Download Right Ventricular Volume Determinations in 18 Patients

Right Ventricular Volume Determinations
in 18 Patients with Pulmonary Atresia
and Intact Ventricular Septum
Analysis of Factors Influencing
Right Ventricular Growth
RAMAN G. PATEL, M.B., ROBERT M. FREEDOM, M.D., C.A.F. MOES, M.D.,
KENNETH R. BLOOM, M.D., PETER M. OLLEY, M.D., W.G. WILLIAMS, M.D.,
GEORGE A. TRUSLER, M.D., AND RICHARD D. ROWE, M.D.
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SUMMARY Right ventricular growth was assessed angiocardiographically in 18 patients with pulmonary
atresia, intact ventricular septum, and hypoplastic and hypertensive right ventricle. A variety of surgical procedures were performed. In only 12 patients (66.7%) was right ventricular-pulmonary artery continuity
achieved (group 1). Nine of these 12 patients persisted with systemic or suprasystemic right ventricular
pressures. Among the six patients in whom right ventricular-pulmonary artery continuity was not achieved
(group 2), all maintained suprasystemic right ventricular pressures.
Right ventricular growth was assessed in groups 1 and 2. The patients were also subdivided according to the
qualitative degree of tricuspid regurgitation as determined angiocardiographically on right ventricular
cineangiocardiograms at the preoperative catheter study. Right ventricular growth to normal levels as
evidenced by change in right ventricular end-diastolic volume was rarely observed in group 2 patients. Among
the four patients with severe tricuspid regurgitation and a large tricuspid valve, right ventricular growth to normal levels was achieved whether they were in group 1 or group 2. Right ventricular growth is thus predicated on
numerous morphologic factors in these patients. However, reconstitution of right ventricular-pulmonary artery
continuity and a nonobstructive tricuspid valve are probably two of the more important factors.
ALTHOUGH the morphologic, clinical, angiocardiographic and surgical features of the patient with
pulmonary atresia, intact ventricular septum and
small or diminutive right ventricle are extensively
recorded, surgical results in this group of patients are,
for the most part, disappointing.'-50 Survival for even
a short time depends on augmentation or maintenance
of an adequate pulmonary blood flow, and several institutions have summarized their experience in this
regard. Yet long-term functional and hemodynamic
improvement, predicated in part on right ventricular
growth, is seldom achieved.31 37, 51-56
To extend these observations, we analyzed the
hemodynamics, angiocardiograms, angiocardiographically derived ventricular volumes, and types of
surgical intervention in 18 patients with pulmonary
atresia, intact ventricular septum and small right ventricle who survived initial operation and have had at
least one postoperative complete hemodynamic and
angiocardiographic investigation.
From the Division of Cardiology, Departments of Pediatrics,
Pathology, Surgery and Radiology, The Hospital for Sick Children,
and the Departments of Pediatrics, Pathology, Surgery and
Radiology, University of Toronto, Toronto, Ontario, Canada.
Presented in part at the 51st Scientific Sessions of the American
Heart Association, Dallas, Texas, November 1978.
Address for correspondence: Robert M. Freedom, M.D., The
Hospital for Sick Children, 555 University Avenue, Toronto, Ontario, Canada M5G 1X8.
Received January 17, 1979; revision accepted August 8, 1979.
Circulation 61, No. 2, 1980.
428
Materials and Methods
Eighteen patients with pulmonary atresia, intact
ventricular septum, and hypoplastic and hypertensive
right ventricle form the basis of this study. All had an
initial hemodynamic and angiocardiographic investigation, followed by surgical intervention, and
finally a postoperative complete catheter hemodynamic and angiocardiographic investigation. Some
of these patients have been included in previous
reports from this institution.21 27, 32, 56 Six of the patients were boys and 12 were girls. The age at the initial study ranged from 1 day to 7 months (median 1
day; mean 20 days), and only two were older than 1
month. At the second cardiac catheter investigation,
their ages ranged from 7 months to 9 years (median
21/2 years; mean 3 years).
All 18 patients underwent balloon septostomy and
five were treated by prostaglandin E, and E2 infusion
before surgery.57 The number and types of surgical
procedures are presented in table 1. At the initial
operation, a transarterial pulmonary valvotomy was
performed in 15 patients, and the transventricular approach was used in one. Twelve of the 18 patients
(61%) had a pulmonary valvotomy combined with a
systemic-pulmonary artery shunt as the initial surgical
therapy. One patient initially had only a pulmonary
valvotomy but subsequently underwent a cavopulmonary shunt. Five patients initially underwent a
systemic-to-pulmonary artery anastomosis without
pulmonary valvotomy. Pericardial reconstruction of
the right ventricular outflow tract was performed as a
subsequent operation in six patients (ranging in age
RV VOLUME DETERMINATIONS/Patel et al.
