A Radiographic
Study
of Congenital
Pulmonary
Septal
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BENIGNO
SOTO,1
ALBERT
D. PACIFICO,2
RODRIGO
F. LUNA,1
congenital
pulmonary
septal
defect
which tomic
are characterized continuity of the
(2) varying
describes
degrees
distal
pulmonary monary blood analysis ing two into
two
a group
of atresia
of 66 patients atria, and two
with
of cardiac
by (1) pulmonary
arteries, flow [1-6].
respective
atresia
or agenesis
and (3) varying We present
of direct with the of the
similar
well
developed
and
situs,
(2) atrioventricular
relation,
of reestablishing
flow,
the
ventricle,
location
and
of the atretic we
the
the
(1)
bilateral
Pa-
atria,
aorta, defect.
(3) origin
defect.
presence
December
1977
aorta
with
septal
artery
arteries;
(truncus definition
respect
defect.
bears
little
(2)
position
This
1027
these
The
clinical
three
pulmonary bilateral
classification
atresia
major
with
divisions
arteries,
are
further
de-
of left
and
of
pulmonary
absence
right
type IV); and (3) mixed types. of the situs of the viscerae and
relation,
and
malformations
pulmonary
or nonconfluence
arteriosus is made
atnioventricular
and
classify
The
of
as confluence
pulmonary
arteries Further
to
position
size
and
of the
is similar
origin
of the
ventricular to
that
of
septal Edwards
and McGoon [1]. When the pulmonary arteries are present and confluent, the entity has been called pseudotruncus arteniosus. The majority of these cases have a biventricular origin
of
the
aorta,
and
Received February 3, 1977; accepted after revision August 23, 1977. Department of Diagnostic Radiology, University of Alabama School of Medicine, 619 South 19th Street. reprint requests to B. Soto. Department of Surgery, University of Alabama School of Medicine, Birmingham, Alabama 35294. Department of Pediatrics. University of Alabama School of Medicine. Birmingham, Alabama 35294. 129:1027-1037,
of the
of congenital
septal
lineated
of aorta, (4) presence and distribution of the pulmonary arteries, and (5) sources of pulmonary blood flow. A correlative analysis of features identified on the plain chest films is presented.
Are J Roentgenol
ventricubopulmon-
ventricular
pulmonary
prefer
heading
ventricular
and
tients with truncus arteriosus types I-Ill were excluded [7]. This report describes the angiographically determined frequency of the following anatomic variations: (1) visceroatrial
consists
of pulmonary
under
each hayemptying
ventricles.
of Terms
ary artery continuity with a valved external conduit. This usually involves closing the source of pulmonary blood flow and closing the ventricular septal defect so that the aorta arises from the systemic ventricle. When there is valvular pulmonary atresia, a main pulmonary artery segment, and normally related great arteries, an outflow tract patch may be used in place of a valved external conduit. The cardiac radiologist must define the presence and distribution of pulmonary arteries, the source
Therefore,
anaheart,
sources of pula radiographic
with this malformation atrioventnicular valves
and
main
JR.3
significance.
malformations
absence arteries
L. M. BARGERON,
Definition
origin
ventricular
AND
Patients with these congenital cardiac defects may be classified, using embryologic and pathologic considerations, into a variety of categories: tetrabogy of Falbot with congenital pulmonary atresia, end-stage tetrabogy of Fallot, transposition of the great arteries, double outlet right or left ventricle, and corrected transpositon-each with pulmonary atresia. However, the separation into these subsets often cannot be made by usual angiographiti and surgical methods, because it is not always possible to determine the ventricle from which the main pulmonary artery would have arisen were it not atretic. In addition, it is usually of no clinical importance to make this differentiation. The surgical problem in each of these malformations is
to each phrase
with Ventricular
Defect
A radiographic analysis of 66 patients with congenital pulmonary atresia and ventricular septal defect was made to determine the frequency and variability of the (1) atrioventricular and ventriculoarterial relation, (2) source of pulmonary blood flow, and (3) pulmonary arterial anatomy. Of the 66 patients, 63 had situs solitus and 62 had atrioventricular concordance; the aorta arose from the right ventricle In six, left ventricle in six, and biventricularly in 54. Pulmonary arteries were present and confluent in 41 patIents, nonconfluent in five, absent in 15, and In five patients a mixed distribution to selected areas of each lung existed. In almost half of those in whom pulmonary arteries were present, the source of pulmonary blood flow was from systemic arteries (bronchials) originating from the upper descending thoracic aorta. In some, these collaterals obscured detection of pulmonary arteries when conventional anteroposterior views of the aortogram were made. The use of semiaxial craniocaudal projections and/or selective angiography were required for proper definition. Chest radlographs were correlated with angiographic details and did not permit differentiation of the various subsets of congenital pulmonary atresia from each other nor from classical tetralogy of Fallot. Abnormal central vessels were identifled in 31 patients suggesting this diagnosis. The right or left pulmonary artery identified by angiography was not detected on the plain chest film in about half of the group. Angiograms of the intra- and extracardiac details of this group of malformations are presented, correlated with plain chest films, and their surgical implications discussed. The
Atresia
the
morphology
Birmingham.
Alabama
of
the
35294.
right
Address
1028
SOTO
ventricle This
is similar
has
been
pulomonary
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When
to that
atresia
the
found
described
aorta
as
in the tetralogy tetralogy
is further
of Fallot.
with
or as end-stage
have
artery
arisen
from
the
been
from
right
the
connected
Angiographic
originating
left
ventricle,
with
ventricle.
in which
the
heart
It may case
the
en-
it would
have
entity
arisen
might
indicated
Patients tricular
above
with septal
are
congenital defect
preferred have
atresia
positional
with
yen-
anomalies
of
the heart, abnormal visceroatrial situs, and abnormal atrioventricular connections. When atrioventricular discordance is present, the phrase corrected transposition with pulmonary atresia is sometimes preferred, since corrected transposition is a malformation characterized by atrioventricular discordance and a discordant yentriculoarterial relation. We prefer to describe this malformation as congenital pulmonary atresia with ventricular septal defect, and atrioventricular discordance as origin of the aorta from the right ventricle. The pulmonary arterial system is the usual anatomic arterial tree which supplies blood to the alveolar capillares within the lungs. It is formed by main pulmonary artery, right and left pulmonary arteries, and their lobar, segmental, and subsegmental branches. In some patients part of this usual anatomic sequence may be absent. Right and left pulmonary arteries are seen angiographically as gently curved structures, concave toward the
midline,
extending
downward
and
outward
from
each hilum. The descriptive terms “palm leaf” “comma” have been used for these configurations
or [10,
11].
join
The
together confluence.
left
and
right
pulmonary
(nonconfluent) This
arteries
or they
confluence
may
may
or
may
be connected may
not
join
not
by a
artery,
a surgically
created
systemic
nary arterial shunt, or large bronchial some patients well developed bronchial ies join the pulmonary arteries in the others these extend within each lung to and separate well developed intrapulmonary These vessels, which originate from the ing thoracic aorta, usually have a spiral and may have areas of stenosis along
39 (95.1)
Inversus
0
5
4
0
1
con-
Concordant Discordant Origin
Mixed
fluent Pulmonany Arteries
15 (100.0)
2 (4.9)
Atnioventnicular nections:
Noncon’
Absent Pulmonary Antenies
40 (97.0) 1 (2.4)
14 (93.3) 1 (6.7)
4 1
4 1
31 (75.6) 4 (9.8) 6 (14.6)
13 (87.0) 2 (13.0) 0
4 1 0
3 0 0
1
2
of the aorta:
Biventnicular Right ventricle Left ventricle Systemic-pulmonary connections: Patent ductus
arter13(32.0)
iosus
Large systemic antenies Small systemic antenies Surgically created shunts Note-Numbers in parentheses - Tnuncus arteniosus type IV.
0
19 (46.0)
14 (93.0)
3
1
1 (2.0)
1 (6.7)
0
0
8 (20.0)
0
1
2
are percentages.
