed
based upon the work of Fixler et al.14 Pulmonary and
systemic resistance was calculated from the pressure
and flow data expressed both in absolute terms
(units/m2) and as a ratio of pulmonary to systemic
resistance (Rp/Rs). Presence of right-to-left and leftto-right shunting was evaluated by utilizing Fick
techniques, indicator dilution Cardio-Green dye
curves, radionuclide techniques and cineangiocardiography. Shunting detected by cineangiocardiography was evaluated for only those beats with
normal conduction. Thus, the effect of premature ventricular contractions was eliminated. All data were independently reviewed by at least two authors without
knowledge of the echocardiographic results.
Results
Group I
Contrast echocardiography in patients of this group
(fig. 2) demonstrated the appearance of echo-dense
material only within the right ventricular cavity and
right ventricular outflow tract. No echo-dense
material was seen within the left atrium, left ventricle
or the aorta. All patients, whether they had an intact
ventricular septum or a small VSD, exhibited the
same contrast echocardiographic pattern.
Group 11
Contrast echocardiography (fig. 3) demonstrated
the initial appearance of the echo-dense material
within the right ventricular cavity. During the first
ventricular diastole following appearance of the echodense material in the right ventricle, this echo-dense
material appeared in the left ventricular cavity
anterior to the mitral valve at the peak of the mitral
valve opening. This was a consistent finding in all
patients of this group. With the onset of ventricular
systole and closure of the mitral valve, this echo-dense
material cleared from the left ventricular cavity.
Moreover, no echo-dense material ever appeared in
the aorta or left atrium. This correlates with the
results of the right ventricular cineangiogram in which
there was opacification of the left ventricle during
diastole with no radiopaque material appeating in
either the left atrium or aorta. The interval from the
QRS to the left ventricular appearance of the echodense material (echo-dense appearance time) varied
from 0.22-0.30 seconds, depending on the heart rate
(table 1). The ratio of the appearance time to the R-R
interval (appearance time/R-R interval ratio) varied
from 0.62-0.69, mean 0.65 + 0.01 (sEM). There was
no statistical correlation of this ratio with heart rate
for the patients in this group, P > 0.5 (fig. 4).
Group IlI
Contrast echocardiography (fig. 5) demonstrated
initial appearance in the right ventricle followed by
CONTRAST ECHO IN VSD/Serwer et al.
331
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,,
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*t\LV T
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w.~~~~~~~~~~ _
FIGURE 2. Record from a patient in Group l. Panel A shows echo-dense material only in the R Vfirst
appearing at the arrow. Panel B shows echo-dense material only within the right ventricular outflow tract.
Simultaneous ECG is shown only for panel A. R V right ventricle; LV left ventricle; MV mitral
valve; R VOFT = right ventricular outflow tract; Ao = aorta; LA = left atrium.
=
appearance of echo-dense material in the left ventricle
during the first ventricular diastole following its
appearance in the right ventricle. Echo-dense material
appeared in the left ventricle at the peak of the mitral
valve opening anterior to the mitral valve echo (fig.
5A). In contrast to group II, it remained in the left
ventricle during ventricular systole and subsequently
appeared in the aorta (fig. 5B). Echo-dense
appearance time ranged from 0.25-0.34 seconds with
the appearance time/R-R interval ratio ranging from
0.62 to 0.69, mean 0.66 ± 0.01. There was no significant difference in this ratio between groups IL and III
(fig. 6). Again, for those patients in group III, there
was no relationship to heart rate (P > 0.5, fig. 4).
332
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VOL 58, No 2, AUGUST 1978
CIRCULATION
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.
FIGURE 3. Recordfrom a patient in Group II with echo-dense material appearing in the R Vand L Vat the
arrow. Note that there is no echo-dense material in the L V during systole. R V = right ventricle; L V - left
ventricle; MV = mitral valve.
oup IV
Two patients, JP and MC, in this group each underwent a second study. JP continued to exhibit a flow
pattern characteristic of group III patients. MC, at
restudy, exhibited a flow pattern characteristic of
group II patients. The catheterization data did show a
decrease in the right ventricular pressure of MC and
no change in the hemodynamic status of JP.