429
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TABLE 1. Pre- and Postoperative Hemodynamics and Operative Procedures in 18 Patients with Pulmonary
Atresia and Intact Ventricular Septum
Preoperative hemodynamics
Postoperative hemodynamics
Pressures
Pressures
(mm Hg)
(mm Hg)
Additional
Group
Case
Age
RV/LV
Operation RV/LV/PA
operation
Outcome
1A
1
1 day
75/80
4, 6
75/111
A
2
4 mos
79/66
4, 6
184/111/75
9
A
3
1 day
120/70
4, 6
180/97/30
8, 5
A
4
1 day
95/73
4, 6
123/95/14
8, 5
A
5
3 wks
108/85
110/90
7
4, 7
D
1B
6
1 day
105/83
4, 6
54/96/38
A
7
6 mos
114/99
4
145/115
10
D
8
1 day
146/86
4, 6
54/100/24
A
9
1 day
117/58
4, 7
148/100/22
9
A
iC
10
1 day
80/66
4, 6
140/75
D
11
1 wk
92/73
4, 6
104/88/27
A
12
1 day
125/60
5, 7
115/100/75
9
D
2A
13
1 day
120/65
4, 6
154/90/15
9
D
14
3 days
127/62
6
145/92/15
4, 10
A
15
3 days
110/60
7
148/92
5
A
16
1 day
128/72
4
160/82
8
D
17
1 day
120/75
4
196/78
8, 5
9
D
18
2C
1 day
100/60
4
166/113
5
D
Abbreviations: RV = right ventricular; LV = left ventricular; PA = pulmonary artery; A = alive; D died. For operations: 4 = pulmonary valvotomy (arterial approach); 5 = pulmonary valvotomy (transventricular); 6 = Potts procedure; 7 = aortopulmonary shunt (Waterston 1, aortopulmonary grafts 2); 8 =
Blalock-Taussig procedure; 9 = right ventricular outflow patch/conduit reconstruction; 10 = Glenn procedure.
from 4 months to 10 years; median 3 years), with two
survivors (currently ages 6 and 7 years).
Right and left ventricular or aortic pressures were
measured at both cardiac catheterizations using standard fluid-filled catheters.`8 Complete oximetric data
were obtained using Water's Oximeter (The Waters
Co.) or co-oximeter IL 82 (Instrumentation Lab), and
arterial Po2 (mm Hg) was recorded in all patients.
Biplane right and left ventricular cineangiocardiograms were recorded at 60 frames/sec in both
anteroposterior and lateral projections and were used
for volume determinations. Right and left ventricular
ejection fractions were derived from these data. When
magnification could not be corrected for by the usual
1.0-cm grid, we used the vertebral grid system for correction of magnification as described by Wagner
and colleagues.59 Right ventricular volumes were
calculated from tracings of cineangiocardiograms of
the right ventricle in end-diastole and end-systole by
tracing with a sonic pen interfaced with Nova Computer, using Graham's modification of Simpson's
rule.51 60, 61 Similar to the technique advocated by
Graham and co-workers, the outermost borders of the
right ventricle were traced for volume determinations,
even though this might result in overestimation of
right ventricular volumes. Right ventricular volumes
were corrected for surface area and were also ex-
pressed as a percentage of the expected normal using
the regression equations derived by Graham et al.
Measurements of right ventricular inflow were obtained from biplane cineangiocardiograms in the
anteroposterior projection in diastole (fig. 1). The
amplitude of the tricuspid valve, derived from standard echocardiographic techniques, was obtained in
11 of the 18 patients.62"65 Necropsy examination was
performed in six of the eight patients who died after
the postoperative hemodynamic and angiocardiographic investigation. The diameter of the tricupsid
valve was recorded, and the postmortem dimensions
were compared with the normal values of Rowlatt et
al 66
Right ventricular growth was then evaluated in two
major groups of patients. Group 1 includes patients in
whom right ventricular-pulmonary artery continuity
was surgically achieved, as demonstrated unequivocally by the postoperative hemodynamic and
angiocardiographic study. Patients in whom right ventricular-pulmonary continuity was not achieved comprise group 2 (tables 1-3).
We also assessed the role of tricuspid regurgitation
qualitatively at the initial catheter study in promoting
right ventricular growth. Since all 18 patients had
pulmonary atresia, we hypothesized that there might
be a relationship between the degree of tricuspid
CIRCULATION
430
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FIGURE 1. Case 10. Right ventriculogram from a neonate
with pulmonary atresia, intact ventricular septum, and
nearly normal right ventricular cavity (R V). Tricuspid
regurgitation is severe, with dense opacification of a very
enlarged right atrium (RA). The tricuspid valve inflow is
clearly profiled in this diastolic frame (black arrows). The
dotted black line drawn through points a and b measures the
maximum tricuspid inflow.
regurgitation, tricuspid valve or annulus size and ultimately, right ventricular size and growth. Tricuspid
regurgitation was assessed qualitatively by selective
right ventricular cineangiocardiograms filmed at 60
frames/sec. Only ectopic-free frames were evaluated
for regurgitation. Tricuspid regurgitation was judged
independently by three of the authors as mild, indicating faint opacification of the right atrium;
moderate if there was denser opacification of a
somewhat larger right atrium; and severe if there was
very dense opacification of a very enlarged right
atrium.