TABLE Radiographic
.
2 Findings Confluent Pulmonary Ar tenies
.
Finding
Heart size: Normal Enlarged Aortic arch:
Absent Pulmonary Antenies
Nonconfluent Puimonary Artenies
.
Mixed
5 0
35 6
10 5
5 0
Right
13
9
3
1
Left Pulmonary
28
6
2
4
20
2
3
1
15
2
1
0
artery:
Right
Left Lobar and segmental branches Collateral circulation Abnormal central vessels:
.
Right Left .
Truncus
anteniosus
type
.
17
2
1
1
24
10
4
0
15 11
7 12
3 2
0 1
IV.
a par-
tially developed main pulmonary trunk. The source of pulmonary blood flow may be a ductus arteriosus, well developed bronchial collateral arteries, a coronary-pulmonary fistula, a collateral artery from the innominate
Findings
Confluent Pulmonany Arteries
Situs: Solitus
by others.
pulmonary
may
Finding
be
called double-outlet right ventricle with pulmonary atresia [9]. Similarly, if the aorta arises entirely from the left ventricle, the entity may be described as double-outlet left ventricle with pulmonary atresia or simply as ventnicular septal defect with pulmonary atresia. These considerations and the surgical limitations make us believe it reasonable to group these cardiac malformations under the heading of congenital pulmonary atresia with ventnicular septal defect, although we recognize the variety of terms
1
of Fallot.
tirely from the right ventricle, the entity has been called transposition of the great arteries with congenital pulmonary atresia [8]. However, the angiographer, and sometimes the surgeon, cannot be certain that had the main pulmonary
TABLE
congenital
tetralogy
dextroposed,
ET AL.
pulmo-
collaterals. In collateral arterhilum, while in form a second arterial tree. upper descendcoiled pattern their course.
Whether ies
[12,
these
or other
arteries systemic
are truly arteries
enlarged is a matter
bronchial
arter-
of controversy
13].
When there is absence of the true anatomic pulmonary arterial tree, the arterial supply to the lung is solely from these bronchial collateral arteries and the condition is called truncus arteniosus type IV, as described by Collett and Edwards [7]. Some patients have varying combinations of pulmonary arterial pattern called the mixed type. This usually consists of a pulmonary arterial system to one or more lobes and a separate systemic arterial supply to the remaining lobes.
PULMONARY
ATRESIA
WITH
VENTRICULAR
SEPTAL
DEFECT
1029
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CONGENITAL
#{149}1 TI. Fig. 1.-Origin of aorta. A, Right ventricular angiogram, lateral view, showing septal defect (arrow). B, Right ventnicularorigin ofaorta. C, Rightventnicular origin relation. D, Left ventricular origin of aorta. Note ventricular septal defect (arrow).
aorta
originating
of aorta
in patient
equally
with
from
situs
both
inversus
ventricles.
Note
and discordant
large
ventricular
atrioventnicular
1030
SOTO
ET AL.
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Surgery was performed on 48 of the 66 patients and consisted of corrective procedures in 29, systemic-pulmonaryarteryshunts in 12, and exploratory thoracotomy alone in seven. Surgically correlatable information on intracardiac and pulmonary artery anatomy was therefore available in only 29 of the 66 patients. Confluent
Pulmonary
Arteries
Confluent pulmonary arteries was the most frequent type of congenital pulmonary atresia in this series (41 patients). There were 25 females and 16 males, ranging in age from 2 days to 34 years. The visceroatrial situs was solitus in all but two patients, and the atrioventricular relation was concordant in all but one. Angiographic studies. The origin of the aorta in the 41 patients was biventricular in 31 from the left ventricle in six, and from the right ventricle in four (fig. 1). The source of pulmonary blood flow was the bronchial collateral arteries arising from the descending thoracic aorta in 20 patients, a patent ductus arteriosus in 13, and solely a surgically created systemic-pulmonary artery shunt in eight. In some cases the presence of the pulmonary arterial system was easily demonstrated soon after injection of contrast material into the aorta (fig. 2). However, when the source of pulmonary blood flow is bronchial collateral arteries arising from the descending thoracic aorta, demonstration of the pulmonary arterial system may be difficult and may require selective catheterization of each ofthese vessels [14, 15] (fig. 3). Simple aortography in this patient (fig. 3) did not demonstrate a pulmonary arterial system; without the use of selective arteriography of each collateral vessel, a diagnosis of truncus arteriosus type IV would have been incorrectly made. ,
Fig.
2.-Aortogram
showing
pulmonary
arterial
system
in patient
with
confluent pulmonary catheter is in aortic arteries are opacified
arteries and right Blalock-Taussig shunt. Tip of arch (AO). Right (RPA) and left (LPA) pulmonary through Blalock anastomosis (B). Pulmonary arter-
ies
leaf”
have
usual
“palm
configuration.
Subjects
and
Methods
A total of 66 patients with congenital pulmonary atresia and ventricular septal defect who had complete radiographic studies made at the University of Alabama in Birmingham were selected for this study. Patients with truncus arteniosus types I-Ill were arbitrarily excluded. Patients ranged in age from 2 days to 47 years; there were 34 males and 32 females. Of the 66, 41 had confluent pulmonary arteries, 15 truncus arteniosus type IV, five had nonconfluent pulmonary arteries, and five had the mixed type. The angiognaphic study consisted of a right ventricular angiogram, aortogram after injection of contrast media into the upper descending thonacic aorta, and, in some cases, films made from selective injection of vessels originating from the upper descending thoracic aorta. More recently, the craniocaudal semiaxial
projection
has been
pulmonary
arteries.
film
(roll
series
cine. The segmental disease
was
These
film,
used
to improve
angiognams
identification
of the
were obtained
on large
Elema-Schonanden
changer)
and
35 mm
approach
followed
bronchial
recently
we
caudal the
projections
intra-
time
and
films.
in 35 patients ment
was
[4].
for the diagnosis Definition
of the
of congenital (1) situs
heart
of the vis-
arteries,
but was not evaluated
in this study.
Results
The angiographic details are listed in findings recognized from analysis of the graphs are described in table 2. Heart size ered enlarged ifthe cardiothoracic ratio was 0.56.
table 1. The chest radiowas considgreater than
of
the
of
36
2).
no
patients
The
evidence
(83%)
pulmonary film, but
(fig.
radiographic the syscranio-
angiographic were
details verified
in 31
(75%).
identified peripheral
caliber of
increased
5). The
right
and
half
were
vascularity
of
de-
proportion
the shadow visualized
majority not
films seg-
in about
vessels
in the same
lateral views, arteries was
arteries were were identified
The
only
of
at the
in 29 of these 41 patients. size was normal on chest the main pulmonary artery
were
of normal
but
The
or absent
(table
improved
confluence from the use of semiaxial anatomy
arteries
in this series. On and left pulmonary
whom chest
4.).
surgery The heart (85%) and
and/or
cases,
found right
(fig.
concave
cases
creased
found
extracardiac
of corrective
Chest
have
the pulmonary arteries with
left pulmonary
cerae and atnia, (2) atnioventnicular connection, and (3) ventniculan-great artery relation were made for each patient based upon angiography. Chest films were examined with particular attention to heart size, position of the aortic arch, and identification of the night and left pulmonary arteries and their branches. Abnormal blood vessels in the hilum of the lungs were identified and later correlated with angiographic studies. Indentation in the opacified esophagus has been found useful as an indicator of large
More
separation of temic collateral
was of the in 30 of
patients
identified on the angiographically,
in plain were
infants. Peripheral
collateral
circulation
commonly
observed
in
cyanotic patients with reduced pulmonary blood flow was identified in 24 patients of this group. This term was used by Campbell and Gardner [11] to describe vascular markings which do not appear related to the comma shape of the normal pulmonary artery. These markings are dense vascular shadows high in the mediastinum and nodular hilar structures with abnormal branching in the
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CONGENITAL
PULMONARY
ATRESIA
lung field, and are radiographic evidence of collateral circulation most probably bronchial arteries. The aortic arch was on the right side in 13 patients (32%), a frequency similar to that previously reported for ,
patients
with
this
malformation
[6].
