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Unusual combination of holt-oram syndrome and persistent left superior vena cava.
[holt-oram syndrome]
Holt
-
Oram
(
HO
)
is
a
syndrome
characterized
by
congenital
cardiovascular
malformations
,
specifically
atrial
and
ventricular
septal
defects
,
and
skeletal
abnormalities
of
the
upper
limbs
bones
.
Associations
of
HO
cardiac
disorders
with
other
congenital
cardiac
malformations
,
specifically
persistent
left
superior
vena
cava
(
PLSVC
)
are
rarely
reported
and
its
real
incidence
is
unknown
.
We
present
a
case
of
this
unusual
combination
in
a
patient
undergoing
cardiac
resynchronization
therapy
(
CRT
)
device
implant
.
A
63
-
year
-old
male
with
HO
and
a
history
of
repaired
atrial
septal
defect
was
presented
for
implantable
cardioverter
defibrillator
(
ICD
)
upgrade
to
CRT
.
The
old
implant
was
located
in
the
right
prepectoral
area
.
The
old
device
pocket
in
the
right
was
accessed
and
a
venous
access
to
the
right
subclavian
vein
was
obtained
.
The
coronary
sinus
(
CS
)
was
easily
cannulated
and
a
long
sheath
advanced
into
the
CS
.
A
contrast
injection
revealed
an
unusually
big-sized
CS
,
with
a
diameter
2
.
5
times
the
fully
deployed
balloon
.
A
0
.
035
wire
was
advanced
retrograde
reaching
the
confluence
of
the
innominate
and
left
subclavian
veins
.
The
outer
sheath
was
advanced
to
this
location
and
contrast
venography
through
the
sheath
allowed
visualization
of
the
left
jugular
and
subclavian
veins
and
visualization
of
the
PLSVC
draining
into
the
CS
.
No
target
veins
for
lead
implant
were
identified
.
The
patient
was
referred
for
surgical
implant
of
an
epicardial
lead
.
Transesophageal
echocardiogram
showed
a
CS
identified
as
an
unusually
big
vascular
structure
located
between
the
left
atrium
and
the
left
atrial
appendage
.
We
report
an
uncommon
association
of
HO
and
PLSVC
.
This
association
was
only
reported
twice
in
the
past
and
this
is
the
first
one
that
constitutes
a
casual
finding
during
the
attempt
of
CRT
device
implant
.
This
is
a
combination
that
may
complicate
a
device
implant
and
recognition
of
it
in
advance
may
avoid
performing
potentially
unsuccessful
procedures
.