In the tables, mild tricuspid regurgitation is indicated by the designation A, a moderate degree of
tricuspid regurgitation by B, and severe regurgitation
by C. Thus group IA, B, or C indicates patients in
whom right ventricular-pulmonary artery continuity
was surgically achieved, and in whom the initial
catheter study indicated mild, moderate, or severe
tricuspid regurgitation. Group 2A or C identifies
patients in whom continuity was not achieved, and in
whom tricuspid regurgitation was similarly judged
as mild or severe. No patient had right ventricular-pulmonary artery discontinuity and moderate
tricuspid regurgitation. (This notation is used
throughout the text and in tables 1-3.)
Results
Identifying data, separating of patients in two
1 and 2 (presence or absence of right ventricular-pulmonary artery continuity), and their subgroups based on the initial degree of tricuspid
groups
VoI 61, No 2, FEBRUARY 1980
regurgitation, types of surgery and pre- and postoperative right ventricular pressures are shown in
table 1. Right ventricular-pulmonary artery continuity was established in 12 of the 16 patients who
had initial pulmonary valvotomy (group 1).
Preoperatively, 11 of these 12 had suprasystemic right
ventricular pressures (ranging from 115-208% of
peak systemic systolic pressure [mean 143%]).
Postoperatively, right ventricular pressures were still
either systemic or suprasystemic in nine patients
(ranging from 115-186% of peak systemic systolic
pressure). In two patients, the right ventricular peak
systolic pressures was 54 mm Hg. The pulmonary
artery was not probed during the initial study. Peak
systolic pulmonary artery pressures ranged from
14-75 mm Hg (mean 31.8 mm Hg) at the
postoperative investigation.
Six patients (33%) had right ventricular-pulmonary artery discontinuity (group 2) at the postoperative cardiac catheterization. Severe infundibular
pulmonary stenosis or atresia had precluded
attempted pulmonary valvotomy in five of these six.
Right ventricular pressure remained suprasystemic in
all six group 2 patients (table 1).
Systemic arterial Po2 ranged from 17-31 torr
(mean 24 torr) at the initial study in group 1 (before
prostaglandin administration), and at the subsequent
study ranged from 32-74 torr (mean 58 torr). Group 2
had a similar initial range (24-32 torr, mean 28 torr);
at the subsequent study, systemic Po2 was lower, ranging from 25-48 torr (mean 40 torr).
Right Ventricular Volume Determinations
Preoperative right ventricular volumes calculated
by Simpson's rule and modified by Graham et al.51' 60
reflect the severity of right ventricular hypoplasia
(tables 2 and 3). Right ventricular end-diastolic
volumes (RVEDV) were less than 2.0 ml in four
patients (tables 2 and 3). Since RVEDV increases with
age and body surface area, the right ventricular
volumes are expressed as ml/m2 as well as percentage
of expected normal, using the regression equations of
Graham and colleagues51 60 (tables 2 and 3).
The smallest RVEDVs were observed in the
patients with mild and moderate tricuspid regurgitation (and presumably more obstructive tricuspid
valves). Thus, patients in groups IA, lB and 2A, as
shown in table 2 (individually) and summarized by
groups in table 3, had the smallest RVEDV per square
meter at the initial study, ranging from 11-22 ml/m2,
or 19-45% of normal (tables 2 and 3). The changes in
RVEDV vs body surface area are shown in figures 2A
and B. In figure 2, the patients are divided into groups
1 and 2 and subgroups A-E. Right ventricular growth
was maximal in patients with the most severe tricuspid
regurgitation, whether or not right ventricular-pulmonary artery continuity was achieved. Right
ventricular growth was least in group 2 patients who
had mild tricuspid regurgitation (fig. 2B; table 3).
Similarly, right ventricular growth was unsatisfactory
in patients with a perforate but severely obstructive
RV VOLUME DETERMINATIONS/Patel et al.
right ventricular outflow tract and mild or moderate
tricuspid regurgitation. Although an increment in
right ventricular dimensions was observed in patients
with initially moderate degrees of tricuspid regurgitation and continuity between the right ventricle and the
pulmonary artery, right ventricular dimensions
assumed nearly normal values at the postoperative
431
study in only five patients (figs. 2A and B; tables 2
and 3).