Unusual
vascular
markings were identified in the hilum in 15 of the 41 patients in this group. The angiograms indicated that these vessels were abnormal systemic connections arising
from
the
upper
descending
thoracic
aorta
(fig.
6).
They were identified more frequently in the right (37%) than left hilum (27%), which differs from the results of Jefferson et al. [13], who reported an equal distribution. Our angiographic studies identified these abnormal hilar vessels
in four
suspected from tion of normal
additional
patients,
although
the plain chest radiograph. heart size, right aortic
this
was
not
The associaarch, concave
VENTRICULAR
WITH
SEPTAL
DEFECT
pulmonary segment, and decreased ity are the most important chest patients
tetralogy Absence
and
are
not
significantly
of Pulmonary
Arteries
1031
pulmonary film findings different
vascularin these
from
classical
of Fallot. (Truncus
Arteriosus
Type
IV) The
absence
of
pulmonary
arteries
was
the
second
most common subset in this series, present in 15 of the 66 patients (22%). There were nine males and six females, ranging in age from 2 to 26 years. Angiographic studies. Situs solitus with a concordant atrioventricular relation was found in 14 patients; one patient had a discordant relation. The origin of the aorta was
biventricular
in 13 patients
cle in two (fig. 7).
and
from
the
right
ventri-
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1032
SOTO
ET AL.
Fig. 4.-Aortogram
in patient with conA, Anteropostenor view showing systemic arteries (SA) originating from upper descending aorta (Ao) and supplying both lungs. Pulmonary arterial system not evident because of superimposition of systemic branches. B, Semiaxial craniocaudal view of same patient showing separation of systemic arteries (SA) above, and right (RPA) and left (LPA) pulmonary arteries below. fluent
The diagnosis of truncus arteriosus type IV in these patients rests upon the absence of an identifiable pulmonary artery. The source of pulmonary blood flow is solely bronchial arteries originating from the descending thoracic aorta. This must be differentiated from patients who have the same source of pulmonary blood flow, but who in addition have a true and separate pulmonary arterial system, as discussed above. The importance of this differentiation is clear, since surgical correction is possible only when a true pulmonary arterial system is present. Four of these 14 patients underwent exploratory thoracotomy, but a pulmonary artery could not be found in any, verifying the angiographic diagnosis. Chest films. Ten patients had normal sized hearts and five had cardiomegaly on chest radiography (fig. 8). The main pulmonary artery segment was absent in 13 patients, reduced from normal in one, and normal in another. Enlarged systemic arteries in the latter two patients
were
shown
by
angiography
to
account
for
this
misinterpretation of the plain films. Peripheral vessels were of small caliber in each case, and peripheral collateral circulation was identified in 10 patients. The posteroanterior film chest more frequently demonstrated abnormal hilar vessels in patients with absent pulmonary arteries, but these films were not significantly different from those of patients with confluent pulmonary arteries when the whole group is considered. However, absence of pulmonary artery branches was more clearly demonstrated in the lateral projection (fig. 8), as emphasized by Vix and Klatte [16]. Nonconfluence
of Pulmonary
Arteries
There were five patients who had nonconfluence of pulmonary arteries. All were male, ranging in age from 2 months to 13 years.
pulmonary
arteries.
Angiographic studies. Situs solitus was present in all five patients; the atrioventricular relation was concordant in four patients and discordant in one. The aorta had a biventricular origin in four patients and arose from the right
ventricle
flow
was
in
9). Corrective the
one.
bronchial
The
surgery
of
arteries
performed
angiographically
pulmonary confirming artery.
source
collateral
in one
determined
artery shunts the presence
pulmonary
in each
blood
patient
patient
anatomy.
(fig.
verified Systemic-
were made in three patients, of a nonconfluent pulmonary
Chest films. The heart size was normal in all five (fig. 10). Although the main pulmonary artery was absent in each patient, large bronchial collateral arteries were responsible for the incorrect identification of a pulmonary artery segment, reported as normal in two and diminshed in three patients. The right pulmonary artery was identified in three patients and the left pulmonary artery in
one.
four
The
cases
peripheral and
vessels
normal
in one.
were
of
small
Peripheral
collateral
caliber
in
circu-
lation was uniformly present. Abnormal hilar vessels were identified on the right in three patients and on the left in two. These findings were similar to those found by analysis of the plain chest films of patients with confluent pulmonary
arteries
nary arteries; these entities. Mixed
thus
and
they
also
those
were
with
not helpful
absent
pulmo-
in differentiating
Type
Five patients had the mixed nary atresia with ventricular four males 47 years.
Angiographic patients and
and
one
female,
type of congenital pulmoseptal defect. There were
ranging
in age
from
3 days
studies. Situs solitus was present in four situs inversus totalis in one. Four patients
to
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CONGENITAL
PULMONARY
ATRESIA
WITH
VENTRICULAR
Fig.
Fig. 5.-Chest radiographs in posteroantenion (A) and lateral (B) views showing congenital pulmonary atresia, ventricular septal defect, and confluent pulmonary arteries. Normal-sized heart and diminished pulmonary vasculanity are prominent features. Note right (arrowheads) and left (arrows) pulmonary arteries. Signs of peripheral collateral circulation are seen in lower segments.
had a concordant atrioventricular relation and one, a discordant connection. The source of pulmonary blood flow was in part a patent ductus arteriosus in two patients, a surgically created systemic-pulmonary artery shunt in two, and solely large bronchial arteries in one.
6.-A,
SEPTAL
Postenoantenion
chest
1033
DEFECT
film
of patient
with
confluent
pulmo-
nary arteries showing diminished peripheral pulmonary vascularity and large abnormal vascular shadows at left hilum (arrows) and more penipherally (arrowheads). Abnormal vessel is in higher position than pulmonary artery, indicating collateral vessel. B, Angiogram of same patient showing that abnormal vessel (arrow) is systemic artery supplying left lung
and
originating
pulmonary
arteries
Each
patient
from
were
had
by a pulmonary plied
solely
Chest normal
films. in all
descending
some
and
arteries
size
cases.
on the The
aorta.
Right
and
left
frames.
of either
system
by bronchial
Heart
in later
segments
arterial
five
thoracic
demonstrated
lung
other
(fig.
chest
pulmonary
supplied
segments
sup-
11).
radiographs artery
was segment
1034
ET AL.
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SOTO
Fig. 7.- Aortogram, anteropostenior projection, showing systemic circulation of lungs in patient with absent pulmonary arteries (truncus arteniosus type lv). Tip of catheter is at upper descending aorta. Two systemic arteries supply right and left lungs, respectively. Notice tortuosity channels and areas of stenosis
lowing
Fig. 8.-Posteroantenior (A) and lateral (B) chest films of main pulmonary artery are not visualized.
of patient
with
absent
pulmonary
arteries
(truncus
arteniosus
classical
type
“spiral”
IV).
