Download Congenital Interruption of the Inferior Vena Cava

Congenital
Interruption
.
Inferior
Vena
Cava
Ronald
L. van der Horst,
AloLs R. Hostreiter,
M.D.
M.D.,
and
form of Interruption
of the inferior
vena
the post-renal
IVC
continues
as the azygos
and
hemlazygos
vein.
We report
a patient
with complete Interruption
of the IVC in whom
no direct continulty
existed
between
the IVC and the azygos
system.
Connection
between
these
two
systems
was
via the
vertebral
plexus
and ascending
lumbar
veins. Associated
venous
malformations
included
bilateral
azygos
veins
and anomalous
connection
of pulmonary
and hepatic
veins.
In
the
of the
*
cava
W
usual
(IVC),
e describe
an
infant
interruption
The
vessel
of
terminated
vertebra
and
azygos
system.
Blood
to the
had
The
tration
vertebral
The
innominate
of
the
vein
right
right
lobe
of the
liver,
via
a long
cular
defect,
heart,
directly
to
anomalies
patent
and
(IVC).
of the
with
the
IVC
first
the
poste-
ascending
as
the
as
those
anomalously
left
atrium.
of a sinus
arteriosus,
of
sequesthe
Other
venosus
hypoplasia
lung.
from
the
to the
IVC
hepatic
vein
at-
cardiovas-
atrial
septal
of the
Ficunz
1.
(anteroposterior
lateral
lumbar
was
lobe
well
The
right
There
middle
drained
consisted
cardiac
the
atretic.
and
trunk.
ductus
from
and
site,
common
level
continuity
routed
was
this
at the
....
complete
cava
as a bilateral
azygos
system
to
venae
cavae
on either
side.
lower
from
vena
direct
plexus
veins
tached
no
was
veins,
which
continued
connect
to the superior
congenital
inferior
abruptly
lumbar
riorly
with
the
,
with
Venograms
view)
azygos
contrast.
no direct
systems
from
and
and
Hypoplastic
IVC-azygos
right
common
iliac vein,
AP
LAT
(lateral
projection).
Biascending
lumbar
veins
filled
prerenal
segment
of IVC,
with
connection.
as continuation
of ascending
has been
retouched.
Additional
Azygos
vertebral
details
vein
commences
veins.
LAT
picture
in Figure
4.
Refractory
cardiac
failure
and poor weight
gain persisted.
At six months
a repeated
catheterization
confirmed
the previous findings.
The pulmonary
artery
pressure
was lower, and
the shunt at the ductus
level was now left-to-right.
The pulmonary-to-systemic
blood flow and vascular
resistance
ratios
were 2.2 : 1 and 0.4 : 1, respectively.
The
ductus
arteriosus
left
dextroposition.
CASE
REPORT
A 20-day-old
girl had respiratory
distress
and cyanosis
(of the lower
body).
Chest
x-ray film showed
cardiac
dextroposition
and radiopacity
of the middle
and lower
lobes
of the right
lung.
Auscultation
revealed
an accentuated
second
heart
sound,
no murmurs,
and decreased
air entry
at the right base. The infant
was digitalized.
At cardiac
catheterization
the venous
catheter
inserted
in
the right superficial
saphenous
vein could
not be advanced
to the right atrium
or to either
azygos
vein, but coursed
up
the veins draining
the middle
and lower
lobes of the right
lung. The pulmonary
artery
pressures,
obtained
via the right
axillary
vein, were greater
than those of the aorta.
A left-toright shunt was present
at the atrial level and a right-to-left
shunt
at the ductus
arteriosus.
Angiography
showed
a sinus
venous
atrial
septal
defect
with anomalous
right pulmonary
venosus
drainage,
a large patent
ductus,
hypoplasia
of the left
heart,
sequestration
of the right middle
and lower
lobes of
the lung,
and anomalies
of the systemic
veins.
Because
of
the high pulmonary
vascular
resistance,
surgery
was deferred.
*From
the
Section
of
Pediatric
Cardiology,
University
illinois
Medical
Center,
Chicago.
Supported
in part by the University
of Illinois
Foundation
Goodenberger
Medical
Research
grant
2-51-39-66-3-14.
Reprint
requests:
Dr. van der Horst,
Pediatric
Cardiology,
840 South
Wood Street, Chicago
60612
638 VAN DER HORST, HASTREITER
of
Ficuns
2. Venograms
at short
veins
courses
LAz
distance
are visualized
caudally
= left azygos
in AP projection.
from
IVC termination
(A)
tip; ( B ) both
azygos
pulmonary
venous
trunk
( PV)
with
IVC below
diaphragm.
the
catheter
and right
to connect
vein.