.70
Pontrast echocardiography (fig. 7) demonstrated
tial appearance of echo-dense material in the right
itricle, which then appeared in the left ventricle
Lerior to the mitral valve during the first ventricular
Lstole in all patients; just as in the preceding two
ups. It remained in the left ventricular cavity dur-
O
O
K0
0
S
S
0
6
0
09
0
c
AA
A
"I
S
FIGURE 4. Plot of heart rate (beats/min) vs
appearance time/R-R interval ratio. There
is no correlation of these variables for
patients within each group. The follow-up
data of the two patients studied twice is not
included.
A
Ad
A
.57
a
A
A
A
A
Cl
CL
cr
* Group !
o Group M
a Group I
A6
.45
I
I
75
125
Heart Rate
I
175
CONTRAST ECHO IN VSD/Serwer et al.
333
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FIGURE 5. Recordfrom apatientin Group IIIshowingappearance of echo-densematerialin theRV, LV,
right ventricular outflow tract and aorta as indicated by the arrow. Panel A shows appearance ofecho-dense
material in the L Vduring diastole and its remaining there during systole. Panel B shows appearance of echodense material initially in the right ventricular outflow tract and then in the aorta. The ECG has been
omitted from panel B. R V = right ventricle; L V = left ventricle; MV = mitral valve; R VOFT - right ventricle outflow tract; Ao = aorta; LA - left atrium.
ing ventricular systole and was visualized within the
as in group III. Echo-dense material appearance
time ranged from 0.23-0.40 seconds with the
appearance time/R-R interval ratio ranging from
0.47-0.59, mean 0.57 ± 0.01. The ratio was
statistically different (P < 0.001) from the values
found for groups II and III patients (fig. 6). For
patients in this group, the ratio was also independent
of heart rate, as in groups II and III (fig. 4). The effect
of heart rate on this ratio must be evaluated only
aorta
within each group. Combining groups II, III and IV
indicates an apparent correlation of this ratio with
heart rate. This is because the patients in groups II
and III are significantly younger than those patients in
group IV. Therefore, group II and III patients have
higher heart rates than those patients in group IV, by
virtue of age, and larger ratios by virtue of their
different hemodynamic status. This leads to the false
impression that the ratio increases with increasing
heart rate.
t
VOL 58, No 2, AUGUST 1978
CIRCULATION
334
0.70
*
S
~
~~~~~~~
*
es
0~~~~~~~~~
* i 0.65
0.65~~~~~
c
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05
0)
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0.57
0
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0)
0.
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0.45
I
Group E
- -1
Group m
Group I7
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FIGURE 6. Plot of appearance time/R-R interval ratio of each group. The follow-up data of the two patients studied twice as not been included. The brackets indicate the standard error of the mean; the mean
value is indicated by the number. There is no significant difference between Groups II and III. There is a
highly significant difference between Groups II and III and Group IV (P< 0.001).
Discussion
In this study the patterns of intracardiac blood flow,
demonstrated by peripheral injection contrast
echocardiography, showed clear differences which
were directly related to the underlying hemodynamic
status. Specifically, the patterns demonstrated
reflected alterations in the right ventricular
hemodynamics as influenced by the presence and size
of the VSD and the degree of obstruction to
pulmonary blood flow secondary to either pulmonary
stenosis or, in one instance, elevated pulmonary
vascular resistance. The four groups represented were
easily separated by this technique and represent
significantly different hemodynamic situations. As a
result, the hemodynamic significance of a VSD can be
assessed and subsequent changes in a patient's
hemodynamic state can be monitored using contrast
echocardiography.
In patients possessing either intact ventricular septa
or small VSDs, (group I), there was no evidence by
contrast echocardiography of intracardiac shunting
from the right ventricle to the left ventricle. These
results were confirmed by cardiac catheterization
techniques that demonstrated no intracardiac blood
flow from the right to the left ventricle. All patients
who had VSDs in this group had low right ventricular
pressures with small left-to-right shunts at catheterization.