Right
of
these
(arrows) coiled course.
and
left
branches
fol-
CONGENITAL
PULMONARY
ATRESIA
WITH
VENTRICULAR
bronchial
separate bronchial
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identification
Fig. 9.-Aortogram nary arteries showing
of patient
with
nonconfluent
right
and
left pulmo-
several small bronchial arteries supplying right and left lungs. In addition, large bronchial artery supplies right upper lobe (vertical arrow). Vessels outlined by horizontal arrows have angiographic appearance of pulmonary arteries. These two vessels are not confluent and their proximal parts are within hilum of each lung.
was absent in three patients and indeterminate in two. Peripheral pulmonary vascularity was diminished in four patients and normal in one. Peripheral collateral circulation was absent in each patient, and abnormal hilar yessels were identified in only one patient. Analysis of the chest films in this group does not demonstrate any unique characteristics which permit differentiation from other subsets of these malformations. Discussion
The
studyis to provide precise knowledge of the absence or presence, size, and distribution (confluent central, nonconfluent central, on peripheral) of the left and right pulmonary arteries. This information allows the surgeon to decide whether palliative or corrective surgery is possible and/ or advisable. In those cases without pulmonary arteries, ing
major
patients
surgery
is not
challenge
with
to the
congenital
possible.
cardiac
radiologist
pulmonary
Pulmonary
atresia
arteries
were
present
and confluent in 41 of the 66 patients (62%), nonconfluent in five (8%), absent in 15 (23%); in five (8%) a mixed distribution existed. Aortography
in the
conventional
anteroposterior
view
showed systemic arteries (bronchial) originating from the upper descending thoracic aorta supplying both lungs in 24 of 51 patients (47%) in whom pulmonary arteries were identified. It is this subset of patients with congenital pulmonary atresia with ventricular septal defect who require special attention by the cardiac radiologist. The presence of pulmonary arteries may not be identified without either selective angiograms of the
arteries
SEPTAL or
a semiaxial
the overlapping collateral arteries of
pulmonary
1035
DEFECT craniocaudal
view
to
pulmonary artery from the (figs. 3 and 4). Since lack of arteries
deems
the
patient
inoperable, either of these specialized angiographic methods should be used when pulmonary arteries are not readily identified by conventional aortography. The decision for or against surgery also requires the following details which must be defined angiographically: (1) atrioventricular relations; (2) presence, position, and anatomy of both a right and left ventricle and their respective atrioventricular valves; (3) position and origin of the aorta; (4) location, size, and number of ventricular septal defects; (5) absence or presence, size, and distribution (confluent central, nonconfluent central, or peripheral) of the left and right pulmonary arteries; and (6) source of pulmonary blood flow. Situs solitus of the viscera and atria occurred in 95% of this series, and the atrioventricular relation was concordant in 94%. There was biventricular origin of the aorta in 72%; it arose completely from the right ventricle in 9% and from the left ventricle in 9%. Corrective surgery was performed in 29 of the 66 patients; in each case the angiographic definition of right ventricular and central pulmonary artery anatomy was verified. The right ventnicle
had
an underdeveloped
or absent
infundibulum,
with
either atresia of the pulmonary valve or main pulmonary artery. The ventricular septal defect was single, large, and located either in the typical position found in classic tetralogy of Fallot or slightly more anteriorly, as often found in truncus arteriosus types I and II. When the aorta originates both from the left and right ventricle so that it overrides the ventricular septal defect, the anatomy of the right ventricle is similar to that of tetralogy of Fallot. The infundibulum is absent or severely underdeveloped and the cephalad margin of the ventricular
septal
defect
is formed
by
the
aortic
valve
leaflets. The aorta is large and aorticmitral valve continuity is ususally present (fig. 1A). When the aorta originates entirely from the right ventricle, the appearance of the right ventricle is similar to normal. The infundibulum is usually well developed and there is discontinuity between the aortic valve and the atrioventricular values. The ventricular septal defect is usually located more anteriorly than in classical tetralogy of Fallot. The left ventricle is a posterior chamber with no outlet except for the ventricular septal defect (fig. 1 B). When the aorta originates from the morphologic left ventricle, the angiographic appearance of the left ventricle is similar to that of the normal heart. The ventricular septal defect is almost always located in the anterior interventricular septum in the left ventricular outflow tract (fig. 1D). When atrioventricular discordance is associated with pulmonary atresia, the intracardiac angiographic anatomy is similar to that of corrected transposition if the aorta arises from the right ventricle (fig. 1C). When the aorta arises from the left ventricle and there is atnioventricular discordance, the angiographic anatomy is similar to that in isolated ventricular inversion.
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1036
SOTO
ET AL.
Fig. 10.-Posteroanterior (A) and lateral (B) chest films of child with nonconfluent right and left pulmonary arteries showing slightly enlarged heart with upturned apex and right aortic arch. Large vascular shadow is seen in right suprahilar region, indicating presence of abnormal vessel. Right and left pulmonary arteries not identified in B.
The plain chest radiograph does not permit differentiation of the various subsets of congenital pulmonary atresia with ventricular septal defect from each other or from classical tetralogy of Fallot. These types of malformations may be suspected when a heart of normal size is associated with a concave or absent pulmonary artery segment, reduced peripheral pulmonary vascularity, and, in some cases, the presence of unilateral or bilateral abnormal hilar vessels. REFERENCES 1 . Edwards JE, McGoon DC: Absence of anatomic origin from the heart of pulmonary arterial supply. Circulation 47:393398, 1973 2. Kinklin JW, Pacifico AD: Surgical treatment of congenital heart disease, in The Heart, edited by Hurst JW, New York, McGraw-Hill, 1974, pp 758-761
3. Pacifico
AD, Kirklin
JW, Bargenon
LM Jn, Soto
B: Surgical
treatment of common arterial trunk with pseudotruncus arteniosus. Circulation 49, suppl. 2:11 20-lI 26, 1974 4. Shinebounne EA, Macartney FJ, Anderson RH: Sequential chamber localization: logical approach to diagnosis in congenital heart disease. Br Heart J 38 :327-340, 1976 S. Kinklin JW: Surgical treatment of patients with absence of direct anatomic continuity between pulmonary arterial systern and the heart and ventricular septal defects, in Heart
Disease Harris
in Infancy,
edited
EA, Baltimore,
6. Somerville
J: Management
32:641-651,
by Banratt-Boyes
Williams
& Wilkins,
of pulmonary
Fig. ing aorta
11.-Aortogram providing
pp 211-220
atnesia. Br Heart
J
1970
7. Collett RW, Edwards JE: Persistent classification according to anatomic Am 29:1245-1270, 1949
phase showing
BG, Newtze JN, 1973,
in patient
systemic
bronchial
circulation
(LPA) pulmonary arteries, upper lobe and left lower (arrows).
with
mixed
arteries
to right
and
truncus arteniosus: a types. Surg Clin North
type.
Early
(5) originating left lungs.
Right
(A) and late (B) from descend(RPA)
and left
not seen in A, are well visualized in B. Right lobe are supplied solely by bronchial arteries
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CONGENITAL
PULMONARY
ATRESIA
WITH
8. Marcelletti C, Main DD, McGoon DC, Wallace RB, Danielson GK: Complete repair of transposition of the great arteries with pulmonary atresia. J Thorac Cardiovasc Surg 72 : 215220, 1976 9. Baker WP, Kelminson LL, Turner WM Jr, Blount SG Jn: Absence of pulmonic valve associated with double-outlet right ventricle. Circulation 36:452, 1967 10. Proceedings of the annual meeting of the Netherlands’ Society of Radiology, April 1971.Radiol Clin Biol 42:169-176, 1973 11. Campbell M, Gardner F: Radiographic features of enlarged bronchial arteries. Br Heart J 12:183-200, 1950
13.
12.
16.
Klinkharner AC: Angiography in candidates for total connection of persistent truncus arteniosus on pseudotnuncus anteniosus. Radio! Clin Biol 42:169-176, 1973
14.
VENTRICULAR
SEPTAL
DEFECT
1037
Jeftenson K, Rees 5, Somerville J: Systemic arterial supply to the lungs in pulmonary atresia and its relation to pulmonary artery development. Br Heart J 34:418-427, 1972 Cheslen E, Beck W, Schnire V: Selective catheterization of pulmonary on bronchial arteries in the preoperative assess-
ment
of pseudotruncus arteniosus and truncus arteniosus, IV.Am J Cardiol 26:20-24, 1970 15. Levin DC, Baltaxe HA, Goldbert HP, Engle MA, Ebert PA, Sos TA, Levin AR: The importance of selective angiography of systemic arterial supply to the lungs in planning surgical correction of pseudotnuncus arteniosus. Am J Roentgeno! type
121 :606-613, Vix VA, Klatte of hilar and 1970
1974 EC: The lateral chest mediastinal masses.
radiograph Radiology
in diagnosis
96:307-316,
Study
of Congenital
Pulmonary
Septal
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BENIGNO
SOTO,1
ALBERT
D. PACIFICO,2
RODRIGO
F. LUNA,1
congenital
pulmonary
septal
defect
which tomic
are characterized continuity of the
(2) varying
describes
degrees
distal
pulmonary monary blood analysis ing two into
two
a group
of atresia
of 66 patients atria, and two
with
of cardiac
by (1) pulmonary
arteries, flow [1-6].
respective
atresia
or agenesis
and (3) varying We present
of direct with the of the
similar
well
developed
and
situs,
(2) atrioventricular
relation,
of reestablishing
flow,
the
ventricle,
location
and
of the atretic we
the
the
(1)
bilateral
Pa-
atria,
aorta, defect.