Right ventricular ejection fraction was subnormal
at the initial study and improved in most groups (table
3). The ejection fraction in the presence of tricuspid
regurgitation may be spuriously high, but the relative
improvement is encouraging. A summary of pre- and
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TABLE 2. Pre- and Postoperative Right Ventricular End-Diastolic Determinations and Tricuspid Valve Annulus,
Amplitude and Orifice Measurements in Pulmonary Atresia and Intact Ventricular Septum
Angio
RVED
RV volume
TVA
SA
(m1/M2)
volume
(% of normal)
Case
Age
(M2)
(mm)
ED
Group
1A
1
1 day
0.19
8
14.2
33
12
8 mos
0.35
14
31
2
4 mos
0.24
8
13.8
33
12
5 yrs
0.59
17.8
32
3
1 day
0.19
9
17.8
45
11
0.64
5 yrs
19.6
35
4
1 day
0.21
16.6
41
1 yr, 10 mos
11
0.45
25.5
54
12
2 yrs, 10 mos
0.49
27.5
52
12
1B
0.2
3 wks
5
7.6
19
6 mos
0.32
15
27.5
53
6
1 day
0.22
15
15
36
0.31
7 mos
17
41.3
98
11
0.28
6 mos
7
20.7
44
4 yrs, 8 mos
0.61
17
38.5
74
1 day
0.19
15
8
21.7
33
22
36.8
7 yrs
0.83
63
14
1 day
0.17
7.1
9
26
4 yrs, 3 mos
0.61
17
23.7
47
iC
1 day
0.22
16
23.3
10
49
0.31
1 yr, 1 mo
20
63.8
142
1 wk
0.22
11
17
23.2
57
6 mos
20
0.31
85.1
192
1 day
12
0.21
16
16.2
40
111
3 mos
0.23
19
43.4
0.18
7
2B
13
1 day
8.9
17
22
0.23
8
7 mos
9.5
14
1 mO
0.23
10
46
17.3
4 yrs, 3 mos
14
0.64
14.8
27
12
21
15
4 days
0.22
8.7
23.6
3 yrs, 5 mos
0.58
15
45
1 day
8
53
16
20.5
0.17
12
46
2 yrs
0.41
20.7
41
1 day
8
0.21
17
15.4
42
4 yrs, 3 mos
11
23.8
0.72
12
38
23
9 yrs
0.89
18
51
1 day
0.21
20.5
2C
15
18
138
0.36
50.5
9 wks
Abbreviations: SA = surface area; TVA = tricuspid valve annulus; RV = right ventricular; ED = enddiastole; RVED = right ventricUlar end-diastoliC.
CIRCULATION
432
VOL 61, No 2, FEBRUARY 1980
TABLE 3. Summary of Right Ventricular End-systolic and End-diastolic Volume Measutrements and Right
Ventricular Ejection Fraction Based on Right Ventricular-to-Pulmonary Artery Patency (Group 1) and Right
Ventricular-to-Pulmonary Artery Nonpatency (Group 2) at the Second AStudy
Preoperative values
Postoperative valuies
RVE1V
RVEDV
EF
RVEDV
RVEI)V
EF
Group
(mi/M2) (, normal)
(ml/m2) (C normal)
(')
(C/)
IA
n - 4
13.8-17.8
33-455
0.32-0.44
14-27.5
31--54
0.45-0.58
Mean
(15.6)
(38)
(0.39)
(19.7)
(38)
(0.51)
lB
n = 5
7.1-21.7
19-44
0.16-0.34
23.7-41.3
47-98
0.49-0.82
(14.4)
(31.6)
(0.28)
(33.5)
(67)
(0.66)
iC
n
3
16.2-23.3
40-57
0.44-0.61
43.4-85.1
111-192
0.64-0.85
=
2A
n
5
(20.9)
(48.6)
8.7-20.5
(14.2)
20.6
17-53
(35.6)
51
(0.53)
0.25-0.53
(0.39)
0.48
(64.1)
(148)
9.5-23.6
(18.3)
50..5
22-46
(35.6)
138
(0.73)
0.25-0.53
(0.39)
2C
n - 1
0.5
Subgroups A, B and C are based on the trictuspid valve measurements and the degree of tricuspid valve
incompetence as indicated.
Abbreviations: RVEDV right ventrictular end-diastolic volume; EF = ejection fraction.
=
=
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z
a
Valvotomy (patent) with Severe TI
C Valvotomy (patent) with Mild TI, Small TVA
".g Valvotomy (patent) with mod. TI, Normal TVA
E
v
0
f40
z
mu
0.4
0.6
SURFACE AREA (in2)
A
A-
0.8
,. RV/PA Discontinuity, Normal or Small TVA, Mild TI
. TVA, Severe TI
-----o RV/PA Discontinuity,.Large
E
,- /, 7'
LU
2
:I-
0
40
0
a
z
BU
B
0.2
0.4
0.6
SURFACE AREA (m2)
0.8
1
FiGURE 2. A) Right ventricular (RV) enddiastolic volumes in 12 patients with
pulmonary valve patency at postoperative
catheter study (group 1) vs surface area.
Open squares represent group ]A, closed
squares group lB, and open triangles group
IC. TVA = measured tricuspid valve annulus; TI = tricuspid regurgitation; R V/PA
right ventricula
ventricular-pulmonary
artery. B)
~~~~~~~=
ulmonary
RRi
V end-diastolic
volumes
in sixpartier.
patients
without pulmonary valve patency at
postoperative catheter study (group 2).
Closed triangles represent group 2A, and the
open circles group 2C.