CHEST, 80: 5, NOVEMBER, 1981
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the NC
termination
this vessel received
a long vertical
pulmonary
venous
trunk
from the middle
and lower
lobes
of
the right lung ( Fig 2B). This vessel,
in turn, interconnected
with a large
right
hepatic
venous
trunk
and its tributaries
( Fig 3B). The latter had no direct connection
to the heart.
p’%’r:’
.
,
,
.
:::.r
The left hepatic vein drained
into the right atrium ( Fig 3C).
The right adrenal
vein also connected
to the vertical
venous
,.
trunk.
The
ascending
posterior
vertebral
veins
connected
with the lumbar
veins, which
continued
as a bilateral azygos
system
into the thorax
connecting
directly
to
large bilateral
superior
venae
cavae.
The left superior
vena
cava
connected
to the right
atrium
by a coronary
sinus.
There
was only a small remnant
of the innominate
vein attached
to the right superior
vena
cava
( Fig 3A).
anteriorly
DiscussioN
Several
tion
the
reports
of the
previous
have
described
IVC
with
reports
infrahepatic
interrup-
azygos
continuation.
In
was
the
IVC
completely
none
of
inter-
rupted.18
The
IVC
The
Ficums
atretic
3. Venograms
in AP projection.
Hypoplastic
and
innominate
vein
( IV ) connecting
to right
superior
vena cava ( SVC) is shown
( A). ( B) illustrates
right lower
pulmonary
veins
interconnecting
with
and
draining
right
hepatic
vein
( RHV ) via venous
plexus,
forming
a common
vein
draining
caudally.
C shows
catheter
advanced
from
right superior
vena cava to right atrium
and contrast
filling
left hepatic
vein ( LVH),
which
connects
directly
to right
atrium.
was
ligated,
but death
occurred
24 hours
later and was attributed
to pulmonary
vascular
obstruction.
At autopsy,
the dilated
right
atrium
drained
a large corenary sinus
( connected
the left superior
vena ) a large
left
hepatic
venous
trunk,
and
a normal
right
superior
vena
cava; it communicated
with the left atrium
via a fossa ovalis
defect.
The dilated
main
pulmonary
artery
divided
into a
large left and a small right branch,
which
supplied
the upper
lobe of the right lung only. The right middle
and lower lobes
were perfused
by a vessel
arising
from the aorta
below
the
diaphragm.
The ductus
arteriosus
was adequately
ligated.
The small left atrium
received
one right
and two left pulmonary
veins.
A single
spleen
was
normally
positioned.
There
was
,
severe
hypoplasia
of the
right
lower
and
middle
lobes
of
the lung.
The large
liver
had a dominant
left lobe.
The
right
kidney
was 1 cm higher
than
the left, and the renal
vessels
were
normal.
The
left adrenal
gland
was located
normally,
and the right
was 1.5 cm higher
than
the upper
pole
of the right
kidney
just beneath
the diaphragmatic
insertion.
The left ovarian
vein drained
into the left renal
vein and the right into the inferior
vena cava.
( IVC).
The NC and tributaries
were abnormal
( Fig 1 to 4). The
NC was to the right of the spine, smaller
than normal
( Fig
1 ), and ended
abruptly
a short
distance
above
the right
renal vein at the level of the first lumbar
vertebra
( Fig 2A).
Here, the IVC connected
posteriorly
with the vertebral
plexus
and the ascending
lumbar
veins situated
behind
the vertebral bodies.
These
veins were extremely
prominent
and
dilated
above
and below
the NC
termination.
Just below
CHEST, 80: 5, NOVEMBER, 1981
develops
junction
of
remnant
of the
segment
pracardinal
caudal
to
system.
cephalad
system.
to
The
posterior
segment,
The
most
cephalad
derived
life
the
The
ample
and
external
the
entirely
from
of
the
which
is
In early
continuity
future
into
the
be-
azygos
veins.
azygos
the
segments.
life,
system
are
thoracic
ex-
ie, the
or
but
there
azygos
was
system.
of
of
to
sys-
form
the
segment
of
interrupted
IVC
the subcardinal
venous
only
direct
anomalies
usually
continuous
was
connection
To
our knowledge,
type
of anomaly.
development
extension
prerenal
channels
directly
not
no
this
venous
join
the
veins.
due
occur.
with
systems.