(3) origin
defect.
presence
December
1977
aorta
with
septal
artery
arteries;
(truncus definition
respect
defect.
bears
little
(2)
position
This
1027
these
The
clinical
three
pulmonary bilateral
classification
atresia
major
with
divisions
arteries,
are
further
de-
of left
and
of
pulmonary
absence
right
type IV); and (3) mixed types. of the situs of the viscerae and
relation,
and
malformations
pulmonary
or nonconfluence
arteriosus is made
atnioventricular
and
classify
The
of
as confluence
pulmonary
arteries Further
to
position
size
and
of the
is similar
origin
of the
ventricular to
that
of
septal Edwards
and McGoon [1]. When the pulmonary arteries are present and confluent, the entity has been called pseudotruncus arteniosus. The majority of these cases have a biventricular origin
of
the
aorta,
and
Received February 3, 1977; accepted after revision August 23, 1977. Department of Diagnostic Radiology, University of Alabama School of Medicine, 619 South 19th Street. reprint requests to B. Soto. Department of Surgery, University of Alabama School of Medicine, Birmingham, Alabama 35294. Department of Pediatrics. University of Alabama School of Medicine. Birmingham, Alabama 35294. 129:1027-1037,
of the
of congenital
septal
lineated
of aorta, (4) presence and distribution of the pulmonary arteries, and (5) sources of pulmonary blood flow. A correlative analysis of features identified on the plain chest films is presented.
Are J Roentgenol
ventricubopulmon-
ventricular
pulmonary
prefer
heading
ventricular
and
tients with truncus arteriosus types I-Ill were excluded [7]. This report describes the angiographically determined frequency of the following anatomic variations: (1) visceroatrial
consists
of pulmonary
under
each hayemptying
ventricles.
of Terms
ary artery continuity with a valved external conduit. This usually involves closing the source of pulmonary blood flow and closing the ventricular septal defect so that the aorta arises from the systemic ventricle. When there is valvular pulmonary atresia, a main pulmonary artery segment, and normally related great arteries, an outflow tract patch may be used in place of a valved external conduit. The cardiac radiologist must define the presence and distribution of pulmonary arteries, the source
Therefore,
anaheart,
sources of pula radiographic
with this malformation atrioventnicular valves
and
main
JR.3
significance.
malformations
absence arteries
L. M. BARGERON,
Definition
origin
ventricular
AND
Patients with these congenital cardiac defects may be classified, using embryologic and pathologic considerations, into a variety of categories: tetrabogy of Falbot with congenital pulmonary atresia, end-stage tetrabogy of Fallot, transposition of the great arteries, double outlet right or left ventricle, and corrected transpositon-each with pulmonary atresia. However, the separation into these subsets often cannot be made by usual angiographiti and surgical methods, because it is not always possible to determine the ventricle from which the main pulmonary artery would have arisen were it not atretic. In addition, it is usually of no clinical importance to make this differentiation. The surgical problem in each of these malformations is
to each phrase
with Ventricular
Defect
A radiographic analysis of 66 patients with congenital pulmonary atresia and ventricular septal defect was made to determine the frequency and variability of the (1) atrioventricular and ventriculoarterial relation, (2) source of pulmonary blood flow, and (3) pulmonary arterial anatomy. Of the 66 patients, 63 had situs solitus and 62 had atrioventricular concordance; the aorta arose from the right ventricle In six, left ventricle in six, and biventricularly in 54. Pulmonary arteries were present and confluent in 41 patIents, nonconfluent in five, absent in 15, and In five patients a mixed distribution to selected areas of each lung existed. In almost half of those in whom pulmonary arteries were present, the source of pulmonary blood flow was from systemic arteries (bronchials) originating from the upper descending thoracic aorta. In some, these collaterals obscured detection of pulmonary arteries when conventional anteroposterior views of the aortogram were made. The use of semiaxial craniocaudal projections and/or selective angiography were required for proper definition. Chest radlographs were correlated with angiographic details and did not permit differentiation of the various subsets of congenital pulmonary atresia from each other nor from classical tetralogy of Fallot. Abnormal central vessels were identifled in 31 patients suggesting this diagnosis. The right or left pulmonary artery identified by angiography was not detected on the plain chest film in about half of the group. Angiograms of the intra- and extracardiac details of this group of malformations are presented, correlated with plain chest films, and their surgical implications discussed. The
Atresia
the
morphology
Birmingham.
Alabama
of
the
35294.
right
Address
1028
SOTO
ventricle This
is similar
has
been
pulomonary
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When
to that
atresia
the
found
described
aorta
as
in the tetralogy tetralogy
is further
of Fallot.
with
or as end-stage
have
artery
arisen
from
the
been
from
right
the
connected
Angiographic
originating
left
ventricle,
with
ventricle.
in which
the
heart
It may case
the
en-
it would
have
entity
arisen
might
indicated
Patients tricular
above
with septal
are
congenital defect
preferred have
atresia
positional
with
yen-
anomalies
of
the heart, abnormal visceroatrial situs, and abnormal atrioventricular connections. When atrioventricular discordance is present, the phrase corrected transposition with pulmonary atresia is sometimes preferred, since corrected transposition is a malformation characterized by atrioventricular discordance and a discordant yentriculoarterial relation. We prefer to describe this malformation as congenital pulmonary atresia with ventricular septal defect, and atrioventricular discordance as origin of the aorta from the right ventricle. The pulmonary arterial system is the usual anatomic arterial tree which supplies blood to the alveolar capillares within the lungs. It is formed by main pulmonary artery, right and left pulmonary arteries, and their lobar, segmental, and subsegmental branches. In some patients part of this usual anatomic sequence may be absent. Right and left pulmonary arteries are seen angiographically as gently curved structures, concave toward the
midline,
extending
downward
and
outward
from
each hilum. The descriptive terms “palm leaf” “comma” have been used for these configurations
or [10,
11].
join
The
together confluence.
left
and
right
pulmonary
(nonconfluent) This
arteries
or they
confluence
may
may
or
may
be connected may
not
join
not
by a
artery,
a surgically
created
systemic
nary arterial shunt, or large bronchial some patients well developed bronchial ies join the pulmonary arteries in the others these extend within each lung to and separate well developed intrapulmonary These vessels, which originate from the ing thoracic aorta, usually have a spiral and may have areas of stenosis along
39 (95.1)
Inversus
0
5
4
0
1
con-
Concordant Discordant Origin
Mixed
fluent Pulmonany Arteries
15 (100.0)
2 (4.9)
Atnioventnicular nections:
Noncon’
Absent Pulmonary Antenies
40 (97.0) 1 (2.4)
14 (93.3) 1 (6.7)
4 1
4 1
31 (75.6) 4 (9.8) 6 (14.6)
13 (87.0) 2 (13.0) 0
4 1 0
3 0 0
1
2
of the aorta:
Biventnicular Right ventricle Left ventricle Systemic-pulmonary connections: Patent ductus
arter13(32.0)
iosus
Large systemic antenies Small systemic antenies Surgically created shunts Note-Numbers in parentheses - Tnuncus arteniosus type IV.
0
19 (46.0)
14 (93.0)
3
1
1 (2.0)
1 (6.7)
0
0
8 (20.0)
0
1
2
are percentages.
