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Partial anomalous left pulmonary artery along with aortic coarctation in an infant with Kabuki syndrome.
[kabuki syndrome]
We
report
an
antenatally
diagnosed
fetal
coarctation
delivered
prematurely
and
confirmed
to
have
coarctation
as
well
as
additional
nonobstructing
anomalous
left
pulmonary
artery
branch
(
aLPA
)
from
right
pulmonary
artery
(
RPA
)
and
significant
dysmorphic
features
.
The
baby
underwent
an
uncomplicated
arch
repair
,
but
had
numerous
multisystem
and
growth
related
issues
prompting
a
diagnosis
of
Kabuki
syndrome
(
KS
)
at
1
year
of
age
.
While
coarctation
and
hypoplastic
left
heart
syndrome
are
observed
in
this
syndrome
,
this
is
the
second
reported
case
of
aLPA
in
KS
and
the
first
with
the
forme
fruste
of
left-sided
obstruction
as
well
as
aLPA
in
this
group
of
patients
.
