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A
22
year
old
man
was
admitted
to
our
hospital
complaining
shortness
of
breath
Case
Presentation
accompanied
by
fever
and
productive
cough.
Dyspnea
on
effort
and
bluish
Bingers
since
3
years.
Physical
examination
revealed
arterial
blood
pressure
of
90/60
mmHg
with
body
height
127
cm
and
weight
34
kg.
There
are
down
slanting
eyes,
low
set
ears
with
helices
prominence,
High
nasal
bridge,
normal
JVP,
pansystolic
murmur
in
upper
and
lower
sternal
border
grade
4/6
radiating
to
carotid
artery.
Lung
examination
was
unremarkable
and
clubbing
of
all
extremities.
ECG
Binding
was
sinus
tachycardia,
superior
axis
and
Right
Ventricular
Hypertrophy.
Chest
X-ray
showed
CTR
was
59%,
aortic
and
pulmonic
segment
was
normal,
cardiac
waist
was
Blattened,
reduced
bronchovascular
pattern
and
active
tuberculosis.
Echocardiography
Bindings
revealed
dilated
RV
and
RA,
D-shaped
LV,
hyperthropic
LV,
Normal
LV
function
with
EF
82%,
Normokinetic,
Diastolic
dysfunction
(impaired
relaxation),
severe
infundibular
stenosis,
severe
TR
,
normal
RV
contractility,
and
mild
pericardial
effusion
(0.8
cm).
Laboratory
Bindings
showed
leukocytosis
with
absolute
neutrophil
count
of
88%
and
hyponatremia.
A
diagnosis
of
Noonan
syndrome
with
short
stature,
facial
feature,
pulmonary
stenosis,
hypertrophic
LV,
and
cryptorchidism,
active
tuberculosis
with
community
acquired
pneumonia
was
made.
The
patient
was
admitted
to
general
ward
and
treated
for
the
lung
infection.
Patient
was
discharged
after
5
days
and
was
scheduled
for
routine
follow
up.
The
patient
was
scheduled
for
elective
surgical
management.
Conclusion
The
management
of
noonan
syndrome
is
based
on
the
speciBic
Bindings,
severity,
and
secondary
disease.
No
speciBic
treatment
for
noonan
syndrome.
Focused
management
on
the
disease
and
complications
control
should
be
routinely
done
to
reduce
the
morbidity
and
mortality
of
noonan
syndrome.
References
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2011; 124 : 2761-2796Allen HD, Driscoll DJ, Shaddy RE dan Feltes TF. Moss and Adams: Heart Disease in Infants, Children, and Adolescents: Including the Fetus and Young Adult. Edisi 7. Lippincott Williams & Wilkins Co, Philadelphia; 2008.