TABLE Radiographic
.
2 Findings Confluent Pulmonary Ar tenies
.
Finding
Heart size: Normal Enlarged Aortic arch:
Absent Pulmonary Antenies
Nonconfluent Puimonary Artenies
.
Mixed
5 0
35 6
10 5
5 0
Right
13
9
3
1
Left Pulmonary
28
6
2
4
20
2
3
1
15
2
1
0
artery:
Right
Left Lobar and segmental branches Collateral circulation Abnormal central vessels:
.
Right Left .
Truncus
anteniosus
type
.
17
2
1
1
24
10
4
0
15 11
7 12
3 2
0 1
IV.
a par-
tially developed main pulmonary trunk. The source of pulmonary blood flow may be a ductus arteriosus, well developed bronchial collateral arteries, a coronary-pulmonary fistula, a collateral artery from the innominate
Findings
Confluent Pulmonany Arteries
Situs: Solitus
by others.
pulmonary
may
Finding
be
called double-outlet right ventricle with pulmonary atresia [9]. Similarly, if the aorta arises entirely from the left ventricle, the entity may be described as double-outlet left ventricle with pulmonary atresia or simply as ventnicular septal defect with pulmonary atresia. These considerations and the surgical limitations make us believe it reasonable to group these cardiac malformations under the heading of congenital pulmonary atresia with ventnicular septal defect, although we recognize the variety of terms
1
of Fallot.
tirely from the right ventricle, the entity has been called transposition of the great arteries with congenital pulmonary atresia [8]. However, the angiographer, and sometimes the surgeon, cannot be certain that had the main pulmonary
TABLE
congenital
tetralogy
dextroposed,
ET AL.
pulmo-
collaterals. In collateral arterhilum, while in form a second arterial tree. upper descendcoiled pattern their course.
Whether ies
[12,
these
or other
arteries systemic
are truly arteries
enlarged is a matter
bronchial
arter-
of controversy
13].
When there is absence of the true anatomic pulmonary arterial tree, the arterial supply to the lung is solely from these bronchial collateral arteries and the condition is called truncus arteniosus type IV, as described by Collett and Edwards [7]. Some patients have varying combinations of pulmonary arterial pattern called the mixed type. This usually consists of a pulmonary arterial system to one or more lobes and a separate systemic arterial supply to the remaining lobes.
PULMONARY
ATRESIA
WITH
VENTRICULAR
SEPTAL
DEFECT
1029
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CONGENITAL
#{149}1 TI. Fig. 1.-Origin of aorta. A, Right ventricular angiogram, lateral view, showing septal defect (arrow). B, Right ventnicularorigin ofaorta. C, Rightventnicular origin relation. D, Left ventricular origin of aorta. Note ventricular septal defect (arrow).
aorta
originating
of aorta
in patient
equally
with
from
situs
both
inversus
ventricles.
Note
and discordant
large
ventricular
atrioventnicular
1030
SOTO
ET AL.
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Surgery was performed on 48 of the 66 patients and consisted of corrective procedures in 29, systemic-pulmonaryarteryshunts in 12, and exploratory thoracotomy alone in seven. Surgically correlatable information on intracardiac and pulmonary artery anatomy was therefore available in only 29 of the 66 patients. Confluent
Pulmonary
Arteries
Confluent pulmonary arteries was the most frequent type of congenital pulmonary atresia in this series (41 patients). There were 25 females and 16 males, ranging in age from 2 days to 34 years. The visceroatrial situs was solitus in all but two patients, and the atrioventricular relation was concordant in all but one. Angiographic studies. The origin of the aorta in the 41 patients was biventricular in 31 from the left ventricle in six, and from the right ventricle in four (fig. 1). The source of pulmonary blood flow was the bronchial collateral arteries arising from the descending thoracic aorta in 20 patients, a patent ductus arteriosus in 13, and solely a surgically created systemic-pulmonary artery shunt in eight. In some cases the presence of the pulmonary arterial system was easily demonstrated soon after injection of contrast material into the aorta (fig. 2). However, when the source of pulmonary blood flow is bronchial collateral arteries arising from the descending thoracic aorta, demonstration of the pulmonary arterial system may be difficult and may require selective catheterization of each ofthese vessels [14, 15] (fig. 3). Simple aortography in this patient (fig. 3) did not demonstrate a pulmonary arterial system; without the use of selective arteriography of each collateral vessel, a diagnosis of truncus arteriosus type IV would have been incorrectly made. ,
Fig.
2.-Aortogram
showing
pulmonary
arterial
system
in patient
with
confluent pulmonary catheter is in aortic arteries are opacified
arteries and right Blalock-Taussig shunt. Tip of arch (AO). Right (RPA) and left (LPA) pulmonary through Blalock anastomosis (B). Pulmonary arter-
ies
leaf”
have
usual
“palm
configuration.
Subjects
and
Methods
A total of 66 patients with congenital pulmonary atresia and ventricular septal defect who had complete radiographic studies made at the University of Alabama in Birmingham were selected for this study. Patients with truncus arteniosus types I-Ill were arbitrarily excluded. Patients ranged in age from 2 days to 47 years; there were 34 males and 32 females. Of the 66, 41 had confluent pulmonary arteries, 15 truncus arteniosus type IV, five had nonconfluent pulmonary arteries, and five had the mixed type. The angiognaphic study consisted of a right ventricular angiogram, aortogram after injection of contrast media into the upper descending thonacic aorta, and, in some cases, films made from selective injection of vessels originating from the upper descending thoracic aorta. More recently, the craniocaudal semiaxial
projection
has been
pulmonary
arteries.
film
(roll
series
cine. The segmental disease
was
These
film,
used
to improve
angiognams
identification
of the
were obtained
on large
Elema-Schonanden
changer)
and
35 mm
approach
followed
bronchial
recently
we
caudal the
projections
intra-
time
and
films.
in 35 patients ment
was
[4].
for the diagnosis Definition
of the
of congenital (1) situs
heart
of the vis-
arteries,
but was not evaluated
in this study.
Results
The angiographic details are listed in findings recognized from analysis of the graphs are described in table 2. Heart size ered enlarged ifthe cardiothoracic ratio was 0.56.
table 1. The chest radiowas considgreater than
of
the
of
36
2).
no
patients
The
evidence
(83%)
pulmonary film, but
(fig.
radiographic the syscranio-
angiographic were
details verified
in 31
(75%).
identified peripheral
caliber of
increased
5). The
right
and
half
were
vascularity
of
de-
proportion
the shadow visualized
majority not
films seg-
in about
vessels
in the same
lateral views, arteries was
arteries were were identified
The
only
of
at the
in 29 of these 41 patients. size was normal on chest the main pulmonary artery
were
of normal
but
The
or absent
(table
improved
confluence from the use of semiaxial anatomy
arteries
in this series. On and left pulmonary
whom chest
4.).
surgery The heart (85%) and
and/or
cases,
found right
(fig.
concave
cases
creased
found
extracardiac
of corrective
Chest
have
the pulmonary arteries with
left pulmonary
cerae and atnia, (2) atnioventnicular connection, and (3) ventniculan-great artery relation were made for each patient based upon angiography. Chest films were examined with particular attention to heart size, position of the aortic arch, and identification of the night and left pulmonary arteries and their branches. Abnormal blood vessels in the hilum of the lungs were identified and later correlated with angiographic studies. Indentation in the opacified esophagus has been found useful as an indicator of large
More
separation of temic collateral
was of the in 30 of
patients
identified on the angiographically,
in plain were
infants. Peripheral
collateral
circulation
commonly
observed
in
cyanotic patients with reduced pulmonary blood flow was identified in 24 patients of this group. This term was used by Campbell and Gardner [11] to describe vascular markings which do not appear related to the comma shape of the normal pulmonary artery. These markings are dense vascular shadows high in the mediastinum and nodular hilar structures with abnormal branching in the
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CONGENITAL
PULMONARY
ATRESIA
lung field, and are radiographic evidence of collateral circulation most probably bronchial arteries. The aortic arch was on the right side in 13 patients (32%), a frequency similar to that previously reported for ,
patients
with
this
malformation
[6].
