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Congenital heart defects in Kabuki syndrome.
[kabuki syndrome]
Kabuki
syndrome
(
KS
)
is
an
entity
of
multiple
congenital
malformations
with
mental
retardation
with
undetermined
etiology
.
Congenital
heart
defects
are
one
of
the
clinical
manifestations
of
KS
with
insufficient
elucidations
.
Literature
of
congenital
heart
defects
associated
with
KS
was
comprehensively
retrieved
,
collected
and
reviewed
.
The
clinical
features
of
the
congenital
heart
defects
in
the
patients
with
KS
were
summarized
.
Congenital
heart
defects
were
one
of
the
clinical
manifestations
of
KS
with
90
.
6
%
of
the
patients
being
diagnosed
prenatally
or
at
an
early
age
.
Left-sided
obstructions
/
aortic
dilation
and
septal
defects
were
the
fi
rst
two
types
of
anomalies
,
accounting
up
to
46
.
1
%
and
32
.
9
%
,
respectively
.
The
most
common
congenital
heart
defects
were
coarctation
of
the
aorta
,
and
atrial
and
ventricular
septal
defects
.
Fifteen
(
19
.
7
%
)
patients
received
surgical
repair
of
congenital
heart
defects
at
a
mean
age
of
0
.
8
±
1
.
3
years
.
Congenital
heart
defects
are
one
of
the
clinical
manifestations
of
KS
with
90
.
6
%
of
the
patients
being
diagnosed
prenatally
or
at
an
early
age
.
About
20
%
of
the
patients
warranted
surgical
repair
of
the
heart
defects
.
Patients
with
KS
require
close
follow-up
in
terms
of
their
etiology
,
clinical
presentations
and
long
-term
prognosis
.