In group II patients, moderate sized VSDs with
moderate elevation of the right ventricular pressure,
there was a transient flow of blood from the right to
the left ventricle that could readily and easily be identified by contrast echocardiography. The patients in
this group were easily separated from those in group I.
There was a flow of echo-dense material from the right
to the left ventricle even though there was no pulmonic
stenosis or elevation of the pulmonary vascular
resistance present. This is a particularly important
point, in that previous descriptions of contrast
echocardiography have reported such a right ventricular to left ventricular flow only in the presence of
pulmonic stenosis or severe pulmonary vascular
obstructive disease.8 11
Continuing the spectrum from normal to systemic
right ventricular pressure, the patients in group III
possessed systemic right ventricular pressures with
large VSDs. Again, there was a flow of echo-dense
material from the right to the left ventricle even
though no patient had elevated pulmonary vascular
resistance or pulmonary stenosis. Although the time
of appearance of contrast in the left ventricle was the
same as in group II, these patients were clearly and
easily distinguished from those patients in group II by
appearance of echo-dense material within the aorta in
all group III patients. This never occured in those patients with moderate sized VSDs (group II).
Finally, group IV patients exhibited significantly
earlier appearance of echo-dense material in the left
ventricle than did those patients in group III; Again,
all these patients had large VSDs, as did the patients
in group III, but the two groups had markedly
different hemodynamics. The patients in group IV had
obstruction to pulmonary blood flow due to either
severe pulmonary stenosis or elevated pulmonary
vascular resistance, while those patients in group III
had no such obstruction.
Thus, patients with large VSDs and right ventricular hypertension could be separated by the flow
patterns of echo-dense material into two groups: 1)
one group with hyperkinetic pulmonary hypertension
and low pulmonary vascular resistance and 2) another
group with systemic right ventricular pressure with
obstruction to pulmonary blood flow due to either
CONTRAST ECHO IN VSD/Serwer et al.
335
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FIGURE 7. Recordfrom a patient in Group IVshowing early diastolic appearance of echo-dense material
in the L V at the arrow with it remaining in the L V during systole. R V = right ventricle; L V = left ventricle;
MV mitral valve.
elevated pulmonary vascular resistance or pulmonary
stenosis. There were no false positive or negative
results in this study.
The patterns of intracardiac shunting in the
presence of VSDs were first presented by Levin et
al.'2 13 Angiographically, they showed this same spectrum - no right-to-left ventricular flow with low right
ventricular pressure, transient spilling of blood from
the right ventricle to the left ventricle with moderate
right ventricular pressure elevation, and, finally, net
right-to-left shunting with progressive elevation of the
right ventricular pressure. This echocardiographic
study demonstrates the same spectrum as their study.
The one difference between their study and this study
is the timing of the onset of the right ventricular to the
left ventricular flow in those patients with moderate
and large VSDs. They found it began during
isovolumic relaxation, whereas we first observed the
shunt just after mitral valve opening. The difference is
probably related to the limited area being observed by
the echo beam.
Two patients, JP and MC, were each studied on two
occasions - first in the neonatal period and second
near I year of age. Both patients initially showed flow
patterns indicative of systemic right ventricular pressure but no obstruction to pulmonary blood flow
(group III). At catheterization, both had systemic
right ventricular pressure with large left-to-right
shunts and normal pulmonary vascular resistance. At
restudy, both were doing well clinically. Patient JP
still showed the echocardiographic flow pattern of
group 1II. Subsequent cardiac catheterization verified
the persistence of systemic right ventricular pressure
with no pulmonary stenosis and no change in
pulmonary resistance, and complete repair was undertaken to avoid the development of elevated pulmonary
vascular resistance. However, at restudy MC showed
the echocardiographic flow pattern of group II, pointing
toward a decrease in the right ventricular pressure. This
was confirmed at cardiac catheterization (right ventricular pressure 2/3 systemic pressure), and repair was
not felt to be indicated, as there was no risk of elevated
pulmonary vascular resistance developing. The patient's
subsequent clinical course has supported this decision.
Once the validity of contrast echocardiography is established, the need for repeat cardiac catheterization in
patients such as MC could be obviated. Conversely, if a
patient were to show a change from flow patterns
characteristic of group II to those characteristic of group
III or group III to group IV, then cardiac catheterization
should be considered, as this change would point to a
changing hemodynamic state.