Unusual
vascular
markings were identified in the hilum in 15 of the 41 patients in this group. The angiograms indicated that these vessels were abnormal systemic connections arising
from
the
upper
descending
thoracic
aorta
(fig.
6).
They were identified more frequently in the right (37%) than left hilum (27%), which differs from the results of Jefferson et al. [13], who reported an equal distribution. Our angiographic studies identified these abnormal hilar vessels
in four
suspected from tion of normal
additional
patients,
although
the plain chest radiograph. heart size, right aortic
this
was
not
The associaarch, concave
VENTRICULAR
WITH
SEPTAL
DEFECT
pulmonary segment, and decreased ity are the most important chest patients
tetralogy Absence
and
are
not
significantly
of Pulmonary
Arteries
1031
pulmonary film findings different
vascularin these
from
classical
of Fallot. (Truncus
Arteriosus
Type
IV) The
absence
of
pulmonary
arteries
was
the
second
most common subset in this series, present in 15 of the 66 patients (22%). There were nine males and six females, ranging in age from 2 to 26 years. Angiographic studies. Situs solitus with a concordant atrioventricular relation was found in 14 patients; one patient had a discordant relation. The origin of the aorta was
biventricular
in 13 patients
cle in two (fig. 7).
and
from
the
right
ventri-
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1032
SOTO
ET AL.
Fig. 4.-Aortogram
in patient with conA, Anteropostenor view showing systemic arteries (SA) originating from upper descending aorta (Ao) and supplying both lungs. Pulmonary arterial system not evident because of superimposition of systemic branches. B, Semiaxial craniocaudal view of same patient showing separation of systemic arteries (SA) above, and right (RPA) and left (LPA) pulmonary arteries below. fluent
The diagnosis of truncus arteriosus type IV in these patients rests upon the absence of an identifiable pulmonary artery. The source of pulmonary blood flow is solely bronchial arteries originating from the descending thoracic aorta. This must be differentiated from patients who have the same source of pulmonary blood flow, but who in addition have a true and separate pulmonary arterial system, as discussed above. The importance of this differentiation is clear, since surgical correction is possible only when a true pulmonary arterial system is present. Four of these 14 patients underwent exploratory thoracotomy, but a pulmonary artery could not be found in any, verifying the angiographic diagnosis. Chest films. Ten patients had normal sized hearts and five had cardiomegaly on chest radiography (fig. 8). The main pulmonary artery segment was absent in 13 patients, reduced from normal in one, and normal in another. Enlarged systemic arteries in the latter two patients
were
shown
by
angiography
to
account
for
this
misinterpretation of the plain films. Peripheral vessels were of small caliber in each case, and peripheral collateral circulation was identified in 10 patients. The posteroanterior film chest more frequently demonstrated abnormal hilar vessels in patients with absent pulmonary arteries, but these films were not significantly different from those of patients with confluent pulmonary arteries when the whole group is considered. However, absence of pulmonary artery branches was more clearly demonstrated in the lateral projection (fig. 8), as emphasized by Vix and Klatte [16]. Nonconfluence
of Pulmonary
Arteries
There were five patients who had nonconfluence of pulmonary arteries. All were male, ranging in age from 2 months to 13 years.
pulmonary
arteries.
Angiographic studies. Situs solitus was present in all five patients; the atrioventricular relation was concordant in four patients and discordant in one. The aorta had a biventricular origin in four patients and arose from the right
ventricle
flow
was
in
9). Corrective the
one.
bronchial
The
surgery
of
arteries
performed
angiographically
pulmonary confirming artery.
source
collateral
in one
determined
artery shunts the presence
pulmonary
in each
blood
patient
patient
anatomy.
(fig.
verified Systemic-
were made in three patients, of a nonconfluent pulmonary
Chest films. The heart size was normal in all five (fig. 10). Although the main pulmonary artery was absent in each patient, large bronchial collateral arteries were responsible for the incorrect identification of a pulmonary artery segment, reported as normal in two and diminshed in three patients. The right pulmonary artery was identified in three patients and the left pulmonary artery in
one.
four
The
cases
peripheral and
vessels
normal
in one.
were
of
small
Peripheral
collateral
caliber
in
circu-
lation was uniformly present. Abnormal hilar vessels were identified on the right in three patients and on the left in two. These findings were similar to those found by analysis of the plain chest films of patients with confluent pulmonary
arteries
nary arteries; these entities. Mixed
thus
and
they
also
those
were
with
not helpful
absent
pulmo-
in differentiating
Type
Five patients had the mixed nary atresia with ventricular four males 47 years.
Angiographic patients and
and
one
female,
type of congenital pulmoseptal defect. There were
ranging
in age
from
3 days
studies. Situs solitus was present in four situs inversus totalis in one. Four patients
to
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CONGENITAL
PULMONARY
ATRESIA
WITH
VENTRICULAR
Fig.
Fig. 5.-Chest radiographs in posteroantenion (A) and lateral (B) views showing congenital pulmonary atresia, ventricular septal defect, and confluent pulmonary arteries. Normal-sized heart and diminished pulmonary vasculanity are prominent features. Note right (arrowheads) and left (arrows) pulmonary arteries. Signs of peripheral collateral circulation are seen in lower segments.
had a concordant atrioventricular relation and one, a discordant connection. The source of pulmonary blood flow was in part a patent ductus arteriosus in two patients, a surgically created systemic-pulmonary artery shunt in two, and solely large bronchial arteries in one.
6.-A,
SEPTAL
Postenoantenion
chest
1033
DEFECT
film
of patient
with
confluent
pulmo-
nary arteries showing diminished peripheral pulmonary vascularity and large abnormal vascular shadows at left hilum (arrows) and more penipherally (arrowheads). Abnormal vessel is in higher position than pulmonary artery, indicating collateral vessel. B, Angiogram of same patient showing that abnormal vessel (arrow) is systemic artery supplying left lung
and
originating
pulmonary
arteries
Each
patient
from
were
had
by a pulmonary plied
solely
Chest normal
films. in all
descending
some
and
arteries
size
cases.
on the The
aorta.
Right
and
left
frames.
of either
system
by bronchial
Heart
in later
segments
arterial
five
thoracic
demonstrated
lung
other
(fig.
chest
pulmonary
supplied
segments
sup-
11).
radiographs artery
was segment
1034
ET AL.
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SOTO
Fig. 7.- Aortogram, anteropostenior projection, showing systemic circulation of lungs in patient with absent pulmonary arteries (truncus arteniosus type lv). Tip of catheter is at upper descending aorta. Two systemic arteries supply right and left lungs, respectively. Notice tortuosity channels and areas of stenosis
lowing
Fig. 8.-Posteroantenior (A) and lateral (B) chest films of main pulmonary artery are not visualized.
of patient
with
absent
pulmonary
arteries
(truncus
arteniosus
classical
type
“spiral”
IV).