RV VOLUME DETERMINATIONS/Patel et al.
assessed as mild (implying a very obstructive tricuspid
valve). This dimension was consistently 9-11 mm initially, and 1 1-12 mm at the subsequent study (table 2).
Figures 2, 3 and 5 show that among patients with mild
tricuspid regurgitation and a small tricuspid annulus,
right ventricular growth was not achieved. In contrast,
the largest tricuspid annulus size, ranging from 16-18
mm at the initial study and 19-20 mm at the
postoperative study, were observed in the three group
1 patients with qualitatively severe tricuspid regurgitation. These three patients did achieve a normal right
ventricular dimension (figs. 2, 3 and 6). Among five
other group 1 patients, the tricuspid valve annulus size
ranged from 11-15 mm at the initial study and 15-22
mm at the postoperative study. Among five of six
group 2 patients in whom the initial degree of tricuspid
regurgitation was assessed as mild, the tricuspid annulus size was initially 7-12 mm and 8-15 mm subsequently. In the only group 2 patient in whom the initial degree of tricuspid regurgitation was assessed as
severe (patient 18, tables 1 and 2), the tricuspid annulus initially measured 15 mm and postoperatively
was 18.0 mm.
The tricuspid valve orifice was measured at postmortem examination in six patients (table 2). While
the actual dimensions obtained by the three methods
(angiography, echocardiography and postmortem) are
not the same, a distinction can be made between those
with a small tricuspid valve annulus and those with a
large tricuspid valve annulus. Angiocardiography
measures the annulus size; at postmortem, the tricuspid orifice is measured. The echocardiogram only
reflects the amplitude of the tricuspid valve motion,
and we believe that the amplitude is reduced in
patients with tricuspid stenosis. The amplitude is
probably nearer normal in those with severe tricuspid
incompetence. A comparison of tricuspid valve an-
postoperative RVEDVs in each subgroup is shown in
figure 3. The ejection fraction showed little change in
group 2A.
The Tricuspid Valve: Correlation between Angiographically
and Echocardiographically Derived Dimensions
and Correlation with Right Ventricular
Dimensions and Growth
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The tricuspid annulus was measured in each patient
in an anteroposterior right ventricular cineangiocardiogram corrected for magnification, and these values
were compared with the echo measurement of the
tricuspid valve amplitude (fig. 4). Good correlation (r
= 0.79) was achieved. Although the number of
patients in each subgroup was small, the patient
assessed qualitatively as having only mild tricuspid
regurgitation had the smallest tricuspid valve dimension as measured from magnification corrected right
ventriculogram (i.e., groups lA and 2A; tables 2 and
3). Slight overlap in angiocardiographically derived
tricuspid valve dimension was found between groups
IA and lB. Similarly, in the patients with
qualitatively the most severe degrees of tricuspid
regurgitation, the angiocardiographically derived
tricuspid valve measurement had the largest dimension. Thus, group IA had the smallest tricuspid valve
measurements; group lB had moderate tricuspid valve
dimensions, and Group IC, the largest tricuspid valve
dimensions. Therefore, these groups also represent
relative tricuspid valve dimension and can be
evaluated in terms of change in right ventricular
dimension (tables 2 and 3; figs. 2A and B, 3 and 4).
When the anteroposterior measurement of the
tricuspid valve ring was less than 12 mm, the right
ventricular volume was the smallest (group lA, table
2), and the degree of tricuspid regurgitation was
140-
In..-n
u-
0
E
0
120-
c
-A
..C!
-o 100-
433
Large TVA, Severe TR, RV/PA Potency
'I
Normal TVA, Mod TR,
It
Small TVA, Mild TR,
Small TVA, Mild TR, RV/PA Discontinuity
11
Large TVA, Severe TR,
1n=31
(n=5)
(n=4)
(n=5)
(n=l)
-0
0
CL) 80-
0:/
mu
60>
0
40C
z
£u 20U
a
I
PREOPERATIVE
I
POSTOPERATIVE
FIGURE 3. Mean right ventricular (R V) end-diastolic volumes expressed as percentage of expected
volumes in the various groups. Open squares represent group ]A; closed squares, group ]B; open trianglefs,
group 1 C; closed triangles represent group 2A; and open circles, group 2C. Abbreviations: see figure 2.
CI1RC ULATION
434
Discussion
Graham and colleagues have emphasized the inability to characterize right ventricular size correctly
without volume measurements, and have called attention to errors incurred by using either routine chest
* Large TVA, Severe TR, RV/PA Patency
o Intermediate TVA, Mod. TR
11
x Small TVA, Mild TR,
11
Voi 61, No 2, FEB3RLARY 1980
* Small TVA, Mild TR, RV/PA Discontinuity
28-
-tE 24Co
0
E
z 200
I- 160
X/
12-
rO
rv
z
0)
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0
~~~~r=0.79
/
4-
z
a
O
1
1
4
8
_
12
1
1
1
1
16
20
24
28
ECHO MEASUREMENT OF TV AMPLITUDE (mm)
FiGUR[ 4. A comparison of tricuspid valve annulus
dimension measured from anteroposterior right ventriculogram vs tricuspid valve amplitude obtained from Mmode echocardiogram. X represents group 1A; open circles,
group IB; closed circles, group 1B; and closed diamonds,
group 24.