The patterns presented in this study are not presented
primarily as a means of diagnosing structural heart disease, although certain implications concerning structure
certainly can be drawn. The major point is that these distinct flow patterns are indicative of specific hemodynamic
situations and are extremely valuable in evaluating the
hemodynamic status of the patient, particularly on a
serial basis. These studies are readily and easily performed in the outpatient setting and provide an accurate
means of serially assessing the hemodynamic status in
CI RCULATION
336
such patients to determine the best course of future
medical management.
Acknowledgment
Appreciation is expressed to Mrs. Pat Parker for her technical
assistance.
References
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5. Pieroni D, Varghese PJ, Rowe RD: Echocardiography to detect
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Two-Dimensional Echocardiographic
Assessment of Ebstein's Anomaly
THOMAS A. PORTS, M.D., NORMAN H. SILVERMAN, M.D.,
AND
NELSON B. SCHILLER, M.D.
SUMMARY Nine patients with Ebstein's anomaly of the tricuspid valve were studied by two-dimensional echocardiography, using the standard long and short axis views as well as the apex four chamber view. With this latter view, the displacement of the tricuspid valve into the right ventricle was clearly seen in all nine cases of Ebstein's anomaly and was not noted in
a control population. The severity of the tricuspid displacement was assessed by comparing the position of the mitral and
tricuspid valves relative to the cardiac apex. The apex four chamber view allowed visualization of the atrioventricular (AV)
ring simultaneously with the displaced tricuspid valve, and therefore the size of the "atrialized" right ventricle, true right ventricle and right atrium could be determined. These dimensions compared favorably with angiography.
EBSTEIN'S ANOMALY of the tricuspid valve is a
rare congenital heart disorder. It consists of
downward displacement of all or part of a malformed
tricuspid valve and associated architectural abnormalities of the right ventricle. Recent reviews have
stressed the pathologic and clinical spectrum of this
disorder.1-3 In the newborn it is frequently accompanied by cyanosis and heart failure, and must be
differentiated from other forms of cyanotic heart disease.4 Patients with milder forms may remain asymptomatic, even in adulthood. This varied expression
often makes the clinical diagnosis difficult, and a nonFrom the Department of Medicine, Cardiovascular Division, the
Department of Pediatrics, and the Cardiovascular Research
Institute, University of California, San Francisco, California.
Address for reprints: Thomas A. Ports, M.D., Cardiology, 1186Moffitt Hospital, University of California, San Francisco, California 94143.
Received January 20, 1978; revision accepted April 16, 1978.
invasive means to diagnose and accurately assess the
severity of this disorder is important. Phonocardiography, M-mode echocardiography and, more recently,
two-dimensional imaging systems have proved useful
in the diagnosis of Ebstein's anomaly, but fail to estimate its severity. We evaluated nine patients with
Ebstein's anomaly, using a wide-angle (80°) phasedarray sector scanner, using both conventional precordial images and apex echocardiography to define the
anatomic severity of this deformity.
Materials and Methods
Nine patients with Ebstein's anomaly were studied.
They ranged in age from 6 hours to 32 years. Clinical
severity was variable, ranging from asymptomatic in
four to severely symptomatic with cyanosis and/or
congestive heart failure in three. Cardiac catheterization and biplane cineangiographic data were available
for all patients. The diagnosis of Ebstein's anomaly
Use of contrast echocardiography for evaluation of right ventricular hemodynamics in
the presence of ventricular septal defects.
G A Serwer, B E Armstrong, P A Anderson, D Sherman, D W Benson and S B Edwards
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Circulation. 1978;58:327-336
doi: 10.1161/01.CIR.58.2.327
Circulation is published by the American Heart Association, 7272 Greenville Avenue, Dallas, TX 75231
Copyright © 1978 American Heart Association, Inc. All rights reserved.
Print ISSN: 0009-7322. Online ISSN: 1524-4539
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the World Wide Web at:
http://circ.ahajournals.org/content/58/2/327.citation
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