Right
of
these
(arrows) coiled course.
and
left
branches
fol-
CONGENITAL
PULMONARY
ATRESIA
WITH
VENTRICULAR
bronchial
separate bronchial
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identification
Fig. 9.-Aortogram nary arteries showing
of patient
with
nonconfluent
right
and
left pulmo-
several small bronchial arteries supplying right and left lungs. In addition, large bronchial artery supplies right upper lobe (vertical arrow). Vessels outlined by horizontal arrows have angiographic appearance of pulmonary arteries. These two vessels are not confluent and their proximal parts are within hilum of each lung.
was absent in three patients and indeterminate in two. Peripheral pulmonary vascularity was diminished in four patients and normal in one. Peripheral collateral circulation was absent in each patient, and abnormal hilar yessels were identified in only one patient. Analysis of the chest films in this group does not demonstrate any unique characteristics which permit differentiation from other subsets of these malformations. Discussion
The
studyis to provide precise knowledge of the absence or presence, size, and distribution (confluent central, nonconfluent central, on peripheral) of the left and right pulmonary arteries. This information allows the surgeon to decide whether palliative or corrective surgery is possible and/ or advisable. In those cases without pulmonary arteries, ing
major
patients
surgery
is not
challenge
with
to the
congenital
possible.
cardiac
radiologist
pulmonary
Pulmonary
atresia
arteries
were
present
and confluent in 41 of the 66 patients (62%), nonconfluent in five (8%), absent in 15 (23%); in five (8%) a mixed distribution existed. Aortography
in the
conventional
anteroposterior
view
showed systemic arteries (bronchial) originating from the upper descending thoracic aorta supplying both lungs in 24 of 51 patients (47%) in whom pulmonary arteries were identified. It is this subset of patients with congenital pulmonary atresia with ventricular septal defect who require special attention by the cardiac radiologist. The presence of pulmonary arteries may not be identified without either selective angiograms of the
arteries
SEPTAL or
a semiaxial
the overlapping collateral arteries of
pulmonary
1035
DEFECT craniocaudal
view
to
pulmonary artery from the (figs. 3 and 4). Since lack of arteries
deems
the
patient
inoperable, either of these specialized angiographic methods should be used when pulmonary arteries are not readily identified by conventional aortography. The decision for or against surgery also requires the following details which must be defined angiographically: (1) atrioventricular relations; (2) presence, position, and anatomy of both a right and left ventricle and their respective atrioventricular valves; (3) position and origin of the aorta; (4) location, size, and number of ventricular septal defects; (5) absence or presence, size, and distribution (confluent central, nonconfluent central, or peripheral) of the left and right pulmonary arteries; and (6) source of pulmonary blood flow. Situs solitus of the viscera and atria occurred in 95% of this series, and the atrioventricular relation was concordant in 94%. There was biventricular origin of the aorta in 72%; it arose completely from the right ventricle in 9% and from the left ventricle in 9%. Corrective surgery was performed in 29 of the 66 patients; in each case the angiographic definition of right ventricular and central pulmonary artery anatomy was verified. The right ventnicle
had
an underdeveloped
or absent
infundibulum,
with
either atresia of the pulmonary valve or main pulmonary artery. The ventricular septal defect was single, large, and located either in the typical position found in classic tetralogy of Fallot or slightly more anteriorly, as often found in truncus arteriosus types I and II. When the aorta originates both from the left and right ventricle so that it overrides the ventricular septal defect, the anatomy of the right ventricle is similar to that of tetralogy of Fallot. The infundibulum is absent or severely underdeveloped and the cephalad margin of the ventricular
septal
defect
is formed
by
the
aortic
valve
leaflets. The aorta is large and aorticmitral valve continuity is ususally present (fig. 1A). When the aorta originates entirely from the right ventricle, the appearance of the right ventricle is similar to normal. The infundibulum is usually well developed and there is discontinuity between the aortic valve and the atrioventricular values. The ventricular septal defect is usually located more anteriorly than in classical tetralogy of Fallot. The left ventricle is a posterior chamber with no outlet except for the ventricular septal defect (fig. 1 B). When the aorta originates from the morphologic left ventricle, the angiographic appearance of the left ventricle is similar to that of the normal heart. The ventricular septal defect is almost always located in the anterior interventricular septum in the left ventricular outflow tract (fig. 1D). When atrioventricular discordance is associated with pulmonary atresia, the intracardiac angiographic anatomy is similar to that of corrected transposition if the aorta arises from the right ventricle (fig. 1C). When the aorta arises from the left ventricle and there is atnioventricular discordance, the angiographic anatomy is similar to that in isolated ventricular inversion.
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1036
SOTO
ET AL.
Fig. 10.-Posteroanterior (A) and lateral (B) chest films of child with nonconfluent right and left pulmonary arteries showing slightly enlarged heart with upturned apex and right aortic arch. Large vascular shadow is seen in right suprahilar region, indicating presence of abnormal vessel. Right and left pulmonary arteries not identified in B.
The plain chest radiograph does not permit differentiation of the various subsets of congenital pulmonary atresia with ventricular septal defect from each other or from classical tetralogy of Fallot. These types of malformations may be suspected when a heart of normal size is associated with a concave or absent pulmonary artery segment, reduced peripheral pulmonary vascularity, and, in some cases, the presence of unilateral or bilateral abnormal hilar vessels. REFERENCES 1 . Edwards JE, McGoon DC: Absence of anatomic origin from the heart of pulmonary arterial supply. Circulation 47:393398, 1973 2. Kinklin JW, Pacifico AD: Surgical treatment of congenital heart disease, in The Heart, edited by Hurst JW, New York, McGraw-Hill, 1974, pp 758-761
3. Pacifico
AD, Kirklin
JW, Bargenon
LM Jn, Soto
B: Surgical
treatment of common arterial trunk with pseudotruncus arteniosus. Circulation 49, suppl. 2:11 20-lI 26, 1974 4. Shinebounne EA, Macartney FJ, Anderson RH: Sequential chamber localization: logical approach to diagnosis in congenital heart disease. Br Heart J 38 :327-340, 1976 S. Kinklin JW: Surgical treatment of patients with absence of direct anatomic continuity between pulmonary arterial systern and the heart and ventricular septal defects, in Heart
Disease Harris
in Infancy,
edited
EA, Baltimore,
6. Somerville
J: Management
32:641-651,
by Banratt-Boyes
Williams
& Wilkins,
of pulmonary
Fig. ing aorta
11.-Aortogram providing
pp 211-220
atnesia. Br Heart
J
1970
7. Collett RW, Edwards JE: Persistent classification according to anatomic Am 29:1245-1270, 1949
phase showing
BG, Newtze JN, 1973,
in patient
systemic
bronchial
circulation
(LPA) pulmonary arteries, upper lobe and left lower (arrows).
with
mixed
arteries
to right
and
truncus arteniosus: a types. Surg Clin North
type.
Early
(5) originating left lungs.
Right
(A) and late (B) from descend(RPA)
and left
not seen in A, are well visualized in B. Right lobe are supplied solely by bronchial arteries
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CONGENITAL
PULMONARY
ATRESIA
WITH
8. Marcelletti C, Main DD, McGoon DC, Wallace RB, Danielson GK: Complete repair of transposition of the great arteries with pulmonary atresia. J Thorac Cardiovasc Surg 72 : 215220, 1976 9. Baker WP, Kelminson LL, Turner WM Jr, Blount SG Jn: Absence of pulmonic valve associated with double-outlet right ventricle. Circulation 36:452, 1967 10. Proceedings of the annual meeting of the Netherlands’ Society of Radiology, April 1971.Radiol Clin Biol 42:169-176, 1973 11. Campbell M, Gardner F: Radiographic features of enlarged bronchial arteries. Br Heart J 12:183-200, 1950
13.
12.
16.
Klinkharner AC: Angiography in candidates for total connection of persistent truncus arteniosus on pseudotnuncus anteniosus. Radio! Clin Biol 42:169-176, 1973
14.
VENTRICULAR
SEPTAL
DEFECT
1037
Jeftenson K, Rees 5, Somerville J: Systemic arterial supply to the lungs in pulmonary atresia and its relation to pulmonary artery development. Br Heart J 34:418-427, 1972 Cheslen E, Beck W, Schnire V: Selective catheterization of pulmonary on bronchial arteries in the preoperative assess-
ment
of pseudotruncus arteniosus and truncus arteniosus, IV.Am J Cardiol 26:20-24, 1970 15. Levin DC, Baltaxe HA, Goldbert HP, Engle MA, Ebert PA, Sos TA, Levin AR: The importance of selective angiography of systemic arterial supply to the lungs in planning surgical correction of pseudotnuncus arteniosus. Am J Roentgeno! type
121 :606-613, Vix VA, Klatte of hilar and 1970
1974 EC: The lateral chest mediastinal masses.
radiograph Radiology
in diagnosis
96:307-316,