A
nulus and tricuspid valve amplitude is shown in figure
3 and table 2.
Myocardial Sinusoidal-Coronary Artery Communications
Ten of the 18 patients had extensive myocardial
sinusoidal-coronary artery communications. These
seemed to be the most extensive in those patients with
the most diminutive and hypertensive right ventricles
(groups IA and 2A). In nine patients at the initial
study. dense opacification of the left anterior descending coronary artery with retrograde opacification of
the aortic sinus of Valsalva was recorded. In only one
patient was dense retrograde visualization of the right
coronary artery achieved by right ventricular
angiocardiography. Of the 12 group 1 patients, these
sinusoids persisted in only two. Among the six group 2
patients, four had these abnormal communications
initially; at the second study, little change in the
angiocardiographic appearance had occurred (fig. 5).
In four patients, right ventricular growth had occurred, and it appeared that the area of growth incorporated the region of extensive myocardial
sinusoidal-trabecular spaces (fig. 6). At the initial
study, there was no difference in peak systolic pressure
among patients with and those without sinusoids.
rv
FiCouiRA 5. Case 17. Anteroposterior right ventriculoin a patient with pulmonary atresia, intact ventricular septum and diminutive right ventricle (R V). The tricuspid valve dimension is small (black arrows) and the initial
degree of tricuspid regurgitation was assessed as mild. Pulmonary artery patency was not achieved as determined at
the postoperative catheter study. There was minimal change
grams
in right ventricular dimension, and myocardial sinusoids
persisted. A) Neonatal study. B) Anteroposterior right ventriculogram at 9 years, with persistent severe ventricular underdevelopment.
RV VOLUME DETERMINATIONS/Patel et al.
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radiography or qualitative assessment of right ventricular angiocardiograms as indicators of right ventricular dimensions.", ` We are also aware of the real
and potential errors of applying currently used right
ventricular volume quantitative methods to the
diminutive, bizarrely shaped right ventricle, with its
"astral" projection of sinusoids, fistulous coronary
artery communications and trabecular spaces. In
Graham's study, right ventricular shape was
characterized as not grossly abnormal in five of seven
patients. This experience is somewhat unusual and
reflects the relatively small number of patients
studied.5' We agree with Graham that the Simpson's
rule method is theoretically more applicable to the abnormally shaped ventricle. Furthermore, we have included the outermost borders of the right ventricle in
our images for calculation and have attempted to
smooth out any irregularities in these borders, partially obviating objections to performing these
measurements on an irregularly shaped ventricle.
Such a method, however, probably does overestimate
right ventricular cavity size. Thus, this quantitative
approach must be interpreted within the limitations of
the method.51' 60, 67-69 Previous studies have suggested
that right ventricular dimensions tend to increase after
achievement of right ventricular-pulmonary artery
continuity.5'15 Several mechanisms may cause
enhanced right ventricular filling and, presumably,
growth, including: 1) improved right ventricular emptying; 2) ameliorating functional and organic obstruction at tricuspid valve/annulus level, which augments
right ventricular preload; 3) reducing right-to-left
shunting at the atrial level; 4) improving right ventricular compliance; and 5) augmenting right ventricular volumes by varying degrees of pulmonary and
tricuspid valve regurgitation. All of these factors (and
possibly more) are important to augmenting right ventricular filling; but if right ventricular emptying is not
significantly improved, these other factors may prove
trivial.
Right ventricular-pulmonary artery continuity was
surgically achieved in 12 patients (group 1), but in
only two were the postoperative right ventricular
pressures significantly subsystemic. Coincident with
the relief of severe right ventricular hypertension in
these two patients was an increase in right ventricular
dimensions. Our own disappointing results made it
difficult to assess the role that the establishment of
such continuity and relief of severe right ventricular
hypertension have on right ventricular growth. However, one can provide data on the morphologic basis
for failure to achieve right ventricular-pulmonary artery continuity. Among patients with pulmonary atresia
and intact ventricular septum, it is the exception
rather than the rule to have isolated pulmonary valve
fusion and atresia with a widely patent and nonobstructive pulmonary infundibulum. Both morphologic observations and the double-catheter angiocardiographic techniques advocated by Freedom et al.
have demonstrated to advantage the severe infundibular hypoplasia and underdevelopment that often ac-
435
companies pulmonary valve atresia.4' 8, 20-24, 28, 54
Among our 18 patients, severe infundibular obstruction or atresia accompanied pulmonary valve atresia
in 14 patients (78%). A transarterial pulmonary
valvotomy was performed in 12 patients, and in two,
right ventricular hypertension was significantly
relieved. The morphologic observations of Arom and
Edwards help to clarify the reasons for these surgical
results.'9 Their study of the relationship between right
ventricular muscle bundles and pulmonary valve
among 18 patients with pulmonary atresia and intact
ventricular septum suggested that there is only a small
segment of the atretic valve that is in direct contact
with the right ventricular cavity. They observed that
the least prominent subpulmonary muscle bundle is
related to the anterior pulmonary cusp and sinus, and
that a valvotomy through the region of the anterior
cusp and sinus is more apt to provide direct continuity
with the right ventricle. If the double-catheter technique is performed at the initial catheter study,23 this
should demonstrate the morphologic bases for the
pulmonary atresia. Certainly, the longer the segment
of infundibular atresia, the less likely that satisfactory
right ventricular-pulmonary artery continuity will be
established by valvotomy.