case,
description
hepatic
variety
of an
the hepatic
and
hemiazygos
of
segmental
veins
Associated
present
components
via
the
of embryonic
IVC
remains
azygos
the
posterior
subcardinal
prerenal
caudal
the
liver.
exception
the
plexus
junction,
to persistence
The
postrenal
and
almost
the
the
most
ie,
the
represents
embryonic
right
The
common
of fusion
of
failure
The
lumbar
veins,
which
the lumbar
intersegmental
of
and
the IVC.
is failure
to
within
anatomic
flow
venous
in
the
hepatosubcardinal
first
ie,
at
segment.
segment,
the azygos
arch,
cardinal
system.
and
system
anterior
and
the
su-
lumbar
veins
and
is basically
anastomosis
of the intercostal
veins. There
connections
between
the lumbar-azygos
sys-
Normally
and
from
the
segment,
ascending
vertebral
rupted,
of
is direct
blood
the
a longitudinal
are
with
IVC
azygos
of
tem
formed
the
ascending
anastomoses
The
only
postrenal
is established
from
are
there
principal
tension
In
IVC
system,
postrenal
comes
from
longitudinal
the
the
component
the
posterior
from
embryonic
veins.
is derived
prerenal
the
the
The
is formed
by the subcardinal
between
the supracardinal
and
of
veins
supracardinal
is
system.
the kidneys
Most
of
originates
azygos
the
components.
veins
the kidneys
by the
renal
portion
of the
prerenal
hepatic
tween
iliac
cardinal
segments
level
of
proximal
five embryologic
common
the kidneys,
connection
subcardinal
tern
from
the
of
of the
the
hepatic
normal
segment
the
IVC
it
this is the
In addition
hepatic
of the
CONGENITAL INTERRUPTION OF INFERIOR VENA CAVA
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inter-
between
veins
IVC,
639
Ficuna
VIEW
the connection
of the
nary
vein
suggested
the
prerenal
The IVC
whence
IVC
ended
it connected
ascending
veins.
The
lumbar
veins.
The
anterior
connected
of the
the
to
veins
gave
and
heart
via
rise
the
to the
azygos
veins
were
channels.
continuity
in our case
from
segmental
vertebral
venous
veins,
vertebral
large
at T11
posterior
large
anatomic
system
the renal
external
veins
IVC.
the IVC
catheter-
venous
anomaly
should
also block
emboli
in the event
of lower
extremity
venous
thrombosis.
Since
there
is normal
continuity
between
the postenor
renal segment
of the IVC and the azygos
veins
in
early
embryonic
unclear
normalities
life,
embryogenesis.
lobe
veins
( these
with
the
tern,
producing
anomaly
The
in the
middle
the
occurrence
development
of the
pulmonary
veins
being
interconnected)
normal
development
the
venous
described
and
severe
of the
an
ab-
lower
and
right
hepatic
of the
may have
discontinuity.
640 VAN DER HORST, HASTREITER
of
right
has
supracardinal
interfered
sys-
our
and autopsy
conception
patient
of venous
based
on
anom-
angiographic
findings.
REFERENCES
and
GA.
The
Negroes
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system
including
of veins
observations
in American
of the
whites
inferior
caval
venous
system.
Am J Phys Anthropol
1934;
19:39-163
2 Abrams
HL. The relationship
of systemic
venous
anomalies to the paravertebral
veins. Am JR 1958; 80:414-19
3 Chuang
of the
VP, Mena CE, Hoskins
PA. Congenital
anomalies
inferior
vena
cava : review
of the embryogenesis
and presentation
between
prevented
This
4. Artistic
in
the pulmosegment
of
1 Sieb
anterior
lumbar
through
The lack of
and the azygos
adrenal
vein
the
subcardinal
with
and
ization
VIEW
right
that
also failed
to develop.
abrupily
at L1 above
plexus
alies
LATERAL
ANTEROPOSTERIOR
1974;
of a simplified
classification.
Br J Radiol
47:206-13
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RC, Adams P, Burke B. Anomalous
inferior
vena
cava with azygos
continuation
( infrahepatic
interruption
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vena cava).
J Pediatr
1961; 59:370-83
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M, Deuchar
DC.
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vena
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splenic
agenesis,
situs
inversus
and
their
embryology.
Br Heart
J 1967;
29:268-75
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Partial
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anomalous
JE,
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LI, Starr
A.
return : report
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SJ,
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KB, Wolfel
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vena
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AJR 1971;
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cal
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CHEST, 80: 5, NOVEMBER, 1981
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