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Cardiovascular abnormalities and impaired exercise performance in adolescents with Congenital Adrenal Hyperplasia.
[congenital adrenal hyperplasia]
Context
:
Patients
with
classic
Congenital
Adrenal
Hyperplasia
(
CAH
)
are
treated
with
lifelong
glucocorticoids
(
GCS
)
.
Cardiovascular
(
CV
)
and
metabolic
effects
of
such
therapy
in
adolescents
have
never
been
quantified
.
Objective
:
To
investigate
left
ventricular
(
LV
)
morphology
,
function
and
exercise
performance
in
adolescents
with
CAH
.
Design
and
Setting
:
cross-sectional
and
controlled
study
conducted
at
a
tertiary
referral
centre
.
Patients
:
Twenty
patients
with
classic
CAH
(
10
females
)
aged
13
.
6
±
2
.
5
years
and
20
healthy
controls
comparable
for
sex
and
pubertal
status
were
enrolled
in
the
study
and
compared
to
a
group
of
18
patients
without
CAH
receiving
a
similar
dose
of
GCS
for
Juvenile
Idiopathic
Arthritis
(
JIA
)
.
Main
Outcomes
Measures
:
Echocardiographic
assessment
and
symptom-
limited
exercise
testing
were
performed
.
Anthropometric
,
hormonal
and
biochemical
parameters
were
also
measured
.
Results
:
Compared
to
healthy
controls
,
patients
with
CAH
exhibited
an
increased
BMI
(
p
<
0
.
001
)
,
waist-
to
-height
ratio
(
p
<
0
.
001
)
,
percentage
of
body
fat
(
p
<
0
.
001
)
as
well
as
higher
insulin
concentrations
and
HOMA
index
even
after
adjustment
for
BMI
(
p
=
0
.
03
and
p
=
0
.
05
,
respectively
)
.
Moreover
,
CAH
patients
exhibited
an
impaired
exercise
capacity
as
shown
by
reduced
peak
workload
(
99
±
27
vs
126
±
27
W
,
p
<
0
.
01
)
and
higher
systolic
blood
pressure
response
at
peak
(
156
±
18
vs
132
±
11
mmHg
,
p
<
0
.
01
;
Δ
=
45
±
24
vs
22
±
10
mmHg
,
p
=
0
.
05
)
with
respect
to
healthy
controls
.
CAH
males
displayed
mild
LV
diastolic
dysfunction
as
documented
by
significant
prolongation
of
both
isovolumic
relaxation
time
(
IRT
)
(
118
±
18
vs
98
±
11
ms
,
p
<
0
.
05
)
and
mitral
deceleration
time
(
MDT
)
(
138
±
25
vs
111
±
15
ms
,
p
<
0
.
01
)
.
No
significant
differences
in
CV
function
were
found
between
CAH
and
JIA
patients
.
Conclusion
:
Adolescents
with
CAH
exhibit
impaired
exercise
performance
and
enhanced
systolic
blood
pressure
response
during
exercise
.
In
our
population
,
such
abnormalities
appear
related
to
GCS
therapy
rather
than
CAH
per
se
.
CAH
males
,
but
no
females
,
present
mild
LV
diastolic
dysfunction
that
correlates
with
testosterone
concentrations
suggesting
a
sex
hormone
related
difference
.
Diseases
Validation
Diseases presenting
"mitral deceleration time"
symptom
congenital adrenal hyperplasia
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