Although we can hope to achieve right ventricular
pulmonary continuity with decompression of the
hypertensive right ventricle with surgical intervention,
only in the past few years have we begun to appreciate
fully the role of the tricuspid valve in this disorder.
The tricuspid valve among patients with pulmonary
atresia and intact ventricular septum can have a wide
range of morphologic features.2' , 79, 2024, 28, 39 The
valve can be grossly insufficient, with features of
Ebstein's anomaly or profound dysplasia.20 21 In some
patients, despite pulmonary atresia, right ventricular
pressures will be subsystemic or even "low" in a
neonate, with a normal or enlarged right ventricular
cavity and extreme tricuspid valve regurgitation.9 70
In these patients, the right ventricular myocardium
may be extremely thinned or deficient, as in Uhl's
anomaly.9' 20, 707 The therapeutic problems posed by
these patients are considerably different from those in
the patient with a grossly small and hypertensive right
ventricle, and will not be considered further.
However, among patients with pulmonary atresia,
intact ventricular septum and small or diminutive
right ventricle, the tricuspid valve usually assumes a
dominant obstructive role. Although some observers
have suggested that the tricuspid valve in this situation
is- hypoplastic, though normally formed,5' our experience and an extensive literature strongly dispute
this. The tricuspid valve ring is usually grossly underdeveloped, assuming a circumference proportionate to the right ventricular cavity size. The free
valve margin is often thickened; the chordal attachments are poorly excavated, and the papillary
muscles are often hypoplastic. Our observations,
based on a necropsy study of 60 patients, suggest that
inflow obstruction at the tricuspid annulus and valve
level pose a serious, if not irreparable disturbance, es-
436
CIRCU LATION
VOL 61, No 2, FEBRUARY 1980
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FIGURE 6. Case 8. Pre- and postoperative anteroposterior
and lateral right ventricular cineangiocardiograms showing
that excellent right ventricular growth was achieved after
neonatal pulmonary valvotomy and Pott's shunt. A and B)
Anteroposterior and lateral right ventricular (R V)
cineangiogram in a neonate. In B, the tricuspid valve is well
seen (black arrows) and the distal infundibulum is considerably narrowed. C and D) Anteroposterior and lateral
angiograms at 7 months. Excellent growth was achieved,
and the pulmonary artery (PA) opacifies from the right ventriculogram.
pecially in the patient with a diminutive right ventricular cavity.21 Even with relief of severe right ventricular hypertension in some of these patients,
profound obstruction at right ventricular inflow will
preclude restoration of a hemodynamically normal
circulation. Although our data are still preliminary, it
is important to try to quantitate tricuspid annulus
(and valve) size, as it may have relevance to predicting
right ventricular growth.
Inadequate right ventricular distensibility or compliance may also prevent right ventricular filling even
when the right ventricle is satisfactorily decompressed."i 74 76 This lack of compliance may result
from a combination of severe myocardial hypertrophy, fibrosis, and endocardial fibroelastosis and
sclerosis. Although inadequate right ventricular compliance may be reversible, long-standing right ven-
tricular hypertension and myocardial free-wall and
septal hypertrophy may certainly alter right (and left)
ventricular myocardial oxygen supply-demand ratio,
culminating in ischemia, cell death and fibrosis. Such
changes are probably not fully reversible, and underscore the need for early right ventricular decompression.
Anomalous myocardial sinusoidal-coronary artery
fistulous communications complicate pulmonary
atresia, intact ventricular septum and hypoplastic,
hypertensive right ventricle in 8-30% of patients." 47, 11, 13, 14, 16, 22, 23, 28a 29, 39, 48 Initially regarded
as a pathologic curiosity, they may have a more
ominous role. We suggest that extensive fistulous
communications with the coronary arteries, par-
ticularly the left, may predispose the left ventricular
subendocardial myocardium to ischemia and
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fibrosis.22 Such fistulous communications do serve as
one site for egress of right ventricular blood, and
either the left, or less often the right, and infrequently
both coronary arteries in a patient may participate in
these primitive communications (although some
sinusoids appear to terminate blindly into the myocardium). Rarely, as in the case reported by Lenox and
Briner, the proximal connections between aorta and
coronary arteries will be absent, with right ventricular
sinusoidal communications providing continuity with
the coronary arteries.77 In some patients with these
fistulous communications, the involved coronary
arteries may have a variety of histopathologic
alterations, including nodularity, beading, and
obliterative endarteritis, all of which serve
mechanically to compromise oxygen transport.39 78"81
With pulmonary atresia, a nearly competent,
obstructive tricuspid valve, and a grossly underdeveloped right ventricle, right ventricular systolic
ejection time is markedly prolonged, and retrograde
filling of the coronary arteries, and their aortic origin
and sinus of Valsalva can be demonstrated by selective right ventricular angiocardiograms. This welldocumented phenomenon can and does occur during
aortic diastole and thus during maximal coronary
artery perfusion. 11 22, 23, 33, 47' 48 One would anticipate
that oxygen transport to that portion of the left ventricle supplied by the involved segment of the left
anterior coronary artery would be either marginal or
inadequate, resulting in a discrepancy between
myocardial oxygen demand and delivery.82 The
histopathologic correlates to this imbalance, i.e., left
ventricular myocardial ischemia, necrosis and fibrosis,
437
have been amply documented in this disorder.4' 11, 22, 39, 83
The morphogenesis of these communications rests
with the abnormal persistence of the embryonic
pattern of myoarchitecture with its lacunary blood
supply.8' Such postnatal persistence of spongy
myocardium with its embryonic blood supply is most
frequently recorded in patients with pulmonary
atresia, intact ventricular septum and small and
hypertensive right ventricle; but these findings have
also been observed in patients with severe aortic
stenosis, endocardial fibroelastosis, anomalous left
coronary artery from the pulmonary artery,
obliterative fibroelastosis of the coronary arteries,
complex congenital heart disease associated with dextrocardia and situs inversus, and in three patients with
an imperforate tricuspid valve, right ventricular tensor
apparatus, and congenital absence of the pulmonary
valve.8'-87 One assumes that with effective right ventricular decompression, obliteration of the fistulous
communication will result, and that right ventricular
growth is accompanied by incorporation of the embryonal pattern of myoarchitecture into a more normal right ventricle. Our unpublished observations, and
those of others, suggest that the spongy myocardial
and sinusoidal-coronary artery communications will
be absorbed into the ventricular mass, with relief of
right ventricular hypertension. The natural history of
this primitive myocardial architecture thus needs
further definition.
In conclusion, it is probable that long-term functional and hemodynamic improvement in these
patients is predicated on 1) establishing right ven-
438
CIRCULATION
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tricular-pulmonary artery continuity, with significant
reduction in right ventricular hypertension; the direct
corollary to this is promoting both right ventricular
emptying and filling and, therefore, growth; 2)
ameliorating functional and organic disturbances of
right ventricular inflow; 3) preventing right ventricular
endomyocardial sclerosis and fibrosis and thus
minimizing alterations in right ventricular compliance; and 4) protecting the integrity of left ventricular subendocardial myocardium, potentially
jeopardized by the cumulative effects of chronic
hypoxemia, volume overload and by alterations of
myocardial perfusion resulting from the presence of
right ventricular myocardial sinusoidal-coronary
artery communications, and coronary arterial distortions intrinsic to and secondary to these anomalous
communications. Obviously, adequate "early"
decompression of the diminutive or small and
hypertensive right ventricle is mandatory if most of
the above objectives are to be achieved, but the appropriate timing for this is uncertain. Our recent experience has suggested that the fragile neonate does
not tolerate pericardial right ventricular outflow tract
reconstruction. Thus, our medical and surgical approach to these infants includes: 1) balloon septostomy at the initial diagnostic cardiac catheterization; 2) prostaglandin infusion before and during
cardiac surgery;57 3) transpulmonary valvotomy and
construction of a systemic-pulmonary artery anastomosis.27 Then, at or before 6 months of age, we
advocate a second complete hemodynamic and
angiocardiographic investigation. If right ventricular-pulmonary artery continuity has not been
achieved, or if severe right ventricular hypertension
persists (right ventricular pressure greater than 70%
systemic), right ventricular outflow tract reconstruction with autologous pericardium is performed with
resection of infundibular muscle. During the same
procedures, the tricuspid valve ring is measured, and if
the tricuspid valve approximates 60% or more of the
normal, the patent foramen ovale is carefully
narrowed, but not closed. The latter maneuver is obviously designed to augment right ventricular filling,
although in some patients with a grossly obstructive
tricuspid valve ring and apparatus, narrowing of the
foramen ovale is not indicated. Finally, we would
suggest a third full cardiac catheter assessment 1 year
to 18 months after right ventricular outflow tract
reconstruction. We recognize that significant obstruction at the tricuspid annulus or valve level may
preclude restoration of a hemodynamically normal
circulation despite normal right ventricular pressures.
The tricuspid valve could be oversewn, with continuity
between right atrium and either right ventricle or
pulmonary artery provided by a Fontan or Kreutzer
type operation.88' 89
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Circulation. 1980;61:428-440
doi: 10.1161/01.CIR.61.2.428
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